A glycoprotein that is central in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C3 can be cleaved into COMPLEMENT C3A and COMPLEMENT C3B, spontaneously at low level or by C3 CONVERTASE at high level. The smaller fragment C3a is an ANAPHYLATOXIN and mediator of local inflammatory process. The larger fragment C3b binds with C3 convertase to form C5 convertase.
The smaller fragment generated from the cleavage of complement C3 by C3 CONVERTASE. C3a, a 77-amino acid peptide, is a mediator of local inflammatory process. It induces smooth MUSCLE CONTRACTION, and HISTAMINE RELEASE from MAST CELLS and LEUKOCYTES. C3a is considered an anaphylatoxin along with COMPLEMENT C4A; COMPLEMENT C5A; and COMPLEMENT C5A, DES-ARGININE.
A subcomponent of complement C1, composed of six copies of three polypeptide chains (A, B, and C), each encoded by a separate gene (C1QA; C1QB; C1QC). This complex is arranged in nine subunits (six disulfide-linked dimers of A and B, and three disulfide-linked homodimers of C). C1q has binding sites for antibodies (the heavy chain of IMMUNOGLOBULIN G or IMMUNOGLOBULIN M). The interaction of C1q and immunoglobulin activates the two proenzymes COMPLEMENT C1R and COMPLEMENT C1S, thus initiating the cascade of COMPLEMENT ACTIVATION via the CLASSICAL COMPLEMENT PATHWAY.
The minor fragment formed when C5 convertase cleaves C5 into C5a and COMPLEMENT C5B. C5a is a 74-amino-acid glycopeptide with a carboxy-terminal ARGININE that is crucial for its spasmogenic activity. Of all the complement-derived anaphylatoxins, C5a is the most potent in mediating immediate hypersensitivity (HYPERSENSITIVITY, IMMEDIATE), smooth MUSCLE CONTRACTION; HISTAMINE RELEASE; and migration of LEUKOCYTES to site of INFLAMMATION.
C5 plays a central role in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C5 is cleaved by C5 CONVERTASE into COMPLEMENT C5A and COMPLEMENT C5B. The smaller fragment C5a is an ANAPHYLATOXIN and mediator of inflammatory process. The major fragment C5b binds to the membrane initiating the spontaneous assembly of the late complement components, C5-C9, into the MEMBRANE ATTACK COMPLEX.
The larger fragment generated from the cleavage of COMPLEMENT C3 by C3 CONVERTASE. It is a constituent of the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb), and COMPLEMENT C5 CONVERTASES in both the classical (C4b2a3b) and the alternative (C3bBb3b) pathway. C3b participates in IMMUNE ADHERENCE REACTION and enhances PHAGOCYTOSIS. It can be inactivated (iC3b) or cleaved by various proteases to yield fragments such as COMPLEMENT C3C; COMPLEMENT C3D; C3e; C3f; and C3g.
Complement System Proteins
Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY).
A 105-kDa serum glycoprotein with significant homology to the other late complement components, C7-C9. It is a polypeptide chain cross-linked by 32 disulfide bonds. C6 is the next complement component to bind to the membrane-bound COMPLEMENT C5B in the assembly of MEMBRANE ATTACK COMPLEX. It is encoded by gene C6.
A 302-amino-acid fragment in the alpha chain (672-1663) of C3b. It is generated when C3b is inactivated (iC3b) and its alpha chain is cleaved by COMPLEMENT FACTOR I into C3c, and C3dg (955-1303) in the presence COMPLEMENT FACTOR H. Serum proteases further degrade C3dg into C3d (1002-1303) and C3g (955-1001).
A component of the CLASSICAL COMPLEMENT PATHWAY. C2 is cleaved by activated COMPLEMENT C1S into COMPLEMENT C2B and COMPLEMENT C2A. C2a, the COOH-terminal fragment containing a SERINE PROTEASE, combines with COMPLEMENT C4B to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).
A 63-kDa serum glycoprotein encoded by gene C9. Monomeric C9 (mC9) binds the C5b-8 complex to form C5b-9 which catalyzes the polymerization of C9 forming C5b-p9 (MEMBRANE ATTACK COMPLEX) and transmembrane channels leading to lysis of the target cell. Patients with C9 deficiency suffer from recurrent bacterial infections.
A 77-kDa subcomponent of complement C1, encoded by gene C1S, is a SERINE PROTEASE existing as a proenzyme (homodimer) in the intact complement C1 complex. Upon the binding of COMPLEMENT C1Q to antibodies, the activated COMPLEMENT C1R cleaves C1s into two chains, A (heavy) and B (light, the serine protease), linked by disulfide bonds yielding the active C1s. The activated C1s, in turn, cleaves COMPLEMENT C2 and COMPLEMENT C4 to form C4b2a (CLASSICAL C3 CONVERTASE).
Complement Membrane Attack Complex
A product of COMPLEMENT ACTIVATION cascade, regardless of the pathways, that forms transmembrane channels causing disruption of the target CELL MEMBRANE and cell lysis. It is formed by the sequential assembly of terminal complement components (COMPLEMENT C5B; COMPLEMENT C6; COMPLEMENT C7; COMPLEMENT C8; and COMPLEMENT C9) into the target membrane. The resultant C5b-8-poly-C9 is the "membrane attack complex" or MAC.
A 80-kDa subcomponent of complement C1, existing as a SERINE PROTEASE proenzyme in the intact complement C1 complex. When COMPLEMENT C1Q is bound to antibodies, the changed tertiary structure causes autolytic activation of complement C1r which is cleaved into two chains, A (heavy) and B (light, the serine protease), connected by disulfide bonds. The activated C1r serine protease, in turn, activates COMPLEMENT C1S proenzyme by cleaving the Arg426-Ile427 bond. No fragment is released when either C1r or C1s is cleaved.
Complement Inactivator Proteins
Serum proteins that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host. The complement system is tightly regulated by inactivators that accelerate the decay of intermediates and certain cell surface receptors.
A 93-kDa serum glycoprotein encoded by C7 gene. It is a polypeptide chain with 28 disulfide bridges. In the formation of MEMBRANE ATTACK COMPLEX; C7 is the next component to bind the C5b-6 complex forming a trimolecular complex C5b-7 which is lipophilic, resembles an integral membrane protein, and serves as an anchor for the late complement components, C8 and C9.
Complement C3-C5 Convertases
Serine proteases that cleave COMPLEMENT C3 into COMPLEMENT C3A and COMPLEMENT C3B, or cleave COMPLEMENT C5 into COMPLEMENT C5A and COMPLEMENT C5B. These include the different forms of C3/C5 convertases in the classical and the alternative pathways of COMPLEMENT ACTIVATION. Both cleavages take place at the C-terminal of an ARGININE residue.
Complement Factor B
Complement Pathway, Alternative
Complement activation initiated by the interaction of microbial ANTIGENS with COMPLEMENT C3B. When COMPLEMENT FACTOR B binds to the membrane-bound C3b, COMPLEMENT FACTOR D cleaves it to form alternative C3 CONVERTASE (C3BBB) which, stabilized by COMPLEMENT FACTOR P, is able to cleave multiple COMPLEMENT C3 to form alternative C5 CONVERTASE (C3BBB3B) leading to cleavage of COMPLEMENT C5 and the assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.
Complement Pathway, Classical
Complement activation initiated by the binding of COMPLEMENT C1 to ANTIGEN-ANTIBODY COMPLEXES at the COMPLEMENT C1Q subunit. This leads to the sequential activation of COMPLEMENT C1R and COMPLEMENT C1S subunits. Activated C1s cleaves COMPLEMENT C4 and COMPLEMENT C2 forming the membrane-bound classical C3 CONVERTASE (C4B2A) and the subsequent C5 CONVERTASE (C4B2A3B) leading to cleavage of COMPLEMENT C5 and the assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.
A 150-kDa serum glycoprotein composed of three subunits with each encoded by a different gene (C8A; C8B; and C8G). This heterotrimer contains a disulfide-linked C8alpha-C8gamma heterodimer and a noncovalently associated C8beta chain. C8 is the next component to bind the C5-7 complex forming C5b-8 that binds COMPLEMENT C9 and acts as a catalyst in the polymerization of C9.
The first complement component to act in the activation of CLASSICAL COMPLEMENT PATHWAY. It is a calcium-dependent trimolecular complex made up of three subcomponents: COMPLEMENT C1Q; COMPLEMENT C1R; and COMPLEMENT C1S at 1:2:2 ratios. When the intact C1 binds to at least two antibodies (involving C1q), C1r and C1s are sequentially activated, leading to subsequent steps in the cascade of COMPLEMENT ACTIVATION.
Receptors, Complement 3b
Molecular sites on or in some B-lymphocytes and macrophages that recognize and combine with COMPLEMENT C3B. The primary structure of these receptors reveal that they contain transmembrane and cytoplasmic domains, with their extracellular portion composed entirely of thirty short consensus repeats each having 60 to 70 amino acids.
Complement Factor H
An important soluble regulator of the alternative pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It is a 139-kDa glycoprotein expressed by the liver and secreted into the blood. It binds to COMPLEMENT C3B and makes iC3b (inactivated complement 3b) susceptible to cleavage by COMPLEMENT FACTOR I. Complement factor H also inhibits the association of C3b with COMPLEMENT FACTOR B to form the C3bB proenzyme, and promotes the dissociation of Bb from the C3bBb complex (COMPLEMENT C3 CONVERTASE, ALTERNATIVE PATHWAY).
The larger fragment generated from the cleavage of C5 by C5 CONVERTASE that yields COMPLEMENT C5A and C5b (beta chain + alpha' chain, the residual alpha chain, bound by disulfide bond). C5b remains bound to the membrane and initiates the spontaneous assembly of the late complement components to form C5b-8-poly-C9, the MEMBRANE ATTACK COMPLEX.
Receptor, Anaphylatoxin C5a
Complement Activating Enzymes
Complement Inactivating Agents
Complement Hemolytic Activity Assay
A screening assay for circulating COMPLEMENT PROTEINS. Diluted SERUM samples are added to antibody-coated ERYTHROCYTES and the percentage of cell lysis is measured. The values are expressed by the so called CH50, in HEMOLYTIC COMPLEMENT units per milliliter, which is the dilution of serum required to lyse 50 percent of the erythrocytes in the assay.
Complement C1 Inactivator Proteins
Receptors, Complement 3d
Molecular sites on or in B-lymphocytes, follicular dendritic cells, lymphoid cells, and epithelial cells that recognize and combine with COMPLEMENT C3D. Human complement receptor 2 (CR2) serves as a receptor for both C3dg and the gp350/220 glycoprotein of HERPESVIRUS 4, HUMAN, and binds the monoclonal antibody OKB7, which blocks binding of both ligands to the receptor.
Serum peptides derived from certain cleaved COMPLEMENT PROTEINS during COMPLEMENT ACTIVATION. They induce smooth MUSCLE CONTRACTION; mast cell HISTAMINE RELEASE; PLATELET AGGREGATION; and act as mediators of the local inflammatory process. The order of anaphylatoxin activity from the strongest to the weakest is C5a, C3a, C4a, and C5a des-arginine.
Complement Fixation Tests
Serologic tests based on inactivation of complement by the antigen-antibody complex (stage 1). Binding of free complement can be visualized by addition of a second antigen-antibody system such as red cells and appropriate red cell antibody (hemolysin) requiring complement for its completion (stage 2). Failure of the red cells to lyse indicates that a specific antigen-antibody reaction has taken place in stage 1. If red cells lyse, free complement is present indicating no antigen-antibody reaction occurred in stage 1.
Complement Factor D
Complement Factor I
A plasma serine proteinase that cleaves the alpha-chains of C3b and C4b in the presence of the cofactors COMPLEMENT FACTOR H and C4-binding protein, respectively. It is a 66-kDa glycoprotein that converts C3b to inactivated C3b (iC3b) followed by the release of two fragments, C3c (150-kDa) and C3dg (41-kDa). It was formerly called KAF, C3bINF, or enzyme 3b inactivator.
Complement C4b-Binding Protein
Complement C3b Inactivator Proteins
Endogenous proteins that inhibit or inactivate COMPLEMENT C3B. They include COMPLEMENT FACTOR H and COMPLEMENT FACTOR I (C3b/C4b inactivator). They cleave or promote the cleavage of C3b into inactive fragments, and thus are important in the down-regulation of COMPLEMENT ACTIVATION and its cytolytic sequence.
Complement C3-C5 Convertases, Classical Pathway
An adrenal microsomal cytochrome P450 enzyme that catalyzes the 21-hydroxylation of steroids in the presence of molecular oxygen and NADPH-FERRIHEMOPROTEIN REDUCTASE. This enzyme, encoded by CYP21 gene, converts progesterones to precursors of adrenal steroid hormones (CORTICOSTERONE; HYDROCORTISONE). Defects in CYP21 cause congenital adrenal hyperplasia (ADRENAL HYPERPLASIA, CONGENITAL).
Complement C3-C5 Convertases, Alternative Pathway
Complement C1 Inhibitor Protein
An endogenous 105-kDa plasma glycoprotein produced primarily by the LIVER and MONOCYTES. It inhibits a broad spectrum of proteases, including the COMPLEMENT C1R and the COMPLEMENT C1S proteases of the CLASSICAL COMPLEMENT PATHWAY, and the MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES. C1-INH-deficient individuals suffer from HEREDITARY ANGIOEDEMA TYPES I AND II.
Complement C3 Convertase, Alternative Pathway
Complement C5 Convertase, Classical Pathway
Molecular Sequence Data
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
Complement C3 Convertase, Classical Pathway
Lupus Erythematosus, Systemic
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Complement C5 Convertase, Alternative Pathway
Amino Acid Sequence
Complement Pathway, Mannose-Binding Lectin
Complement activation triggered by the interaction of microbial POLYSACCHARIDES with serum MANNOSE-BINDING LECTIN resulting in the activation of MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES. As in the classical pathway, MASPs cleave COMPLEMENT C4 and COMPLEMENT C2 to form C3 CONVERTASE (C4B2A) and the subsequent C5 CONVERTASE (C4B2A3B) leading to cleavage of COMPLEMENT C5 and assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.
A 53-kDa protein that is a positive regulator of the alternate pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It stabilizes the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb) and protects it from rapid inactivation, thus facilitating the cascade of COMPLEMENT ACTIVATION and the formation of MEMBRANE ATTACK COMPLEX. Individuals with mutation in the PFC gene exhibit properdin deficiency and have a high susceptibility to infections.
Complement C5a, des-Arginine
Chronic glomerulonephritis characterized histologically by proliferation of MESANGIAL CELLS, increase in the MESANGIAL EXTRACELLULAR MATRIX, and a thickening of the glomerular capillary walls. This may appear as a primary disorder or secondary to other diseases including infections and autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Various subtypes are classified by their abnormal ultrastructures and immune deposits. Hypocomplementemia is a characteristic feature of all types of MPGN.
Genetic Complementation Test
Enzyme-Linked Immunosorbent Assay
An immunoassay utilizing an antibody labeled with an enzyme marker such as horseradish peroxidase. While either the enzyme or the antibody is bound to an immunosorbent substrate, they both retain their biologic activity; the change in enzyme activity as a result of the enzyme-antibody-antigen reaction is proportional to the concentration of the antigen and can be measured spectrophotometrically or with the naked eye. Many variations of the method have been developed.
Strains of mice in which certain GENES of their GENOMES have been disrupted, or "knocked-out". To produce knockouts, using RECOMBINANT DNA technology, the normal DNA sequence of the gene being studied is altered to prevent synthesis of a normal gene product. Cloned cells in which this DNA alteration is successful are then injected into mouse EMBRYOS to produce chimeric mice. The chimeric mice are then bred to yield a strain in which all the cells of the mouse contain the disrupted gene. Knockout mice are used as EXPERIMENTAL ANIMAL MODELS for diseases (DISEASE MODELS, ANIMAL) and to clarify the functions of the genes.
Inflammation of the renal glomeruli (KIDNEY GLOMERULUS) that can be classified by the type of glomerular injuries including antibody deposition, complement activation, cellular proliferation, and glomerulosclerosis. These structural and functional abnormalities usually lead to HEMATURIA; PROTEINURIA; HYPERTENSION; and RENAL INSUFFICIENCY.
Major Histocompatibility Complex
The genetic region which contains the loci of genes which determine the structure of the serologically defined (SD) and lymphocyte-defined (LD) TRANSPLANTATION ANTIGENS, genes which control the structure of the IMMUNE RESPONSE-ASSOCIATED ANTIGENS, HUMAN; the IMMUNE RESPONSE GENES which control the ability of an animal to respond immunologically to antigenic stimuli, and genes which determine the structure and/or level of the first four components of complement.
RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.
The relatively long-lived phagocytic cell of mammalian tissues that are derived from blood MONOCYTES. Main types are PERITONEAL MACROPHAGES; ALVEOLAR MACROPHAGES; HISTIOCYTES; KUPFFER CELLS of the liver; and OSTEOCLASTS. They may further differentiate within chronic inflammatory lesions to EPITHELIOID CELLS or may fuse to form FOREIGN BODY GIANT CELLS or LANGHANS GIANT CELLS. (from The Dictionary of Cell Biology, Lackie and Dow, 3rd ed.)
Disease Models, Animal
Blood Bactericidal Activity
The natural bactericidal property of BLOOD due to normally occurring antibacterial substances such as beta lysin, leukin, etc. This activity needs to be distinguished from the bactericidal activity contained in a patient's serum as a result of antimicrobial therapy, which is measured by a SERUM BACTERICIDAL TEST.
Differentiation antigens residing on mammalian leukocytes. CD stands for cluster of differentiation, which refers to groups of monoclonal antibodies that show similar reactivity with certain subpopulations of antigens of a particular lineage or differentiation stage. The subpopulations of antigens are also known by the same CD designation.
Electrophoresis, Polyacrylamide Gel
Complement C3 Nephritic Factor
An IgG autoantibody against the ALTERNATIVE PATHWAY C3 CONVERTASE, found in serum of patients with MESANGIOCAPILLARY GLOMERULONEPHRITIS. The binding of this autoantibody to C3bBb stabilizes the enzyme thus reduces the actions of C3b inactivators (COMPLEMENT FACTOR H; COMPLEMENT FACTOR I). This abnormally stabilized enzyme induces a continuous COMPLEMENT ACTIVATION and generation of C3b thereby promoting the assembly of MEMBRANE ATTACK COMPLEX and cytolysis.
Multi-subunit proteins which function in IMMUNITY. They are produced by B LYMPHOCYTES from the IMMUNOGLOBULIN GENES. They are comprised of two heavy (IMMUNOGLOBULIN HEAVY CHAINS) and two light chains (IMMUNOGLOBULIN LIGHT CHAINS) with additional ancillary polypeptide chains depending on their isoforms. The variety of isoforms include monomeric or polymeric forms, and transmembrane forms (B-CELL ANTIGEN RECEPTORS) or secreted forms (ANTIBODIES). They are divided by the amino acid sequence of their heavy chains into five classes (IMMUNOGLOBULIN A; IMMUNOGLOBULIN D; IMMUNOGLOBULIN E; IMMUNOGLOBULIN G; IMMUNOGLOBULIN M) and various subclasses.
A deoxyribonucleotide polymer that is the primary genetic material of all cells. Eukaryotic and prokaryotic organisms normally contain DNA in a double-stranded state, yet several important biological processes transiently involve single-stranded regions. DNA, which consists of a polysugar-phosphate backbone possessing projections of purines (adenine and guanine) and pyrimidines (thymine and cytosine), forms a double helix that is held together by hydrogen bonds between these purines and pyrimidines (adenine to thymine and guanine to cytosine).
Surface Plasmon Resonance
A biosensing technique in which biomolecules capable of binding to specific analytes or ligands are first immobilized on one side of a metallic film. Light is then focused on the opposite side of the film to excite the surface plasmons, that is, the oscillations of free electrons propagating along the film's surface. The refractive index of light reflecting off this surface is measured. When the immobilized biomolecules are bound by their ligands, an alteration in surface plasmons on the opposite side of the film is created which is directly proportional to the change in bound, or adsorbed, mass. Binding is measured by changes in the refractive index. The technique is used to study biomolecular interactions, such as antigen-antibody binding.
Glomerulonephritis associated with autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Lupus nephritis is histologically classified into 6 classes: class I - normal glomeruli, class II - pure mesangial alterations, class III - focal segmental glomerulonephritis, class IV - diffuse glomerulonephritis, class V - diffuse membranous glomerulonephritis, and class VI - advanced sclerosing glomerulonephritis (The World Health Organization classification 1982).
Autoantibodies directed against various nuclear antigens including DNA, RNA, histones, acidic nuclear proteins, or complexes of these molecular elements. Antinuclear antibodies are found in systemic autoimmune diseases including systemic lupus erythematosus, Sjogren's syndrome, scleroderma, polymyositis, and mixed connective tissue disease.
Sequence Homology, Amino Acid
Polymerase Chain Reaction
In vitro method for producing large amounts of specific DNA or RNA fragments of defined length and sequence from small amounts of short oligonucleotide flanking sequences (primers). The essential steps include thermal denaturation of the double-stranded target molecules, annealing of the primers to their complementary sequences, and extension of the annealed primers by enzymatic synthesis with DNA polymerase. The reaction is efficient, specific, and extremely sensitive. Uses for the reaction include disease diagnosis, detection of difficult-to-isolate pathogens, mutation analysis, genetic testing, DNA sequencing, and analyzing evolutionary relationships.
Gene Expression Regulation
Measurable and quantifiable biological parameters (e.g., specific enzyme concentration, specific hormone concentration, specific gene phenotype distribution in a population, presence of biological substances) which serve as indices for health- and physiology-related assessments, such as disease risk, psychiatric disorders, environmental exposure and its effects, disease diagnosis, metabolic processes, substance abuse, pregnancy, cell line development, epidemiologic studies, etc.
Mannose-Binding Protein-Associated Serine Proteases
Serum serine proteases which participate in COMPLEMENT ACTIVATION. They are activated when complexed with the MANNOSE-BINDING LECTIN, therefore also known as Mannose-binding protein-Associated Serine Proteases (MASPs). They cleave COMPLEMENT C4 and COMPLEMENT C2 to form C4b2a, the CLASSICAL PATHWAY C3 CONVERTASE.
Adrenal Hyperplasia, Congenital
A group of inherited disorders of the ADRENAL GLANDS, caused by enzyme defects in the synthesis of cortisol (HYDROCORTISONE) and/or ALDOSTERONE leading to accumulation of precursors for ANDROGENS. Depending on the hormone imbalance, congenital adrenal hyperplasia can be classified as salt-wasting, hypertensive, virilizing, or feminizing. Defects in STEROID 21-HYDROXYLASE; STEROID 11-BETA-HYDROXYLASE; STEROID 17-ALPHA-HYDROXYLASE; 3-beta-hydroxysteroid dehydrogenase (3-HYDROXYSTEROID DEHYDROGENASES); TESTOSTERONE 5-ALPHA-REDUCTASE; or steroidogenic acute regulatory protein; among others, underlie these disorders.
The restriction of a characteristic behavior, anatomical structure or physical system, such as immune response; metabolic response, or gene or gene variant to the members of one species. It refers to that property which differentiates one species from another but it is also used for phylogenetic levels higher or lower than the species.
Protein Structure, Tertiary
The level of protein structure in which combinations of secondary protein structures (alpha helices, beta sheets, loop regions, and motifs) pack together to form folded shapes called domains. Disulfide bridges between cysteines in two different parts of the polypeptide chain along with other interactions between the chains play a role in the formation and stabilization of tertiary structure. Small proteins usually consist of only one domain but larger proteins may contain a number of domains connected by segments of polypeptide chain which lack regular secondary structure.
The number of copies of a given gene present in the cell of an organism. An increase in gene dosage (by GENE DUPLICATION for example) can result in higher levels of gene product formation. GENE DOSAGE COMPENSATION mechanisms result in adjustments to the level GENE EXPRESSION when there are changes or differences in gene dosage.
Sequence Homology, Nucleic Acid
Plasma glycoprotein clotted by thrombin, composed of a dimer of three non-identical pairs of polypeptide chains (alpha, beta, gamma) held together by disulfide bonds. Fibrinogen clotting is a sol-gel change involving complex molecular arrangements: whereas fibrinogen is cleaved by thrombin to form polypeptides A and B, the proteolytic action of other enzymes yields different fibrinogen degradation products.
Technique using an instrument system for making, processing, and displaying one or more measurements on individual cells obtained from a cell suspension. Cells are usually stained with one or more fluorescent dyes specific to cell components of interest, e.g., DNA, and fluorescence of each cell is measured as it rapidly transverses the excitation beam (laser or mercury arc lamp). Fluorescence provides a quantitative measure of various biochemical and biophysical properties of the cell, as well as a basis for cell sorting. Other measurable optical parameters include light absorption and light scattering, the latter being applicable to the measurement of cell size, shape, density, granularity, and stain uptake.
A class of C-type lectins that target the carbohydrate structures found on invading pathogens. Binding of collectins to microorganisms results in their agglutination and enhanced clearance. Collectins form trimers that may assemble into larger oligomers. Each collectin polypeptide chain consists of four regions: a relatively short N-terminal region, a collagen-like region, an alpha-helical coiled-coil region, and carbohydrate-binding region.
Lipid-containing polysaccharides which are endotoxins and important group-specific antigens. They are often derived from the cell wall of gram-negative bacteria and induce immunoglobulin secretion. The lipopolysaccharide molecule consists of three parts: LIPID A, core polysaccharide, and O-specific chains (O ANTIGENS). When derived from Escherichia coli, lipopolysaccharides serve as polyclonal B-cell mitogens commonly used in laboratory immunology. (From Dorland, 28th ed)
Lymphocytes responsible for cell-mediated immunity. Two types have been identified - cytotoxic (T-LYMPHOCYTES, CYTOTOXIC) and helper T-lymphocytes (T-LYMPHOCYTES, HELPER-INDUCER). They are formed when lymphocytes circulate through the THYMUS GLAND and differentiate to thymocytes. When exposed to an antigen, they divide rapidly and produce large numbers of new T cells sensitized to that antigen.
Non-antibody proteins secreted by inflammatory leukocytes and some non-leukocytic cells, that act as intercellular mediators. They differ from classical hormones in that they are produced by a number of tissue or cell types rather than by specialized glands. They generally act locally in a paracrine or autocrine rather than endocrine manner.
Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization
A mass spectrometric technique that is used for the analysis of large biomolecules. Analyte molecules are embedded in an excess matrix of small organic molecules that show a high resonant absorption at the laser wavelength used. The matrix absorbs the laser energy, thus inducing a soft disintegration of the sample-matrix mixture into free (gas phase) matrix and analyte molecules and molecular ions. In general, only molecular ions of the analyte molecules are produced, and almost no fragmentation occurs. This makes the method well suited for molecular weight determinations and mixture analysis.
Reverse Transcriptase Polymerase Chain Reaction
Studies which start with the identification of persons with a disease of interest and a control (comparison, referent) group without the disease. The relationship of an attribute to the disease is examined by comparing diseased and non-diseased persons with regard to the frequency or levels of the attribute in each group.
Polymorphism, Restriction Fragment Length
Immune Adherence Reaction
A method for the detection of very small quantities of antibody in which the antigen-antibody-complement complex adheres to indicator cells, usually primate erythrocytes or nonprimate blood platelets. The reaction is dependent on the number of bound C3 molecules on the C3b receptor sites of the indicator cell.
A species of gram-negative, facultatively anaerobic, rod-shaped bacteria (GRAM-NEGATIVE FACULTATIVELY ANAEROBIC RODS) commonly found in the lower part of the intestine of warm-blooded animals. It is usually nonpathogenic, but some strains are known to produce DIARRHEA and pyogenic infections. Pathogenic strains (virotypes) are classified by their specific pathogenic mechanisms such as toxins (ENTEROTOXIGENIC ESCHERICHIA COLI), etc.
A technique that combines protein electrophoresis and double immunodiffusion. In this procedure proteins are first separated by gel electrophoresis (usually agarose), then made visible by immunodiffusion of specific antibodies. A distinct elliptical precipitin arc results for each protein detectable by the antisera.
A chronic systemic disease, primarily of the joints, marked by inflammatory changes in the synovial membranes and articular structures, widespread fibrinoid degeneration of the collagen fibers in mesenchymal tissues, and by atrophy and rarefaction of bony structures. Etiology is unknown, but autoimmune mechanisms have been implicated.
Fluorescent Antibody Technique
Test for tissue antigen using either a direct method, by conjugation of antibody with fluorescent dye (FLUORESCENT ANTIBODY TECHNIQUE, DIRECT) or an indirect method, by formation of antigen-antibody complex which is then labeled with fluorescein-conjugated anti-immunoglobulin antibody (FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT). The tissue is then examined by fluorescence microscopy.
The characteristic 3-dimensional shape of a protein, including the secondary, supersecondary (motifs), tertiary (domains) and quaternary structure of the peptide chain. PROTEIN STRUCTURE, QUATERNARY describes the conformation assumed by multimeric proteins (aggregates of more than one polypeptide chain).
Cells that line the inner and outer surfaces of the body by forming cellular layers (EPITHELIUM) or masses. Epithelial cells lining the SKIN; the MOUTH; the NOSE; and the ANAL CANAL derive from ectoderm; those lining the RESPIRATORY SYSTEM and the DIGESTIVE SYSTEM derive from endoderm; others (CARDIOVASCULAR SYSTEM and LYMPHATIC SYSTEM) derive from mesoderm. Epithelial cells can be classified mainly by cell shape and function into squamous, glandular and transitional epithelial cells.
The intracellular transfer of information (biological activation/inhibition) through a signal pathway. In each signal transduction system, an activation/inhibition signal from a biologically active molecule (hormone, neurotransmitter) is mediated via the coupling of a receptor/enzyme to a second messenger system or to an ion channel. Signal transduction plays an important role in activating cellular functions, cell differentiation, and cell proliferation. Examples of signal transduction systems are the GAMMA-AMINOBUTYRIC ACID-postsynaptic receptor-calcium ion channel system, the receptor-mediated T-cell activation pathway, and the receptor-mediated activation of phospholipases. Those coupled to membrane depolarization or intracellular release of calcium include the receptor-mediated activation of cytotoxic functions in granulocytes and the synaptic potentiation of protein kinase activation. Some signal transduction pathways may be part of larger signal transduction pathways; for example, protein kinase activation is part of the platelet activation signal pathway.
Genetic Predisposition to Disease
A condition characterized by the recurrence of HEMOGLOBINURIA caused by intravascular HEMOLYSIS. In cases occurring upon cold exposure (paroxysmal cold hemoglobinuria), usually after infections, there is a circulating antibody which is also a cold hemolysin. In cases occurring during or after sleep (paroxysmal nocturnal hemoglobinuria), the clonal hematopoietic stem cells exhibit a global deficiency of cell membrane proteins.
Polymorphism, Single Nucleotide
Immune Complex Diseases
Group of diseases mediated by the deposition of large soluble complexes of antigen and antibody with resultant damage to tissue. Besides SERUM SICKNESS and the ARTHUS REACTION, evidence supports a pathogenic role for immune complexes in many other IMMUNE SYSTEM DISEASES including GLOMERULONEPHRITIS, systemic lupus erythematosus (LUPUS ERYTHEMATOSUS, SYSTEMIC) and POLYARTERITIS NODOSA.
Combined genetic deficiency of C6 and C7 in man. (1/127)By routine screening of sera, a subject was discovered who showed a sub-total deficiency of C6 and C7. No clinical disease was associated with this deficiency which was transmitted through the subject's family as a single genetic characteristic, the C6 deficiency being associated with a silent allele at the structural locus. The propositus was found to have low quantities of an abnormal C6 which was both antigenically deficient and smaller in size than normal C6 (110,000 daltons compared with 140,000 daltons) and small quantities of apparently normal C7. It is concluded that the most likely explanation for this defect is that the subject has a structural mutation in his C6 gene which produces hyopsynthesis not only of C6 but also of the closely linked gene for C7. These findings suggest the possibility that C6 and C7 may function as a single genetic unit and that the primary transcript copied from the genome includes information for both proteins. (+info)
Reaction of an activated complex of guinea-pig complement components, C56, with unsensitized erythrocytes and with erythrocytes carrying C3b molecule. (2/127)During the interaction of guinea-pig complement intermediate cells, EAC423, with guinea-pig C5 and C6, an activated complex of C5 and C6, C56, was demonstrated in the fluid phase of the reaction mixture. C56 also was eluted from EAC42356 which had been generated by the interaction of EAC423 with C5 and C6. Both preparations of C56 showed quite similar characteristics and were not distinguished from one another. Both were capable of reacting with unsensitized erythrocytes (E) in the presence of C7 to form EC567. Further, they were able to react with EAC43 in the absence of C7 to form EAC43568 but did react with EAC43 pretreated with C3b inactivator, dithiothreitol or N-bromosuccinimide. These results indicate that guinea-pig C56 generated on EAC423 has a tendency to dissociate into the fluid phase. Nevertheless, the dissociated C56 can bind again to intact C3b molecule on the cells. The ability of cell-bound C3b to combine with C56 may lead to localization of C56 to the cell membrane carrying C3b, resulting in acceleration of attachment of C567 to the membrane. This assumption could be supported by the finding that the replacement of E by EAC43 increased the susceptibility of the cells to lytic action of complement induced by cobra venom factor. Thus, a new function of cell-bound C3b as localizing C56 to the membrane of sensitized cells was indicated. (+info)
C6 produced by macrophages contributes to cardiac allograft rejection. (3/127)The terminal components of complement C5b-C9 can cause significant injury to cardiac allografts. Using C6-deficient rats, we have found that the rejection of major histocompatibility (MHC) class I-incompatible PVG.R8 (RT1.A(a)B(u)) cardiac allografts by PVG.1U (RT1.A(u)B(u)) recipients is particularly dependent on C6. This model was selected to determine whether tissue injury results from C6 produced by macrophages, which are a conspicuous component of infiltrates in rejecting transplants. We demonstrated that high levels of C6 mRNA are expressed in isolated populations of macrophages. The relevance of macrophage-produced C6 to cardiac allograft injury was investigated by transplanting hearts from PVG. R8 (C6-) donors to PVG.1U (C6-) rats which had been reconstituted with bone marrow from PVG.1U (C6+) rats as the sole source of C6. Hearts grafted to hosts after C6 reconstitution by bone marrow transplantation underwent rejection characterized by deposition of IgG and complement on the vascular endothelium together with extensive intravascular aggregates of P-selectin-positive platelets. At the time of acute rejection, the cardiac allografts contained extensive perivascular and interstitial macrophage infiltrates. RT-PCR and in situ hybridization demonstrated high levels of C6 mRNA in the macrophage-laden transplants. C6 protein levels were also increased in the circulation during rejection. To determine the relative contribution to cardiac allograft rejection of the low levels of circulating C6 produced systemically by macrophages, C6 containing serum was passively transferred to PVG.1U (C6-) recipients of PVG.R8 (C6-) hearts. This reconstituted the C6 levels to about 3 to 6% of normal values, but failed to induce allograft rejection. In control PVG.1U (C6-) recipients that were reconstituted with bone marrow from PVG.1U (C6-) donors, C6 levels remained undetectable and PVG.R8 cardiac allografts were not rejected. These results indicate that C6 produced by macrophages can cause significant tissue damage. (+info)
Complement membrane attack complex (C5b-9) mediates interstitial disease in experimental nephrotic syndrome. (4/127)Accumulating evidence suggests that the generation of complement activation products from filtered complement components in urine with nonselective proteinuria leads to tubulointerstitial disease, resulting in progressive loss of renal function. To elucidate the role of C5b-9 in complement-mediated effects on renal tubular cells exposed to proteinuric urine, equivalent levels of proteinuria were induced (using the aminonucleoside of puromycin) in normocomplementemic and genetically C6-deficient piebald viral glaxo (PVG) rats. Semiquantitative histologic analysis revealed that complement-sufficient animals developed more severe tubulointerstitial disease than did C6-deficient rats. Amelioration of tubulointerstitial damage in C6-deficient animals was confirmed by studies with three independent markers of tubular damage, i.e., vimentin, osteopontin, and proliferating cell nuclear antigen. More tubular epithelial cells expressed osteopontin (an early marker of tubular injury) in normocomplementemic rats, compared with C6-deficient rats, at both days 7 and 12. Staining of vimentin in the tubules, near areas of tubular damage, was increased in normocomplementemic rats at day 12, and more proliferating cell nuclear antigen-positive tubular cells were observed at day 12 in complement-sufficient animals. The tubulointerstitial damage in complement-sufficient rats was also associated with greater accumulation of extracellular matrix (fibronectin) at day 12. These studies document for the first time an important role for C6, and therefore C5b-9, in the pathogenesis of nonimmunologic tubulointerstitial injury induced by proteinuria. These findings suggest that C5b-9 formation resulting from proteinuria contributes to the loss of nephron function by damaging the tubulointerstitium and that prevention of C5b-9 formation in tubules could slow the deterioration of renal function. (+info)
Function of the factor I modules (FIMS) of human complement component C6. (5/127)In order to elucidate the function of complement component C6, truncated C6 molecules were expressed recombinantly. These were either deleted of the factor I modules (FIMs) (C6des-748-913) or both complement control protein (CCP) modules and FIMs (C6des-611-913). C6des-748-913 exhibited approximately 60-70% of the hemolytic activity of full-length C6 when assayed for Alternative Pathway activity, but when measured for the Classical Pathway, C6des-748-914 was only 4-6% as effective as C6. The activity difference between C6 and C6des-748-913 for the two complement pathways can be explained by a greater stability of newly formed metastable C5b* when produced by the Alternative Pathway compared with that made by the Classical Pathway. The half-lives of metastable C5b* and the decay of (125)I-C5b measured from cells used to activate the Alternative Pathway were found to be about 5-12-fold longer than those same parameters derived from cells that had activated the Classical Pathway. (125)I-C5 binds reversibly to C6 in an ionic strength-dependent fashion, but (125)I-C5 binds only weakly to C6des-FIMs and not at all to C6des-CCP/FIMs. Therefore, although the FIMs are not required absolutely for C6 activity, these modules promote interaction of C6 with C5 enabling a more efficient bimolecular coupling ultimately leading to the formation of the C5b-6 complex. (+info)
On the mechanism of cytolysis by complement: evidence on insertion of C5b and C7 subunits of the C5b,6,7 complex into phospholipid bilayers of erythrocyte membranes. (6/127)The doughnut hypothesis of cytolysis by complement [Mayer, M. M. (1972) Proc. Nat. Acad. Sci. USA 69, 2954-2958] describes an annular structure made up of C5b-9 (complement factors C5b, C6, C7, C8, and C9) which becomes inserted in the lipid bilayer of the cell membrane, thus creating a hole. We now present initial explorations of this hypothesis. EAC1-6 and EAC1-7 (sheep erythrocytes carrying rabbit antibody and complement factors C1 through C6 or C1 through C7, respectively), prepared with either 125I-C3 or 125I-C5 were incubated with trypsin and the release of bound 125I was measured. In the case of 125I-C3, all of the radioactivity was released by trypsin from both intermediates. With 125I-C5, trypsin released all of the 125I from EAC1-6, but only 40-55% from EAC1-7. Possible reasons for resistance of the C5b subunit in EAC1-7 to tryptic digestion are discussed; in terms of the doughnut hypothesis it would be due to shielding by lipid molecules as a consequence of insertion into the lipid bilayer. In accord with this interpretation we have also found that C5b in EAC1-7, but not in EAC1-6, resists elution by 0.3 M NaC1. Similarly, we have found that 125I-C7 in EAC1-7 resists stripping by trypsin. Hence, we now propose the hypothesis that hydrophobic polypeptide chains from the C5b and the C7 subunits of C5b,6,7 complex become inserted in the phospholipid bilayer and that subsequent reactions with C8 and C9 open a channel across the membrane. (+info)
High prevalence of complement component C6 deficiency among African-Americans in the south-eastern USA. (7/127)Complement component C6 is a part of the membrane attack complex that forms a pore-like structure in cell membranes following complement activation. Deficiency of terminal complement components including C6 predisposes individuals to infection with Neisseriae. Using polymerase chain reaction/single-strand conformation polymorphism analysis followed by DNA sequencing, we screened genomic DNA from 200 randomly chosen blacks and an equal number from whites for three loss-of-function C6 mutations. Ten blacks and two whites were found to be heterozygous for one of the mutations. Two of the mutations, 1195delC and 1936delG, were found exclusively in black individuals. A third previously undescribed mutation, 878delA, was found at equal frequency among the two groups. The difference between the two groups was significant (P = 0.027), indicating that C6 deficiency due to these three mutations is more common among blacks than whites in the local area, principally Jefferson County, Alabama. In addition, three previously undescribed point mutations, two of which result in amino acid substitutions, were identified within exon 6. A review of the county health department records over the past 6 years revealed a higher incidence of meningococcal meningitis in blacks due to serogroups Y and W-135 which paralleled the difference in the estimated prevalence of C6 deficiency. Among black residents of the county (n = 235 598) there were 15 cases of meningitis due to these two serogroups, compared with two cases in the white population (n = 422 604) (P = 0.002). We conclude that C6 deficiency is more common among blacks than whites in the south-eastern United States, with a frequency approaching 1 in 1600 black individuals. (+info)
Increased ion permeability of planar lipid bilayer membranes after treatment with the C5b-9 cytolytic attack mechanism of complement. (8/127)The ion permeability of planar lipid bilayers, as measured electrically, was found to increase modestly upon treatment with purified complement complex C5b,6 and complement components C7 and C8. The subsequent addition C9 greatly amplified this change. No permeability changes occurred when components were added individually to the membrane, or when they were used in paired combinations, or when C5b, C7, C8, and C9 were admixed prior to addition. Thus, there is a significant parallel between the permeability changes induced in the model membrane and damage produced in biological membranes by the C5b-9 complement attack sequence. The efficiency of membrane action by C5b-9 was critically dependent on the order in whcih components were added to the membrane. There were also differences in the electrical properties of membranes treated with C5b-8 and C5b-9, though in both cases the enhanced bilayer permeability is best attributed to the formation of trans-membrane channels. Collectively, the data are consistent with the hypothesis that the mechanism of membrane action by complement involves the production of a stable channel across the lipid bilayer, resulting in cell death by colloid-osmotic lysis. (+info)
Quantitation of activation of the human terminal complement pathway by ELISA<...
TY - JOUR. T1 - Quantitation of activation of the human terminal complement pathway by ELISA. AU - Sanders, Martin E.. AU - Schmetz, Mark A.. AU - Hammer, Carl H.. AU - Frank, Michael M.. AU - Joiner, Keith A.. PY - 1985/12/27. Y1 - 1985/12/27. N2 - We have devised an enzyme-linked immunosorbent assay (ELISA) to quantitate fluid phase terminal complement pathway activation. Upon activation to form C5b-9, terminal complement components express neoantigens not present in the unassembled individual components. Expression of one of these neoantigens occurs at the step of C9 activation. C9 neoantigen is present in fluid phase SC5b-9 complexes, membrane-bound MC5b-9 complexes, and in vitro polymerized C9. Under physiologic conditions, the presence of C9 neoantigen indicates that the terminal complement pathway is activated through the terminal component C9. In our assay for C9 neoantigen, we used rabbit antiserum to polymerized C9 rendered specific for C9 neoantigen c determinants by serial absorption ...
Deficiency of terminal complement pathway inhibitor promotes neuronal tau pathology and degeneration in mice | Journal of...
The neuronal microtubule-associated protein tau becomes hyperphosphorylated and forms aggregates in tauopathies but the processes leading to this pathological hallmark are not understood. Because tauopathies are accompanied by neuroinflammation and the complement cascade forms a key innate immune pathway, we asked whether the complement system has a role in the development of tau pathology. We tested this hypothesis in two mouse models, which expressed either a central inhibitor of complement or lacked an inhibitor of the terminal complement pathway. Complement receptor-related gene/protein y is the natural inhibitor of the central complement component C3 in rodents. Expressing a soluble variant (sCrry) reduced the number of phospho-tau (AT8 epitope) positive neurons in the brain stem, cerebellum, cortex, and hippocampus of aged P301L mutant tau/sCrry double-transgenic mice compared with tau single-transgenic littermates (JNPL3 line). CD59a is the major inhibitor of formation of the membrane attack
Preliminary Report of the Effects of Complement Suppression With Pexelizumab on Neurocognitive Decline After Coronary Artery...
Cerebral IR injury produces a profound inflammatory response characterized by neutrophil, macrophage, and platelet accumulation, upregulation of adhesion molecules, blood-brain barrier destruction, and cytokine production.5 During central nervous system inflammation, complement activation plays a direct role in neuronal cell death6 and has been implicated in many disease processes, including subarachnoid hemorrhage,9 Alzheimer disease,10 trauma,11 and stroke.12,13 In a study of patients who died after ischemic stroke, Lindsberg et al9 demonstrated complement deposition within areas of necrosis and concluded that activation of the terminal complement pathway with membrane attack complex assembly occurs within cerebral infarct zones. Others have shown that complement depletion before cerebral IR injury may have neuroprotective effects in animal models.12,14 Huang et al12 used a mouse model of middle cerebral artery occlusion and reperfusion to demonstrate that administering a potent inhibitor of ...
Immunological aspects of immunodeficiency diseases part 99 | MEDICINE FOR ALL
The key event in complement activation is the proteolytic cleavage of C3 to C3a and C3b. Three pathways can lead to C3 cleavage, namely, classical, alternative, and mannose-binding lectin (MBL) pathways. C3 cleavage leads on to the activation of the terminal complement pathway, causing the generation of the membrane attack complex (MAC), which assembles…
Melted terminal components on Red Brick battery - Batteries/Power - The Steadicam Forum
Melted terminal components on Red Brick battery - posted in Batteries/Power: Hi All, I know this isnt really Steadicam related, but I was hoping I could draw on your experience and knowledge with this nasty situation. We hired a RED from one of my freinds to use at the BSC show, and he has just told me that a terminal on the charger and on one battery has melted. I have seen no photos so im not sure exactly what he means, but he is saying hte plastic parts around the power terminal...
Activation of p38α/β MAPK in myogenesis via binding of the scaffold protein JLP to the cell surface protein Cdo | JCB
MAPKs function as the terminal components of three-tiered cascades of kinases comprised of a MAPK kinase kinase (MAP3K), MAPK kinase (MAP2K), and MAPK and are important signal transducers in development, homeostasis, and disease (Chang and Karin, 2001). For example, the p38 subfamily of MAPKs is involved in a wide variety of biological processes, including inflammation, stress responses, and cell differentiation (Zarubin and Han, 2005). The myriad roles of MAPK cascades indicate that the specificity of MAPK activation and function must be regulated. One mechanism by which this occurs is via MAPK scaffold proteins, which are thought to provide (1) specificity between distinct MAPK subfamilies by assembling individual MAPK modules and (2) precise spatial and temporal regulation to MAPK signaling (Morrison and Davis, 2003). How this latter function is accomplished is unclear, but it suggests that scaffold proteins may interact with cell-type specific factors.. Differentiation of cells in the ...
Glomeruli of Dense Deposit Disease contain components of the alternative and terminal complement pathway<...
TY - JOUR. T1 - Glomeruli of Dense Deposit Disease contain components of the alternative and terminal complement pathway. AU - Sethi, Sanjeev. AU - Gamez, Jeffrey D.. AU - Vrana, Julie A.. AU - Theis, Jason D.. AU - Bergen, H. Robert. AU - Zipfel, Peter F.. AU - Dogan, Ahmet. AU - Smith, Richard J.H.. PY - 2009/5/1. Y1 - 2009/5/1. N2 - Dense Deposit Disease (DDD), or membranoproliferative glomerulonephritis type II, is a rare renal disease characterized by dense deposits in the mesangium and along the glomerular basement membranes that can be seen by electron microscopy. Although these deposits contain complement factor C3, as determined by immunofluorescence microscopy, their precise composition remains unknown. To address this question, we used mass spectrometry to identify the proteins in laser microdissected glomeruli isolated from paraffin-embedded tissue of eight confirmed cases of DDD. Compared to glomeruli from five control patients, we found that all of the glomeruli from patients with ...
Test Database | Derby Teaching Hospitals NHS Foundation Trust
CH50 and CH100 measures the integrity of the classical and terminal complement pathways. Indicated in the investigation of suspected immune deficiency associated with recurrent pyogenic infections, recurrent/atypical meningococcal infections and atypical immune complex disorders.AP100 measures the integrity of the alternative and terminal complement pathways. Rare deficiencies of AP components predispose to neisserial infections.AP100 measures the integrity of the alternative and terminal complement pathways. Rare deficiencies of AP components predispose to neisserial infections ...
Terminal complement complex in synovial tissue from patients affected by rheumatoid arthritis, osteoarthritis and acute joint...
The C5b-9 complex (Terminal Complement Complex-TCC) is the final product of the terminal complement pathway. In this study, using the monoclonal antibody MCaE11 (specific for a C9 neoantigen) and an immunohistochemical technique, we examined the TCC deposits in synovial tissues from 4 patients affected by rheumatoid arthritis (RA) and 6 patients affected by osteoarthritis (OA). Synovial tissues from 8 patients affected by acute joint trauma were examined as controls. Furthermore, plasma TCC levels were measured in 44 RA patients and 51 controls, using the above mentioned antibody and a sandwich ELISA. Eight synovial fluids were also included in this study. Abundant TCC deposits were detected in the cytoplasm of the synovial lining cells and of large stromal mononuclear cells in all the RA and in 3 out of 6 OA synovial tissues characterized by histological signs of inflammation. No TCC deposits were found in non-inflamed synovial tissues from patients with joint trauma. In agreement with previous ...
Granulocytic infiltrate occurs in the absence of demyel | Open-i
Granulocytic infiltrate occurs in the absence of demyelination, terminal complement complex formation, and overt tissue destruction in NMO white matter. a H&
Effect of recombinant growth hormone replacement in a growth hormone deficient subject recovering from mild traumatic brain...
TY - JOUR. T1 - Effect of recombinant growth hormone replacement in a growth hormone deficient subject recovering from mild traumatic brain injury. T2 - A case report. AU - Bhagia, Vinita. AU - Gilkison, Charles. AU - Fitts, Robert H.. AU - Zgaljardic, Dennis J.. AU - High, Walter M.. AU - Masel, Brent E.. AU - Urban, Randall. AU - Mossberg, Kurt A.. PY - 2010. Y1 - 2010. N2 - Objective: To assess the effects of growth hormone (GH) replacement in an individual who sustained mild traumatic brain injury (mTBI) as an adult and was found to have GH deficiency by glucagon stimulation testing. Participant: A 43-year old woman who sustained a mild TBI at age 37 years. She was 6.8 years post-injury when she began supplementation. Intervention: Recombinant human GH (rhGH) subcutaneously per day for 1 year. Main outcome measures: Single fibre muscle function was evaluated from muscle biopsies. Body composition, muscle strength and peak aerobic capacity were also measured. In addition, neuropsychological ...
Complement membrane attack complex - Wikipedia
The membrane attack complex (MAC) or terminal complement complex (TCC) is a structure typically formed on the surface of pathogen cell membranes as a result of the activation of the hosts complement system, and as such is one of the effector proteins of the immune system. The membrane-attack complex (MAC) forms transmembrane channels. These channels disrupt the cell membrane of target cells, leading to cell lysis and death. Active MAC is composed of the subunits C5b, C6, C7, C8 and several C9 molecules. A number of proteins participate in the assembly of the MAC. Freshly activated C5b binds to C6 to form a C5b-6 complex, then to C7 forming the C5b-6-7 complex. The C5b-6-7 complex binds to C8, which is composed of three chains (alpha, beta, and gamma), thus forming the C5b-6-7-8 complex. C5b-6-7-8 subsequently binds to C9 and acts as a catalyst in the polymerization of C9. ...
CD59, an LY-6-like protein expressed in human lymphoid cells, regulates the action of the complement membrane attack complex on...
A novel cell surface antigen has been identified on a wide range of lymphoid cells and erythrocytes. A mAb YTH 53.1 (CD59) against this antigen enhanced the lysis of human red cells and lymphocytes by homologous complement. Studies of reactive lysis using different species of C56, and of whole serum used as a source of C7-9, indicated that the inhibitory activity of the CD59 antigen is directed towards the homologous membrane attack complex. CD59 antigen was purified from human urine and erythrocyte stroma by affinity chromatography using the mAb YTH 53.1 immobilized on Sepharose, and, following transient expression of a human T cell cDNA library in COS cells, the corresponding cDNA also identified using the antibody. It was found that the CD59 antigen is a small protein (approximately 20 kD as judged by SDS-PAGE, 11.5 kD predicted from the isolated cDNA) sometimes associated with larger components (45 and 80 kD) in urine. The sequence of CD59 antigen is unlike that of other complement ...
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GlobalDatas clinical trial report, Neisseria meningitidis Infections Global Clinical Trials Review, H2, 2015 provides an overview of Neisseria meningitidis Infections clinical trials scenario. This report provides top line data relating to the clinical trials on Neisseria meningitidis Infections. Report includes an overview of trial numbers and their average enrollment in top countries conducted across the globe. The report offers coverage of disease clinical trials by region, country (G7 & E7), phase, trial status, end points status and sponsor type. Report also provides prominent drugs for in-progress trials (based on number of ongoing trials). GlobalData Clinical Trial Reports are generated using GlobalDatas proprietary database - Pharma eTrack Clinical trials database. Clinical trials are collated from 80+ different clinical trial registries, conferences, journals, news etc across the globe. Clinical trials database undergoes periodic update by dynamic process.. The report enhances the ...
THE RESPONSE TO THE FEEDING OF CEVITAMIC ACID IN NORMAL AND DEFICIENT SUBJECTS AS MEASURED BY A VITAMIN C EXCRETORY TEST* |...
The criterion for the diagnosis of scurvy had been, until recently, the appearance of the symptoms of scurvy. As a result of the experimental work in vitamin C, three new diagnostic procedures have now come into use: the capillary resistance test, the concentration of vitamin C in the blood and the urinary excretion of vitamin C.1, 2, 3, 4, 5 There is still some controversy as to the value of capillary resistance tests as an index of vitamin C deficiency. We have studied the capillary resistance of 25 young normal adults by the Dalldorf method5 and found no definite relation ...
Factors Influencing Treatment of Atypical Hemolytic Uremic Syndrome | American Society of Nephrology
An example within the aHUS group demonstrates how unique, pathology-related factors influence the treatment of aHUS in these relative settings. As mentioned previously, at the genetic level, a CFH mutation may be transcribed into a nonfunctional protein leading to aHUS. However, loss of factor H function can also be the consequence of autoantibodies to this protein. Although the phenotypic consequence is dysregulation of the alternate pathway of complement and aHUS, different targeted treatments are possible because the pathophysiology is fundamentally different. Certainly, anticomplement therapy appears to work for both types of aHUS. However, in patients with autoimmune-mediated HUS, a more direct therapy would target the antibody and not the terminal complement pathway. This tenet is the basis for considering anticellular immune suppression to reduce antibody titer and limit risk for exacerbation without compromising complement activity in this select patient cohort.. The study by Chinchilla ...
Products (more) - Diatec monoclonals
Diatec Monoclonals offers an excellent monoclonal antibody against human C5b-9, the Terminal Complement Complex (TCC). The antibody is produced in vitro by the aE11 hybridoma and purified with protein A to a purity more than 98%.. This antibody recognizes C5b-9, the terminal complement complex (TCC). TCC consist of C5b, C6, C7, C8 and C9 and contains neoantigens that are absent from the individual native components.. aE11 produces monoclonal antibodies against neoantigens of both membrane bound (MAC) and fluid phase (SC5b-9) complexes. This antibody is directed against a neoepitope exposed in C9 when incorporated into the terminal complement complex (TCC). For research customers we ask you to contact our international distributor:. BioPorto Diagnostics. If you are in need of larger amounts of C5b-9 (,20 mg), please contact us direct.. ...
Complement Component 3 Deficiency disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials
MalaCards based summary : Complement Component 3 Deficiency, also known as c3 deficiency, is related to complement factor i deficiency and neisseria meningitidis infection, and has symptoms including recurrent bacterial infections An important gene associated with Complement Component 3 Deficiency is C3 (Complement C3), and among its related pathways/superpathways are Innate Immune System and Creation of C4 and C2 activators. Affiliated tissues include t cells, skin and brain, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2) ...
CD59 Antibody, anti-human, REAfinity™ | Recombinant antibodies | MACS Antibodies | Products | Miltenyi Biotec | USA
Clone REA496 recognizes the human CD59 antigen, a 20 kDa LY-6 like protein, which regulates the action of the complement membrane attack complex on homologous cells. This glycoprotein is widely distributed on the membranes of human erythrocytes and leukocytes. CD59, also known as protectin, was observed in vascular endothelia throughout the body, in extravascular tissues, and was also found in ductal epithelia of pancreatic, biliary and salivary systems, bronchi, and kidney collecting ducts. Furthermore, CD59 is expressed in the epidermis and in the syncytiotrophoblast of placenta.Additional information: Clone REA496 displays negligible binding to Fc receptors. | USA
JCI - Welcome
Understanding tumor resistance to T cell immunotherapies is critical to improve patient outcomes. Our study revealed a role for transcriptional suppression of the tumor-intrinsic HLA class I (HLA-I) antigen processing and presentation machinery (APM) in therapy resistance. Low HLA-I APM mRNA levels in melanoma metastases prior to immune checkpoint blockade (ICB) correlated with non-responsiveness to therapy and poor clinical outcome. Patient-derived melanoma cells with silenced HLA-I APM escaped recognition by autologous CD8+ T cells. However, targeted activation of the innate immunoreceptor RIG-I initiated de novo HLA-I APM transcription thereby overcoming T cell resistance. Antigen presentation was restored in interferon (IFN)-sensitive but also immunoedited IFN-resistant melanoma models through RIG-I-dependent stimulation of an IFN-independent salvage pathway involving IRF1 and IRF3. Likewise, enhanced HLA-I APM expression was detected in RIG-I (DDX58)-high melanoma biopsies, correlating with ...
Death Following HPV vaccine | AHRP
Herein reported is the case of a 15-year-old female without a relevant medical history, who developed severe headaches, speech problems, dizziness, weakness, inability to walk, depressed consciousness, confusion, amnesia and vomiting, 14 days after receiving her first qHPV vaccine injection. After the second vaccine booster, her symptoms worsened and she expired 15 days later. Autopsy revealed cerebral oedema and cerebellar herniation indicative of a focally disrupted blood-brain barrier.. There was no evidence of an active brain infection. Immunohistochemistry (IHC) examination of the brainstem, hippocampus and the cerebellum showed prominent infiltration of T-lymphocytes and macrophages in all brain areas examined. Notably, marked activation of the complement membrane attack complex (MAC) was detected in the cerebellar Purkinje cells, hippocampal neurons and portions of the brainstem. This pattern of MAC activation in the absence of an active brain infection indicates an abnormal triggering of ...
Research programme: nanoparticle therapeutics - Regenesance - AdisInsight
Regenesance is developing nanoparticle formulations of inhibitors of the complement membrane attack complex (MAC) for the treatment of peripheral neuropathy.
Clinical Implications of Studies of Erythrocytes with Elevated Erythrocytic ATP. | Annals of Internal Medicine | American...
Elevated erythrocyte ATP is a recently described inherited abnormality in which erythrocytes of some affected individuals contain levels of ATP (6.0 µm/g hemoglobin) twice the normal mean (3.1 µm). Genetic studies of this Negro kindred are consistent with simple autosomal inheritance. Genes for glucose-6-phosphate dehydrogenase (G-6-PD) deficiency and sickle hemoglobin also occur in the kindred but segregate independently. The propositus, whose erythrocytes have twice normal levels of ATP, is G-6-PD deficient. He is as susceptible to primaquine-induced hemolysis as other G-6-PD deficient subjects, and drug ingestion does not influence his erythrocytic ATP level; these findings argue strongly against one current ...
Vitamin D - Integrative Energetics™
Vitamin D. In a June 2008 issue of the Archives of Internal Medicine, Austrian researchers checked vitamin D levels in blood tests from more than 3,200 male and female heart patients whose average age was 62. Blood tests were conducted frequently for more than eight years.. Results confirmed the potential danger of vitamin D deficiency. Subjects with the lowest D levels were significantly more likely to die of any cause over the study period. And even when researchers excluded patients with serious heart risk factors, they found that vitamin D deficient subjects were more likely to die of heart-related complications.. Another D study - published within days of the Austrian research - offers very promising results for anyone who experiences chronic pain.. In the June 2008 issue of Pain Treatment Topics, editor Stewart B. Leavitt, Ph.D., reports on a meta-analysis of more than 20 studies that included patients with osteoarthritis, muscle pain, joint pain, bone pain, fibromyalgia, and other chronic ...
The Effect of Pharmacological Antilipolysis on the Metabolic Effects of Ghrelin - Full Text View - ClinicalTrials.gov
Ghrelin is a relatively new hormone that is produced in the stomach and to a lesser extend in the hypothalamus of the brain. The actions of ghrelin are diverse and includes stimulation of the appetite center of the brain and the release of growth hormone. We have for the first time shown that ghrelin also stimulates the metabolism of fatty acids and induces insulin resistance in skeletal muscle. These effects have we confirmed in growth hormone deficient subjects on a stabile substitution treatment with growth hormone and hydrocortisone. With these subjects we can investigate the effects of ghrelin that are independent of growth hormone. The present study is a continuation of these findings, as we wish to investigate whether the insulin resistance effect of ghrelin is dependent of the concomitant metabolism of fatty acids. This is possible by administration of the niacin acid antagonist Acipimox, that blocks the fatty acid metabolism reversibly. We have applied this experimental principle in ...
Ayurvedic Treatment for Anemia
Anemia is a complex condition that occurs due to certain direct medical or physical causes or as a result of some other disease. The wide range of causes is deficiency of RBC formation, hereditary deficiency, Vitamin B 12 deficiency, Iron and folic acid deficiency. Ayurveda uses the inherent power of natural herbs to bring about wonderful results on the human body.
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5.B.1 The gp91phox Phagocyte NADPH Oxidase-associated Cytochrome b558 (Phox) Family The human phagocyte cytochrome b558 is a heterodimeric complex consisting of a heavy (β) chain (gp91phox) and a light (α) chain (p22phox) as well as several auxiliary subunits (Geisz and Leto, 2004). The β-chain is a glycoprotein of 570 amino acyl residues called gp91phox, the product of the X-linked chronic granulomatous disease gene. The protein bears (1) the heme-binding site in its N-terminal 280 residues, and (2) an FAD binding site (residues 338-344) as part of the C-terminal NADPH oxidase domain. The N-terminal domain has 6 putative transmembrane spanners (TMSs) and is the cytochrome binding site. It has been reported to catalyze efflux of protons through an H+ channel that acts as a charge compensation pathway for the electrogenic generation of the superoxide radical, O2&149;-. The proposal that (gp91phox) has H+ channel activity has been effectively disputed and is now in doubt (DeCoursey 2003; ...
SMART: Secondary literature for KIND domain
BACKGROUND: Titin is a huge protein ( approximately 3 MDa) that is present in the contractile unit (sarcomere) of striated muscle and has a key role in muscle assembly and elasticity. Titin is mainly composed of two types of module (type I and II). Type I modules are found exclusively in the region of titin localised in the A band, where they are arranged in a super-repeat pattern that correlates with the ultrastructure of the thick filament. No structure of a titin type I module has been reported so far. RESULTS: We have determined the structure of a representative type I module, A71, using nuclear magnetic resonance (NMR) spectroscopy. The structure has the predicted fibronectin type III fold. Titin-specific conserved residues are either located at the putative module-module interfaces or along one side of the protein surface. Several proline residues that contribute to two stretches in a polyproline II helix conformation are solvent-exposed and line up as a continuous ribbon extending over ...
Neisseria Meningitidis Infections, Pipeline Review, H1 2015
Neisseria Meningitidis Infections-Pipeline Review, H1 2015. Summary. Global Markets Directs, Neisseria Meningitidis Infections-Pipeline Review, H1 2015, provides an overview of the Neisseria Meningitidis Infectionss therapeutic pipeline.. This report provides comprehensive information on the therapeutic development for Neisseria Meningitidis Infections, complete with comparative analysis at various stages, therapeutics assessment by drug target, mechanism of action (MoA), route of administration (RoA) and molecule type, along with latest updates, and featured news and press releases. It also reviews key players involved in the therapeutic development for Neisseria Meningitidis Infections and special features on late-stage and discontinued projects.. Global Markets Directs report features investigational drugs from across globe covering over 20 therapy areas and nearly 3,000 indications. The report is built using data and information sourced from Global Markets Directs proprietary ...
Neisseria Meningitidis Infections - Product Pipeline Review Market Size and Share 2015 - Acute Market Reports
Neisseria Meningitidis Infections - Product Pipeline Review - 2015, provides an overview of the Neisseria Meningitidis Infectionss therapeutic
Molecular organization of C9 within the membrane attack complex of complement. Induction of circular C9 polymerization by the...
TY - JOUR. T1 - Molecular organization of C9 within the membrane attack complex of complement. Induction of circular C9 polymerization by the C5b-8 assembly. AU - Podack, E. R.. AU - Tschoop, J.. AU - Muller-Eberhard, H. J.. PY - 1982. Y1 - 1982. N2 - Evidence has been presented suggesting that during assembly of the membrane attack complex (MAC) of complement, the C5b-8 complex induces polymerization of C9. The C9 polymer was detected by sodium dodecyl sulfate (SDS) gel electrophoresis of MAC isolated from complement-lysed erythrocytes. It resembled the previously described polymerized C9 (poly C9) produced from isolated monomeric C9 by prolonged incubation at 37° C in that it was resistant to dissociation by SDS and reducing agents and had an apparent molecular weight of ~1.1 million. The presence of poly C9 in the MAC was further supported by the expression of identical neoantigens by the MAC and poly C9 and by the high C9 content of the MAC relative to its other constituents. Isolated C8 in ...
Zerebroarterielle Amyloidose vom Holländischen Typ
Egan LJ et. al. (1994) Hereditary deficiency of the seventh component of complement and recurrent meningococcal infection: investigations of an Irish family using a novel haemolytic screening assay for complement activity and C7 M/N allotyping.. [^] ...
Modulation of interleukin production by ascorbic acid
We studied the influence of ascorbate (vitamin C) on peripheral blood mononuclear cells (PBMC) of pigs with hereditary deficiency in ascorbate synthesis. Groups of animals were depleted of, or supplemented with dietary ascorbate for up to 5 weeks. B lymphocytes and T lymphocyte subsets differed in t …
C8B - Complement component C8 beta chain precursor - Homo sapiens (Human) - C8B gene & protein
Constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells.
Variability of Low-Molecular-Weight, Heat-Modifiable Outer Membrane Proteins of Neisseria meningitidis | Infection and Immunity
Analysis of major outer membrane protein (MOMP) profiles of various meningococci by sodium dodecyl sulfate (SDS)-polyacrylamide gel electrophoresis (SDS-PAGE) revealed the presence of 0 to 2 low-molecular-weight, heat-modifiable MOMPs (molecular weight, 25,000 to 32,000) and 1 to 3 high-molecular-weight MOMPs (molecular weight, 32,000 to 46,000). Heat modifiability was investigated by comparing MOMP profiles after heating in SDS solutions at 100°C for 5 min or at 40°C for 1 h. Low-molecular-weight MOMPs shifted to higher apparent molecular weights after being heated at 100°C. Heat modifiability of high-molecular-weight MOMPs varied among strains; whenever modified these proteins shifted to lower apparent molecular weights after complete denaturation. Variability of low-molecular-weight, heat-modifiable MOMPs was demonstrated when MOMP profiles were compared of (i) isolates from index cases and associated cases and carriers among contacts, (ii) different isolates from the same individual, and ...
Eculizumab blocks vaccine-induced opsonophagocytic killing of meningococci by whole blood from immunized adults | Blood Journal
Eculizumab, a humanized anti-complement C5 monoclonal antibody for treatment of paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome, blocks the terminal complement pathway required for serum bactericidal activity (SBA). Because treated patients are at ,1000-fold increased risk of meningococcal disease, vaccination is recommended, but whether vaccination can protect by opsonophagocytic activity in the absence of SBA is not known. Meningococci were added to anticoagulated blood from 12 healthy adults vaccinated with meningococcal serogroup B and serogroup A,C,W,Y vaccines. Bacterial survival was measured after 3 hours incubation in the presence of eculizumab or, a control complement factor D inhibitor, ACH-4471, that blocks the alternative complement pathway (AP) and is in phase 2 development for treatment of PNH. In the absence of inhibitors, CFU/ml in blood from all 12 immunized subjects decreased from ~4000 at time 0 to sterile cultures at 3 hours. In the presence ...
Complement system activation contributes to the ependymal damage induced by microbial neuraminidase -ORCA
Background In the rat brain, a single intracerebroventricular injection of neuraminidase from Clostridium perfringens induces ependymal detachment and death. This injury occurs before the infiltration of inflammatory blood cells; some reports implicate the complement system as a cause of these injuries. Here, we set out to test the role of complement. Methods The assembly of the complement membrane attack complex on the ependymal epithelium of rats injected with neuraminidase was analyzed by immunohistochemistry. Complement activation, triggered by neuraminidase, and the participation of different activation pathways were analyzed by Western blot. In vitro studies used primary cultures of ependymal cells and explants of the septal ventricular wall. In these models, ependymal cells were exposed to neuraminidase in the presence or absence of complement, and their viability was assessed by observing beating of cilia or by trypan blue staining. The role of complement in ependymal damage induced by ...
Oxidative Stress Sensitizes RPE Cells To Complement-Mediated Injury In A Lectin Pathway- And Malondialdehyde Epitope-Dependent...
Purpose: : Uncontrolled activation of the alternative complement pathway is thought to be associated with age-related macular degeneration (AMD). Previously, we have shown that in retinal pigmented epithelium (RPE) monolayers, oxidative stress reduced complement inhibitor expression and function on the cell surface, resulting in sublytic activation of the membrane attack complex . Here we examined the potential ligand and pathway(s) involved in initiating complement-dependent RPE cell damage by oxidative stress. Methods: : ARPE-19 cells were grown as monolayers on transwell plates. Sublytic complement activation was induced by challenging monolayers with H2O2 in the presence of complement-sufficient normal human serum (NHS). Since sublytic complement activation results in VEGF release, which in turn reduces barrier function, transepithelial electrical resistance (TER) measurements were used as a measure of cell injury. Results: : (1) TER deteriorated rapidly in H2O2-exposed monolayers upon ...
The membrane attack complex of complement: Relation of C7 to the metastable membrane binding site of the intermediate complex...
TY - JOUR. T1 - The membrane attack complex of complement. T2 - Relation of C7 to the metastable membrane binding site of the intermediate complex C5b-7. AU - Preissner, K. T.. AU - Podack, E. R.. AU - Muller-Eberhard, H. J.. PY - 1985/1/1. Y1 - 1985/1/1. N2 - Isolated C7 (m.w. 120,000) in 1% deoxycholate (DOC) forms dimers with an apparent m.w. of 230,000 and a DOC-binding capacity of 82 mol per mol of dimer. Dimerization of C7 also occurs in the presence of DOC-phospholipid mixed micelles and eventuates in the insertion of C7 dimers into the lipid bilayer upon the removal of the detergent, C5b-7 complex formation in the fluid phase or on lipid vesicles likewise involves polymerization, C5b-7 sedimented with 17-40S, which suggests a dimeric to hexameric composition. In avidin-biotin binding experiments in which two differentially labeled forms of C5b,6 (biotinyl 125I-C5b,6, and 131I-C5b,6) were used in equimolar amounts to assemble C5b-7, more than 50% of the biotinyl 125I-C5b,6-containing ...
We have been interested in developing a complement dependent opsonophagocytic assay for Neisseria meningitidis serogroups A and C. Our problem is that when we add a complement source (baby rabbit) we get (as expected) bactericidal killing. We have tried to remove one of the terminal complement components but without much success (can remove component(s) but the removal process reduces the lytic complement activity when we reconstitute with the purified complement component). We have had success using a serum from a C7 defficient patient, however, this complement source is in limited supply. QUESTIONS: 1. is anyone else working on this? have you had success? 2. is there a method to remove terminal components in human sera that does not reduce complement activity when the component is added back? can this method be used for large vols. (600 ml)? 3. did I miss something in the literature? 4. any ideas or suggestions? P#: (404)639-3622 F#: (404)639-3115 George ...
Complement Component C9 - 製品情報: Leica Biosystems
Complement component C9 binds to the C5b-8 complex as the final protein of the membrane attack complex. After binding, it undergoes a conformational change and inserts itself into the cell membrane, forming transmembrane channels.
The membrane attack complex of complement: relation of C7 to the metastable membrane binding site of the intermediate complex...
Isolated C7 (m.w. 120,000) in 1% deoxycholate (DOC) forms dimers with an apparent m.w. of 230,000 and a DOC-binding capacity of 82 mol per mol of dimer. Dimerization of C7 also occurs in the presence of DOC-phospholipid mixed micelles and eventuates in the insertion of C7 dimers into the lipid bilayer upon the removal of the detergent. C5b-7 complex formation in the fluid phase or on lipid vesicles likewise involves polymerization. C5b-7 sedimented with 17-40S, which suggests a dimeric to hexameric composition. In avidin-biotin binding experiments in which two differentially labeled forms of C5b,6 (biotinyl 125I-C5b,6, and 131I-C5b,6) were used in equimolar amounts to assemble C5b-7, more than 50% of the biotinyl 125I-C5b,6-containing complexes also contained 131I label; again suggesting that C5b-7 consisted of oligomers rather than monomers. The conformation of C7 in C5b-7 and in dimeric C7 appeared similar by the following criteria. On formation of C5b-7 from C5b,6 and C7, a 20% increase in ...
C9 - Complement component C9 precursor - Homo sapiens (Human) - C9 gene & protein
Constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells (PubMed:9634479, PubMed:9212048, PubMed:26841934). C9 is the pore-forming subunit of the MAC (PubMed:4055801, PubMed:26841934, PubMed:30111885).
Diastereoselective Strecker Synthesis of -Aminonitriles on Carbohydrate Templates.
trisaccharide-3-uloside 19[l is obtained, which, upon normal methyl branching reactions (MeLi, MeMgCI), gives the wrong, namely the D-arabino-configurated, terminal component (cf. ). Thus, a controlled branching reaction was necessary, which was first worked out for the monosaccharide. Reductive debromination (h, 91%) of 10 and subsequent oxidation (b, 75%) affords the deoxygenated 3-uloside 11 .Ish1 The methylene group can be introduced either by Wittig reaction (j,64%) to give 12171 or by Peterson olefination (k, 46%) to give the derivative 13.l7lAll attempts to carry out a Sharpless epoxidationlyl of the exocyclic allylic alcohol system of 13 were u n s u c c e ~ s f u l Apparently, .~~~~ the chiral elements in the saccharide derivatives 12 and 13 already direct the epoxidation with rn-chloroperbenzoic acid (1, 83%) completely stereospecifically to give the (3R)-configurated epoxides 14l7] and 15, respectively. Reductive opening of the epoxide results (c, 75%) in exclusive formation of ...
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Die genetische Anfälligkeit für Meningokokken-Infektionen liegt vor allem in Störungen des Komplementsystems begründet, vor allem der terminale membrane attack complex (MAC), der von C8 und C9 gebildet wird, aber auch Teile des Komplementsystems, die die Bildung des MAC steuern, können betroffen sein (C3, C5, C6, C7).. ...
Complement components are upregulated and correlate with disease progression in the TDP-43 Q331K mouse model of amyotrophic...
Components of the innate immune complement system have been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS) specifically using hSOD1 transgenic animals; however, a comprehensive examination of complement expression in other transgenic ALS models has not been performed. This study therefore aimed to determine the expression of several key complement components and regulators in the lumbar spinal cord and tibialis anterior muscle of TDP-43Q331K mice during different disease ages. Non-transgenic, TDP-43WT and TDP-43Q331K mice were examined at three different ages of disease progression. Expression of complement components and their regulators were examined using real-time quantitative PCR and enzyme-linked immunosorbent assay. Localisation of terminal complement component receptor C5aR1 within the lumbar spinal cord was also investigated using immunohistochemistry. Altered levels of several major complement factors, including C5a, in the spinal cord and tibialis anterior muscle of TDP
Optimization of Selenoprotein P in Chinese Subjects - Full Text View - ClinicalTrials.gov
Selenium is an essential nutrient. The Recommended Dietary Allowance (RDA) for selenium is considered to be the amount required to optimize plasma selenoproteins. There are two selenoproteins present in the plasma: selenoprotein P (Se-P) and glutathione peroxidase 3 (GPX-3). Although the amount of selenium required to optimize GPX-3 has been determined, the amount required to optimize Se-P in the plasma remains unknown. We aim to determine this amount by supplementing selenium deficient subjects with varying amounts of selenium as selenomethionine.. The study will take place in Mianning County, China, where selenium status is low. Approximately 150 people will be screened for eligibility to participate in this study. 98 subjects will be enrolled. Background information obtained from each participant will be: age, height, weight and smoking status.. Subjects will be randomized to a selenium supplement group. The selenium supplement will contain either 0, 20, 40, 60, 80, 100, or 120 µg of ...
Publications | Hart Long Centrum Leiden
OBJECTIVES: Complement activation is considered an important mediator of myocardial ischaemia/reperfusion (I/R) injury. Although complement inhibitors are highly effective in animals, clinical trials fail to show a substantial benefit in humans. This raises questions on the role of complement activation in human myocardial I/R injury. METHODS: Soluble C5b-9, i.e. terminal complement complex, and C5a were assessed in patients with non-ischaemic (n = 10) and ischaemic heart failure (n = 10), and p... Abstract ...
Complement C6 - NB120-11899 | acris-antibodies.com
Complement C6, 50 µg. C6 is a component of complement cascade. It is part of the membrane attack complex which can insert into the cell membrane and cause cell to lyse.
Surgical aspects of islet cell transplantation | [email protected]
In this study, the initial experience of the authors using the lower rectum as a novel site for transplantation of free islets is reported. The reasons for selecting the rectum as a potential site are, firstly, that there is a rich plexus of veins draining into the portal venous system, secondly, that it is safe and convenient for access, both for grafting procedures and biopsies and thirdly, that repeat procedures are possible. About 1500 intact islets from three donor PVG rat pancreases were implanted, using an injection technique,into the submucosa of the lower rectum circumferentially above the dentate line of recipient syngeneic PVG rats (n=31). Biopsy of these transplanted islets showed revascularisation of islet clusters in the submucosa of the rectum for greater than 60 days. Clinical trials of islet cell grafting have commenced worldwide over the last 18 months, and this study describes a simple technique for islet cell grafting which could potentially be applied in diabetic patients. ...
Formation, Characterization and Function of a Stable Fluid Phase C5-C9 Complex | The Journal of Immunology
Previous studies in this laboratory have allowed the formulation of a model for the molecular arrangement of C5, C6, C7, C8, and C9 on the surface of cells undergoing immune cytolysis with an assigned cumulative m.w. of 995,000. To verify directly the existence of a C5-C9 complex, serum samples containing radiolabeled terminal components were activated at 37°C with EA, antigen-antibody complexes, CVF, inulin or zymosan. Subsequent sucrose density gradient ultracentrifugation showed that all treatments cited led to the formation, in varying degrees, of rapidly sedimenting material which incorporated C5, C6, C7, C8, and C9, but not C3. The reaction was inhibited by 0.01 M EDTA and 0°C. The complex had a sedimentation coefficient of 22.4S, a diffusion coefficient of 1.98 × 10-7 cm2 sec-1 and thus a calculated m.w. of 1.04 × 106.. ...
COX7A2 - Wikipedia
Cytochrome c oxidase polypeptide 7A2, mitochondrial is an enzyme that in humans is encoded by the COX7A2 gene. Cytochrome c oxidase (COX), the terminal component of the mitochondrial respiratory chain, catalyzes the electron transfer from reduced cytochrome c to oxygen. This component is a heteromeric complex consisting of 3 catalytic subunits encoded by mitochondrial genes and multiple structural subunits encoded by nuclear genes. The mitochondrially encoded subunits function in electron transfer, and the nuclear-encoded subunits may function in the regulation and assembly of the complex. This nuclear gene encodes polypeptide 2 (liver isoform) of subunit VIIa and the polypeptide 2 is present in both muscle and nonmuscle tissues. In addition to polypeptide 2, subunit VIIa includes polypeptide 1 (muscle isoform), which is present only in muscle tissues, and a related protein, present in all tissues. This gene may have several pseudogenes. GRCh38: Ensembl release 89: ENSG00000112695 - Ensembl, May ...
Tài liệu Complex Interaction chọn lọc - TaiLieu.VN
The complement system (Chap. 308) consists of a group of serum proteins functioning as a cooperative, self-regulating cascade of enzymes that adhere to- and in some cases disrupt-the surface of invading organisms. Some of these surface-adherent proteins (e.g., C3b) can then act as opsonins for destruction of microbes by phagocytes. The later, terminal components (C7, C8, and C9) can directly kill some bacterial invaders (notably, many of the neisseriae) by forming a membrane attack complex and disrupting the integrity of the bacterial membrane, thus causing bacteriolysis. ...
Home Page | Matsubara Lab: Research on Retinal Diseases & Treatments
Membrane attack complex (MAC) is formed under the combined stimulation of amyloid beta (Aβ) and normal human serum (NHS), immunolabeled with a monoclonal mouse anti-human C5b-9 antibody and subsequently visualized by Cy3 (red). RPE cell nuclei are counter-stained with DAPI. Scale bars: 20 μm. See full article online. Read More ...
Anti Human CD59 Antibody, clone MEM-43 | Bio-Rad
|strong|Mouse anti Human CD59 antibody, clone MEM-43|/strong| recognizes CD59, a glycosyl-phosphatidylinositol (GPI) anchored membrane protein also known as membrane attack complex inhibition factor. …
Irv Arons Journal: Gene Therapy in Ophthalmology Update 5: A Complement-Based Gene Therapy for AMD
Dr. Elias Reichel, of Tufts University School of Medicine and a founder of Hemera Biosciences, Inc., of Boston, MA, presented on a new approach to treating the dry form of age-related macula degeneration. His paper was based on the research being done by Hemera Biosciences on HMR59, a naturally occurring protein that protects retinal cells from damage by MAC (Membrane Attack Complex), that can be delivered for long-lasting activity via a gene therapy approach. ...
Functional complement assays were developed to find patients suffering from complement deficiency. Today these assays are also widely used in drug development of new complement targeted drugs.
active C1 - oi
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Complement proteins C6-C9 all contain a MACPF domain and assemble into the membrane attack complex. C6, C7 and C8β appear to be ... proteins of the complement system (C6, C7, C8α, C8β and C9) and perforin (PF). Members of this protein family are pore-forming ... 3OJY Complement regulatory proteins such as CD59 function as MAC inhibitors and prevent inappropriate activity of complement ... "Nonsense mutation in exon 4 of human complement C9 gene is the major cause of Japanese complement C9 deficiency". Hum. Genet. ...
C5 is activated by CVFBb in the presence of complement component C6 and the C5b6 complex is formed. However, when C6 is added ... The binding of C5 is influenced by C6 and C7, components which are thought to act subsequent to it in the complement sequence. ... The target of C5 convertase is complement protein C5. C5 is a two-chain (α, β) plasma glycoprotein (Mr = 196,000). C5 and C3 ... In these respects, the mode of action of C5 is completely analogous to that of the other components of complement. The C5 step ...
Radang bahasa Indonesia, ensiklopedia bebas
A complex of the complement proteins C5b, C6, C7, C8, and multiple units of C9. The combination and activation of this range of ... Able to break down fibrin clots, cleave complement protein C3, and activate Factor XII. ... complement proteins forms themembrane attack complex, which is able to insert into bacterial cell walls and causes cell lysis ...
... and complement components C6 and C7". Immunogenetics. 56 (2): 89-106. doi:10.1007/s00251-004-0665-2. ISSN 0093-7711. PMID ...
... complement complex (C5-C6-C7), interleukin 5, and histamine (though this has a narrow range of concentration). Harm resulting ...
Outline of immunology
C6 C7 C8 C9 Complement pathway inhibitors C1-inhibitor - Classical, Lectin, Alternate Decay-accelerating factor (CD59) - ... system Complement system Classical complement pathway Mannan-binding lectin pathway Alternate complement pathway Complement ... see complement proteins section) Collectins Mannan-binding lectin (MBL) Surfactant protein A (SP-A) Surfactant protein D (SP-D ... Classical complement pathway C1Q complex - C1R / C1S C4 - C4a C2 Mannan-binding lectin pathway MASP1 / MASP2 Mannan-binding ...
Alternative complement pathway
C5b binds sequentially to C6, C7, C8 and then to multiple molecules of C9 to form membrane attack complex. ... there are several different kinds of regulatory proteins that disrupt the complement activation process: *Complement Receptor 1 ... Complement Factor H can inhibit the formation of the C3 convertase by competing with factor B for binding to C3b; accelerate ... "Inhibition of the alternative complement pathway by antisense oligonucleotides targeting complement factor B improves lupus ...
Complement membrane attack complex
MAC is composed of a complex of four complement proteins (C5b, C6, C7, and C8) that bind to the outer surface of the plasma ... Media related to Complement membrane attack complex at Wikimedia Commons. *Complement+Membrane+Attack+Complex at the US ... The membrane attack complex (MAC) or terminal complement complex (TCC) is a structure typically formed on the surface of ... C6-C9 all contain a common MACPF domain. This region is homologous to cholesterol-dependent cytolysins from Gram-positive ...
Complement component 9
C9 is one member of the complement membrane attack complex (MAC), which also includes complement components C5b, C6, C7 and C8 ... MAC formation starts with the assembly of a tetrameric complex with the complement components C6, C7, C8, and C5b. The final ... Complement component 9 (C9) is a MACPF protein involved in the complement system, which is part of the innate immune system. ... Complement+9 at the US National Library of Medicine Medical Subject Headings (MeSH) PDBe-KB provides an overview of all the ...
Complement component 5
The C5b macromolecular cleavage product can form a complex with the C6 complement component, and this complex is the basis for ... Identification of the C5b-binding domain in complement C6". J. Biol. Chem. 264 (30): 18041-51. PMID 2808363. Wetsel RA, Lemons ... Complement component 5 is involved in the complement system. It is cleaved into C5a and C5b: C5a plays an important role in ... Complement component 5 is the fifth component of complement, which plays an important role in inflammatory and cell killing ...
Complement component 6
"Entrez Gene: complement component 6 C6 complement C6 (Homo sapiens (human)) Gene ID: 729". www.ncbi.nlm.nih.gov. 24 November ... Complement component 6 is a protein that in humans is encoded by the C6 gene. Complement component 6 is a protein involved in ... Complement+C6 at the US National Library of Medicine Medical Subject Headings (MeSH) v t e. ... 2005). "Nucleotide sequence analyses of human complement 6 (C6) gene suggest balancing selection". Ann. Hum. Genet. 69 (Pt 3): ...
Terminal complement pathway deficiency
C6, C7, and C8. (While C9 is part of the MAC, and deficiencies have been identified, it is not required for cell lysis.) People ... Terminal complement pathway deficiency is a genetic condition affecting the complement membrane attack complex (MAC). It ... Initial complement tests often include C3 and C4, but not C5 through C9. Instead, the CH50 result may play a role in diagnosis ... Suspect terminal complement pathway deficiency with patients who have more than one episode of Neisseria infection. ...
C6, C7, C8, and polymeric C9. MAC is the cytolytic endproduct of the complement cascade; it forms a transmembrane channel, ... complement factor B, and complement factor I, as well as deletion of complement factor H-related 3 and complement factor H- ... Complement deficiencyEdit. Main article: Complement deficiency. It is thought that the complement system might play a role in ... Three biochemical pathways activate the complement system: the classical complement pathway, the alternative complement pathway ...
Chevrolet Corvette (C6)
The interior had a red interior and complemented accenting on the seats, console and door panels. The arm rest features Ron ... In 2012, the Corvette C6.R won again in the GTE-Am category. In addition to the American Le Mans Series, Corvette C6.Rs also ... Production variants include the Z06, C6 RS, ZR1, Grand Sport, and 427 Convertible. Racing variants include the C6.R, an ... ISBN 978-1-905741-62-5. Chevrolet Corvette C6 at National Corvette Museum Official C6 Vette Registry. ...
The C5b then recruits and assembles C6, C7, C8 and multiple C9 molecules to assemble the membrane attack complex. This creates ... Polymorphisms of complement component 3, complement factor B, and complement factor I, as well as deletion of complement factor ... The complement system, also known as complement cascade, is a part of the immune system that enhances (complements) the ability ... Three biochemical pathways activate the complement system: the classical complement pathway, the alternative complement pathway ...
... protein) in the complement cascade system of immune proteins (native immunity) Cervical vertebra 6, one of the cervical ... C-6), a 1892 United States Navy protected cruiser C-6 Ute, a military version of the Beechcraft King Air airplane C-6, United ... IATA code C6), former air carrier Soprano C, a musical note designated C6 C6 GPMG, Canadian designation of the FN MAG machine ... C6, C06, C VI or C-6 may refer to: AEG C.VI, a German World War I reconnaissance aircraft Bavarian C VI, an 1899 German steam ...
Complement component 1s
... (EC 126.96.36.199, C1 esterase, activated complement C1s, complement C overbar 1r, C1s) is a protein ... complement activation, lectin pathway. • complement activation. • regulation of complement activation. Sources:Amigo / QuickGO ... complement activation, classical pathway. • immune system process. • innate immune response. • ... Sim RB (1981). "The human complement system serine proteases C1r and C1s and their proenzymes". Methods in Enzymology. 80 Pt C ...
Classical complement pathway
Subsequent interactions between C5b and other terminal components C6, C7, C8, and C9 form the membrane attack complex or the ... Alternative complement pathway - another complement system pathway Lectin pathway - another complement system pathway Noris, ... The classical complement pathway is one of three pathways which activate the complement system, which is part of the immune ... Activation of the complement pathway through the classical, lectin or alternative complement pathway is followed by a cascade ...
Autoimmune hemolytic anemia
Cases may also arise with complement alone or with IgA, IgM or a combination of these three antibody classes and complement. ... C6, C7, C8, C9) either can form the membrane attack complex (MAC) or can bind the antibody, aiding phagocytosis by macrophages ... Antibodies are produced against the RBCs, which leads to complement activation. Complement fragments, such as C3a, C4a and C5a ... IgM is a potent activator of the classical complement pathway, thus, AIHA involving IgM is characterized by complement mediated ...
The class E6 and c6 Stadtbahn rolling stock remained in service but was phased out by the end of the year 2008 and has since ... 1995 been complemented and finally replaced by new class T and T1 low-floor rolling stock. On the suburban line the regular ... These are referred to as class E6 (motor cars) and c6 (trailers) and are based on the Duewag "Mannheim" design. These were in ...
Duality (Peter Leitch and John Hicks album)
The AllMusic reviewer concluded that "Leitch, a lyrical player in the style of Jim Hall, is beautifully complemented by Hicks' ... piano technique." "Pas De Trois" - 7:11 "Epistrophy" - 6:23 "For B.C." - 6:45 "H&L" - 7:13 "O'Grand Amour" - 8:59 "Dancing in ...
Complement deficiencies are the result of a lack of any of these proteins. They may predispose to infections but also to ... C6 deficiency (idem) C7 deficiency (idem, vasculitis) C8a deficiency C8b deficiency C9 deficiency (Neisserial infections) C1- ... The complement system is part of the innate as well as the adaptive immune system; it is a group of circulating proteins that ... MASP2 deficiency Complement receptor 3 (CR3) deficiency Membrane cofactor protein (CD46) deficiency Membrane attack complex ...
Bombardment of Qui Nhơn
She had a complement of fifty officers and enlisted men. Commander Schenck arrived off Qui Nhơn of July 30 and prepared to ...
Outwood Academy Acklam
The system also complements the school curriculum, where in many cases, students in Years 9 and 10 and 11 are in the same ... A C5a is given if the student misbehaves in detention or does attend it.C6 is given when C5 is not completed or misbehaving ... The school previously used a system of escalating "Consequences" as a framework for School discipline,ranging from C1 to C6 ( ...
Côn Sơn Island
The personnel complement for the station was two officers and 23 enlisted men. After commissioning on 2 September 1966 the ...
The high-performance version of the C6 Audi A6, the S6 uses the Volkswagen Group C6 platform, and is available in saloon/sedan ... A new Audi S6, now officially known as the Audi S6 quattro appeared in 1999, to complement its A6 platform-mate. It was ... Yet, in a 2007 track test by Road & Track, the C6 S6 went from 0-60 mph (97 km/h) in 5.1 seconds, while it covered the quarter- ... The engine in the C6 S6 is an even firing all-aluminium alloy 5,204 cubic centimetres (317.6 cu in) Fuel Stratified Injection ( ...
List of primary immunodeficiencies
Complement deficiencies are the result of a lack of any of these proteins. They may predispose to infections but also to ... C6 deficiency (idem) C7 deficiency (idem, vasculitis) C8a deficiency C8b deficiency C9 deficiency (Neisserial infections) C1- ... Cherubism COPA defect Otulipenia/ORAS The complement system is part of the innate as well as the adaptive immune system; it is ... MASP2 deficiency Complement receptor 3 deficiency Membrane cofactor protein (CD46) deficiency Membrane attack complex inhibitor ...
C5b associates with C6, C7, C8, and C9, all of which form a complex that results in a pore through the pathogen's membrane. ... The C1 complement complex binds to these antibodies resulting in its activation via cross proteolysis. This activated C1 ... C3b is the larger of two elements formed by the cleavage of complement component 3, and is considered an important part of the ... The key to the success of the complement system in clearing antigens is regulating the effects of C3b to pathogens alone and ...
The resistance of a genospecies of Lyme disease spirochetes to the bacteriolytic activities of the alternative complement ... a whole-cell sonicate enzyme immunoassay followed by a VlsE C6 peptide enzyme immunoassay". Clinical Infectious Diseases. 53 (6 ... inactivating key immune components such as complement, and hiding in the extracellular matrix, which may interfere with the ...
Komplemendisüsteem - Vikipeedia
Zipfel, P. F., Hallström, T., & Riesbeck, K. (2013). Human complement control and complement evasion by pathogenic microbes- ... C6, C7, C8 ja polümeerset C9 valku. Membraaniründe kompleks on komplemendisüsteemi ahelreaktsioonide tsütolüütiline lõpp- ... 1,0 1,1 1,2 1,3 Rus, H., Cudrici, C., & Niculescu, F. (2005). The role of the complement system in innate immunity. Immunologic ... 7,0 7,1 Lambris, J. D., Ricklin, D., & Geisbrecht, B. V. (2008). Complement evasion by human pathogens. Nature Reviews. ...
Type C4-class ship
Complement:. Varied by design type. The Type C4-class ship were the largest cargo ships built by the United States Maritime ... Type C6). Emergency cargo. *Liberty ship ("EC2-S-C1"). *Victory ship ("VC2-S-AP1") ...
Corrocher R, Tedesco F, Rabusin P, De Sandre G (1975). "Effect of human erythrocyte stromata on complement activation". Br. J. ...
p. C6.. Alternate Link via ProQuest.. *^ Gruber, William (April 5, 1978). "Jewel expects trust probe in Skaggs merger". Chicago ... The acquisition of both Osco and Turn Style allowed Jewel to expand into non-food related retailing that would complement their ... to complement their food store division when building one-stop shopping destinations, such as the new Family Centers and Jewel- ...
"The normal human female as a mosaic of X-chromosome activity: studies using the gene for C-6-PD-deficiency as a marker" ... eliminate the differences in gene dosage between affected individuals and individuals with a normal chromosome complement. In ...
Separation of powers
"Esprit des lois (1777)/L11/C6 - Wikisource". fr.wikisource.org (in French). Retrieved 11 March 2018.. ... Calvin suggested setting up several political institutions which should complement and control each other in a system of checks ...
... complement complex (C5-C6-C7), interleukin 5, and histamine (though this has a narrow range of concentration). ...
replace or complement in some plant taxa the starch as storage carbohydrate ... Naphthoquinones (C6-C4). *Phenylpropanoids (C6-C3). *Xanthonoids (C6-C1-C6). *Coumarins and isocoumarins ...
Sengeløv H (1996). "Complement receptors in neutrophils.". Crit. Rev. Immunol. 15 (2): 107-31. PMID 8573284. ... MAC (C6, C7, C8, C9). Inhibitori. CLA: C1-inhibitor - Faktor ubrzanja raspada/CD59 - Faktor I CL: C4BP ... "Function, structure and therapeutic potential of complement C5a receptors". British Journal of Pharmacology 152 (4): 429-48. ...
Sîstema temamker - Wîkîpediya
a b https://www.britannica.com/science/complement-immune-system-component *↑ a b c d e f g h i OpenStax, Microbiology, openstax ... Xeleka ji proteînên C6, C7, C8, û C9 ê pêk tê wekî pêkhateya êrîşa parzûnê bi nav dibe (bi îngilîzî: membrane attack complex ( ... C5 bi proteînên C6, C7, C8 û C9 an ve li ser rûyê hokara nexweşiyê de xelekek ava dikin. Di nav xelekê de C9 hê pirtir kom dibe ... Sîstema temamker an jî sîstema tewawker (bi îngilîzî: complement system) beşek ji sîstema bergiriya giştî ye. Sîstema ...
... is a member of the immunoglobulin superfamily, with a structure related to the putative primordial form of the family. As members of the immunoglobulin superfamily play fundamental roles in intercellular recognition involved in various immunologic phenomena, differentiation, and development, basigin is thought also to play a role in intercellular recognition (Miyauchi et al., 1991; Kanekura et al., 1991). It has a variety of functions. In addition to its metalloproteinase-inducing ability, basigin also regulates several distinct functions, such as spermatogenesis, expression of the monocarboxylate transporter and the responsiveness of lymphocytes. Basigin is a type I integral membrane receptor that has many ligands, including the cyclophilin (CyP) proteins Cyp-A and CyP-B and certain integrins. It is expressed by many cell types, including epithelial cells, endothelial cells and leukocytes. The human basigin protein contains 269 amino acids that form two heavily ...
키네이스 - 위키백과, 우리 모두의 백과사전
ATP의 감마 인산기를 D-포도당의 C6로 전달하여 포도당-6-인산으로 전환한다. 인산기의 음전하로 인해 포도당-6-인산은 세포질 내에 머무르고 해당 과정으로 들어갈 수 있다. 탈인산화된 형태의 포도당은 다시 세포막을 거쳐 세포 ... Manning, G; Whyte, DB; Martinez, R; Hunter, T; Sudarsanam, S (2002년 12월 6일). "The protein kinase complement of the human genome ...
... complement receptors, Fc receptors, B cell receptors and T cell receptors. ...
This domain consists of molecular regions that make hydrophobic interactions with the FKB domain and triene region from C-1-C-6 ...
Pattern recognition receptor
C5b recruits C6, C7, C8 and multiple C9s. C5, C6, C7, C8 and C9 form the membrane attack complex (MAC). This is another large ... Complement receptors, collectins, ficolins, pentraxins such as serum amyloid and C-reactive protein, lipid transferases, ... Once bound to the ligands MBL and Ficolin oligomers recruit MASP1 and MASP2 and initiate the lectin pathway of complement ... "/"self turned nonself" type pathogen pattern are also identified and destroyed (e.g. by complement fixation or other cytotoxic ...
... and complemented by steering wheel button controls. Ford initially anticipated that over 85% of Fiesta orders would include ... driving a Chevrolet Corvette C6 in the Festival Place Basingstoke shopping mall, to participating in an amphibious beach ...
Kasparov versus the World
Exchanging knights on c6 would have been silly for Kasparov, as it would have brought a black pawn to c6, giving the World Team ... By this point in the game, several chess clubs had begun posting daily analysis to complement what was available on the ... Black's queen was safe on the light squares with White's light-squared bishop off and preferred to develop the knight to c6. ...
List of OMIM disorder codes
PSAP Complement component 4, partial deficiency of; 120790; C1NH Complement factor H deficiency; 609814; HF1 Complement factor ... C5 C6 deficiency; 612446; C6 C7 deficiency; 610102; C7 Caffey disease; 114000; COL1A1 Campomelic dysplasia with autosomal sex ...
Adiponectin (adipocyte complement-related protein of 30 kDa) is a cytokine produced by adipose tissue and its deficiency is ... In T-cadherin deficient C6 glioma cell lines, its overexpression results in growth suppression involving p21CIP1/WAF1 ...
St. Xavier High School (Ohio)
p. C6. Retrieved September 13, 2009. 'A lot of times people will ask three times if I'm Phil Cox. I know I've lost business ... Later, a tuition-free elementary school division opened to complement the college. In 1844, the school's elementary division ... p. C6. Retrieved August 9, 2007. "News from the Long Blue Line". St. Xavier High School Magazine. St. Xavier High School. ...
Power Mac G4 Cube
p. C6. Retrieved November 6, 2020. Edwards, Benj (August 12, 2010). "Apple's Cube was ahead of its time". MacUser. IDG. ... Alongside the Cube, Apple introduced a new mouse, keyboard, and displays to complement the machine. Rumors of a cube-shaped ...
Birmingham-Shuttlesworth International Airport
Concourse C, which opened on August 14, 2014, consists of six gates: C1-C6. It is used by Southwest and United. Former ... The current complement of personnel is over 300 full-time personnel, including military and civilian employees. This expands to ...
complement component C6 precursor [Mus musculus] - Protein - NCBI
complement component C6 precursor [Mus musculus] complement component C6 precursor [Mus musculus]. gi,161086891,ref,NP_057913.2 ... Complement and coagulation cascades Complement and coagulation cascadesThe complement system is a proteolytic cascade in blood ... C6) in mice. [Immunogenetics. 1984] Genetic polymorphism of the sixth component of complement (C6) in mice.. Hayakawa JI, ... Genetically determined molecular weight differences in murine complement component C6.. Orren A, Preece CJ, Dowdle EB. Eur J ...
Complement C6 Antibodies: Novus Biologicals
Browse our Complement C6 Antibody catalog backed by our Guarantee+. ... Alternate Names for Complement C6 Antibodies. anti-Complement C6 antibody, anti-C6 antibody, anti-complement component 6 ... Complement C6 Antibodies. We offer Complement C6 Antibodies for use in common research applications: ELISA, Flow Cytometry, ... Choose from our Complement C6 polyclonal antibodies and browse our Complement C6 monoclonal antibody catalog. ...
Complement C6 Antibody
Complement C6 Polyclonal Antibody from Invitrogen for Western Blot and Immunohistochemistry (Frozen) applications. This ... Cite Complement C6 Polyclonal Antibody. The following antibody was used in this experiment: Complement C6 Polyclonal Antibody ... C6 is a component of complement cascade. It is part of the membrane attack complex which can insert into the cell membrane and ... PA1-40290 detects C6 from mouse samples.. PA1-40290 has been successfully used in immunohistochemistry (frozen) and Western ...
C6 - Complement component C6 precursor - Homo sapiens (Human) - C6 gene & protein
"Structural homology of complement protein C6 with other channel-forming proteins of complement.". Chakravarti D.N., Chakravarti ... sp,P13671,CO6_HUMAN Complement component C6 OS=Homo sapiens OX=9606 GN=C6 PE=1 SV=3 ... Complement component C6Add BLAST. 913. Amino acid modifications. Feature key. Position(s). DescriptionActions. Graphical view. ... Belongs to the complement C6/C7/C8/C9 family.Curated. Keywords - Domaini. EGF-like domain, Repeat, Signal, Sushi. Phylogenomic ...
JCI - Molecular basis of subtotal complement C6 deficiency. A carboxy-terminally truncated but functionally active C6.
Individuals with subtotal complement C6 deficiency possess a C6 molecule that is 14% shorter than normal C6 and present in low ... Molecular basis of subtotal complement C6 deficiency. A carboxy-terminally truncated but functionally active C6.. ... Molecular basis of subtotal complement C6 deficiency. A carboxy-terminally truncated but functionally active C6.. ... to this part of C6. Interestingly, all three subjects were probably heterozygous for both subtotal C6 and complete C6 ...
Complement C6 - NB120-11899 | acris-antibodies.com
C6 is a component of complement cascade. It is part of the membrane attack complex which can insert into the cell membrane and ... Product Description for Complement C6. Rabbit anti Mouse Complement C6.. Presentation: Purified. Product is tested for Frozen ... Recommended Secondary Antibodies for Complement C6 (9 products). Catalog No.. Host. Clone/Iso.. Pres.. React.. Applications. ... C6 is a component of complement cascade. It is part of the membrane attack complex which can insert into the cell membrane and ...
Complement component C5 and C6 mutation screening indicated in meningococcal disease in South Africa
4. Wurzner R, Orren A, Potter P, et al Functionally active complement proteins C6 and C7 detected in C6- and C7-deficient ... lack of functional C6 - distinct from patients with subtotal C6 deficiency (C6SD), where a small amount of C6 activity remains. ... We strongly recommend diagnostic testing for complement C5 and C6 deficiency in the routine work-up of all MD cases in South ... Molecular defects leading to human complement component C6 deficiency in an African-American family. Clin Exp Immunol 1998;111: ...
Influence of the terminal complement-complex on reperfusion injury, no-reflow and arrhythmias: a comparison between C6...
We investigated the influence of the C5b-9 complement complex ... The complement system has been suggested to play a role in ... Complement Activation*. Complement C6 / deficiency*. Complement Membrane Attack Complex / analysis*. Electrocardiography. ... C6 deficiency was confirmed by the complement titration test and immunohistology. The triphenyl tetrazolium chloride method was ... METHODS: Twenty-eight C6-competent rabbits and 18 rabbits with congenital C6 deficiency were subjected to either 30 min or 2 h ...
Anti-Complement C6 Antibody | Mouse anti-Human Monoclonal | LSBio
Complement C6 antibody LS-C123308 is an unconjugated mouse monoclonal antibody to human Complement C6. Validated for ELISA, ... Complement C6 antibody LS-C123308 is an unconjugated mouse monoclonal antibody to human Complement C6. Validated for ELISA, ... Complement C6 antibody LS-C123308 is an unconjugated mouse monoclonal antibody to human Complement C6. Validated for ELISA, ... Complement C6 Antibody (C‑Terminus, clone 056B‑188.8.131.52) IHC‑plus™ LS‑B3294 ...
Complement C6 Antibody (aa221-270) for IHC, WB/Western LS-C291316
Complement C6 antibody LS-C291316 is an unconjugated rabbit polyclonal antibody to Complement C6 from human and rat. Validated ... C6 Antibody, Complement component C6 Antibody, Complement C6 Antibody, Complement component 6 Antibody ... Complement C6 antibody LS-C291316 is an unconjugated rabbit polyclonal antibody to Complement C6 from human and rat. Validated ... Complement C6 antibody LS-C291316 is an unconjugated rabbit polyclonal antibody to Complement C6 from human and rat. Validated ...
Complement C6 | The Doctors Laboratory
Pig Complement component C6(C6) EL... - E07C1266-192T B-Gene - Anti...
C6) ELISA kit From B-Gene - A sandwich ELISA for quantitative measurement of Porcine Complement component C6(C6) in samples ... Pig Complement component C6(C6) ELISA kit. Supplier B-Gene · Catalog number: E07C1266-192T Price. 1270.00EUR. Size. 192 tests ... A sandwich ELISA for quantitative measurement of Porcine Complement component C6(C6) in samples from blood, plasma, serum, cell ...
Structural similarities between C6 and C7 of human complement<...
title = "Structural similarities between C6 and C7 of human complement",. abstract = "A new method for the isolation of C6 and ... Structural similarities between C6 and C7 of human complement. / Podack, E. R.; Kolb, W. P.; Esser, A. F.; Muller-Eberhard, H. ... Structural similarities between C6 and C7 of human complement. Journal of Immunology. 1979 Jan 1;123(3):1071-1077. ... Podack, ER, Kolb, WP, Esser, AF & Muller-Eberhard, HJ 1979, Structural similarities between C6 and C7 of human complement, ...
UBX : Summary for Unity Biotechnology, Inc. - Yahoo Finance
C6 cell death gene ontology
C6 Mouse SNP Summary
Low-density lipoprotein (LDL) receptor class A, conserved site (IPR023415) | InterPro | EMBL-EBI
Complement components C6, C7, C8 and C9. They contain each one LDLRA domain. ... Complement factor I, which is responsible for cleaving the alpha-chains of C4b and C3b. It consists of a FIMAC domain (Factor I ... proteins C6/C7), a scavenger receptor-like domain, 2 copies of LDLRA and a C-terminal serine protease domain. ...
PROSITE: PDOC00021 (documentation file)
Aldo-keto Reductase 1C4/AKR1C4 Antibody (2C11) [DyLight 350] (H00001109-M01UV): Novus Biologicals
Table], What is the prevalence of primary immunodeficiency in children and young people with meningococcal disease? - Bacterial...
Complement component C6 deficiency and susceptibility to Neisseria meningitidis infections. South African Medical Journal Suid- ... Complement deficiencies in patients over ten years old with meningococcal disease due to uncommon serogroups: Comment. ... Hereditary complement deficiency in survivors of meningococcal disease: high prevalence of C7/C8 deficiency in Sephardic ( ... Low prevalence of complement deficiencies among patients with meningococcal disease in Norway. Scandinavian Journal of ...
PRF1 - Perforin-1 precursor - Homo sapiens (Human) - PRF1 gene & protein
Complement membrane attack complex - Wikipedia
Another complement protein, C6, binds to C5b. The C5bC6 complex is bound by C7. This junction alters the configuration of the ... MAC is composed of a complex of four complement proteins (C5b, C6, C7, and C8) that bind to the outer surface of the plasma ... The MAC is composed of the complement components C5b, C6, C7, C8 and several C9 molecules. A number of proteins participate in ... ISBN 978-0-323-54943-1. Media related to Complement membrane attack complex at Wikimedia Commons Complement+Membrane+Attack+ ...
Postmenopausal estrogen and progestin effects on the serum proteome | Genome Medicine | Full Text
Packaging systems for human recombinant adenovirus to be used in gene therapy - FALLAUX FRITS JACOBUS
These cell lines express E1A and E1B proteins, are stable (e.g., PER.C6™ for more than 57 passages), and complement E1- ... 8. The method according to claim 1, wherein said complementing cell is a PER.C6 cell such as deposited under number ECACC ... We were able to establish seven clonal cell lines, which we called PER.C1, PER.C3, PER.C4, PER.C5, PER.C6™, PER.C8, and PER.C9 ... These cell lines were designated PER.C1, PER.C3, PER.C4, PER.C5, PER.C6™, PER.C8, and PER.C9. PER denotes PGK-E1-Retinoblasts. ...
Applying Public Health Strategies to Primary Immunodeficiency Diseases: A Potential Approach to Genetic Disorders
Terminal complement protein (C6-8) deficiencies are associated with severe infections with Neisseria meningitidis and N. ... Complement System Defects Defects in the complement system occur less frequently than other PI diseases. They are associated ... The most common defect, C2 deficiency, is an autosomal-recessive inherited defect in the gene for the complement protein C2. ... complement a set of serum proteins that binds antigen-antibody complexes to kill microorganisms ...
Formation of High-Affinity C5 Convertases of the Alternative Pathway of Complement | The Journal of Immunology
Complement proteins, C3 (19, 20), factor B (21), factor D (22), and C6 (23) were all purified from normal human plasma as ... Biochemical characterization of the sixth component (C6) of human complement. Biochemstry 21: 294. ... The binding of human complement proteins C5, factor B, BIH and properdin to complement fragment C3b on zymosan. Biochem. J. 199 ... A covalent dimer of complement C4.b serves as a subunit of a novel C5 convertase that involves no C3 derivatives. J. Immunol. ...
Anti-C7 antibody (ab231047) | Abcam
Recombinant Human C9 protein (ab114199) | Abcam
KEGG PATHWAY: hsa05020
Role of Complement in a Rat Model of Paclitaxel-Induced Peripheral Neuropathy | The Journal of Immunology
Characterization of complement C6 deficiency in a PVG/c rat strain. Clin. Exp. Immunol. 109: 387-396. ... Thus, complement KO rats should be superior to complement KO mice for studying the role of complement in human pathological ... Although complement has been implicated in other pain models, the potential role of complement in CIPN was not clear. It has ... We then assessed complement activation by detecting the deposition of complement activation products C3b/iC3b on the PC12 cells ...
ProteinsDeficiencyPathwayMembraneCascadeAntibodiesInhibitionGeneTerminal complement-complexConvertaseFragmentsBindsPathways of the complement systemInhibitorDeficienciesSusceptibilityOpsonizationRole of complementSystemImmuneClassical and alternative complement signalinPrecursorInflammationGeneticClinicalComposed of the complement componentsMonoclonal antibodyPreclinical and Phase 1 studiesMolecularELISAInhibitorsAntibody to complementCDNASequenceReceptorsPhagocytesPolyclonalComplexActivateDiseasesBacteriaHumanRegulatorsGenesBind
- We now show that this dysmorphic C6 is bactericidally active and lacks an epitope that was mapped to the most carboxy-terminal part of C6 using C6 cDNA fragments expressed as fusion proteins in the pUEX expression system. (jci.org)
- C6 and C7 prepared by this method are hemolytically fully active, homogeneous proteins obtained in 25% yield. (elsevier.com)
- A comparison of the properties of isolated C6 and C7 gave the following results: The amino acid composition of the two proteins is very similar. (elsevier.com)
- It consists of a FIMAC domain (Factor I/MAC proteins C6/C7), a scavenger receptor-like domain, 2 copies of LDLRA and a C-terminal serine protease domain. (ebi.ac.uk)
- The membrane attack complex (MAC) or terminal complement complex (TCC) is a complex of proteins typically formed on the surface of pathogen cell membranes as a result of the activation of the host's complement system, and as such is an effector of the immune system. (wikipedia.org)
- MAC is composed of a complex of four complement proteins (C5b, C6, C7, and C8) that bind to the outer surface of the plasma membrane, and many copies of a fifth protein (C9) that hook up to one another, forming a ring in the membrane. (wikipedia.org)
- C6 inhibition prevents the formation of the destructive membrane attack complex (MAC), a complex of terminal complement proteins that mediate cellular injury following complement activation, and has the potential to treat a variety of central nervous system disorders. (businesswire.com)
- The complement system as understood today is a multimolecular system composed of more than 32 proteins and consisting of serum proteins, serosal proteins, and cell membrane receptors that bind to complement fragments. (medscape.com)
- The complement system consists of 7 serum and 9 membrane regulatory proteins, 1 serosal regulatory protein, and 8 cell membrane receptors that bind complement fragments. (medscape.com)
- The combination and activation of this range of complement proteins forms the membrane attack complex , which is able to insert into bacterial cell walls and causes cell lysis with ensuing death. (wikipedia.org)
- The membrane attack complex ( MAC ) or terminal complement complex ( TCC ) is a structure typically formed on the surface of pathogen cell membranes as a result of the activation of the host's complement system , and as such is one of the effector proteins of the immune system . (wikipedia.org)
- To date, most, if not all, of the serum proteins that constitute the complement system have been identified and their activities characterized. (springer.com)
- C6-C9 or serum proteins is not present. (mpbio.com)
- The complement system consists of a number of small proteins found in the blood, in general synthesized by the liver , and normally circulating as inactive precursors ( pro-proteins ). (wikipedia.org)
- Over 30 proteins and protein fragments make up the complement system, including serum proteins , and cell membrane receptors . (wikipedia.org)
- Most of the proteins and glycoproteins that constitute the complement system are synthesized by hepatocytes . (wikipedia.org)
- a biochemical process involving the C1 to C9 complement proteins in which one protein interacts with another in a specific sequence called a complement pathway. (thefreedictionary.com)
- Complement comprises 25 to 30 discrete proteins, labeled numerically as C1 to C9, and by letters, i.e. (thefreedictionary.com)
- The complement is composed of a complex proteolytic cascade of more than 40 soluble and membranous proteins that interact in a various range of function. (frontiersin.org)
- Inactive circulating complement proteins can be rapidly activated in a cascade fashion by three different activation pathways leading to a common terminal pathway. (frontiersin.org)
- The Classical pathway of activation of the complement system is a group of blood proteins that mediate the specific antibody response. (wikipathways.org)
- 4 Under normal pregnancy conditions, the fetus is protected from maternal immune responses through an array of mechanisms, including trophoblast expression of complement regulatory proteins that inhibit complement at different steps of the activation cascade. (ahajournals.org)
- Encompassing more than 30 proteins, the complement system is an ancient part of innate immunity ( 1 ), and it was originally described for its ability to complement phagocytes and antibodies in microbial killing ( 2 ). (frontiersin.org)
- Results of studies published since 2002 reveal that T cells and antigen-presenting cells (APCs) produce complement proteins. (springer.com)
- Most complement proteins are zymogens which are proteases that become activated when cleaved. (davidson.edu)
- C3b is a membrane associated protein which binds covalently to other complement proteins already on the pathogen surface. (davidson.edu)
- The complex of complement proteins on the cell surface after C3b binds forms the C5 convertase. (davidson.edu)
- After cleavage C5b interacts nonenzymatically with other complement proteins to form the MAC. (davidson.edu)
- The binding domain of C5b for other complement proteins such as C6 and C7 has been recently characterized and is homologous to a complement binding domain on C3 and C4. (davidson.edu)
- Other complement proteins such as C6 and C7 interact with C5 at the active site C345C in forming the MAC. (davidson.edu)
- Genetic abnormalities in the complement proteins or regulators of the cascade in the circulation or on cell surfaces appear to predipose individual patients to these disorders. (renalandurologynews.com)
- Genetic predisposition to MPGN is linked to mutations in factor H or other complement proteins such as C3 that lead to abnormal activation of the alternative pathway of complement. (renalandurologynews.com)
- Fragments resulting from proteolytic cleavage of complement proteins are designated with lower-case-letter suffixes, e.g. (thefreedictionary.com)
- Complement is the term used to describe a group of serum proteins that are critically important in our defense against infection. (primaryimmune.org)
- The complement system consists of more than 30 proteins, present in blood and tissues, as well as other proteins anchored on the surfaces of cells. (primaryimmune.org)
- Complement proteins in the circulation are not activated until triggered by an encounter with a bacterial cell, a virus, an immune complex, damaged tissue or other substance not usually present in the body. (primaryimmune.org)
- Both FH and FHL-1 (which is a truncated version of FH that results from an alternative splice site in the FH transcript [ 71 ]) are serum proteins that negatively regulate the alternative pathway of complement. (asm.org)
- A series of reactions that result in formation of a pore, composed of complement proteins C5b, C6, C7, C8, and C9, in the membrane of a pathogenic cell. (bioontology.org)
- Without these GPI anchor proteins, such as CD59, terminal complement molecules would bind to the target cell and lyse the cell through the membrane attack complex (MAC). (davidson.edu)
- There are many molecules and proteins that are used in collaboration to activate different pathways of the complement system. (davidson.edu)
- The MAC is formed by sequential assembly of soluble complement proteins C5b, C6, C7, C8 and C9, but little is known about the rate-limiting steps in this process. (epfl.ch)
- The decreased expression of complement regulatory proteins (CRP) seems to play an important role in RA activity, and is associated with worsening of the clinical symptoms. (scielo.br)
- rheumatoid arthritis, complement system proteins, complement activation. (scielo.br)
- The complement system (CS) is composed of receptors and regulators bound to the cell membrane and different plasma proteins that interact with cells and mediators of the immune system ( Figure 1 ). (scielo.br)
- The complement system is an enzyme cascade that is a collection of blood and cell surface proteins to help the abilities of antibodies to clear pathogens from an organism. (sinobiological.com)
- The complement system that comprises 30 different proteins, including serum proteins, serosal proteins, and cell membrane receptors is an important part of the innate immune system. (sinobiological.com)
- Some complement proteins bind to immunoglobulins or to membrane components of cells. (sinobiological.com)
- Others are proenzymes that, when activated, cleave one or more other complement proteins and initiate an amplifying cascade of further cleavages. (sinobiological.com)
- People with C6 deficiency are prone to bacterial infection. (thermofisher.com)
- Molecular basis of subtotal complement C6 deficiency. (jci.org)
- Individuals with subtotal complement C6 deficiency possess a C6 molecule that is 14% shorter than normal C6 and present in low but detectable concentrations (1-2% of the normal mean). (jci.org)
- Interestingly, all three subjects were probably heterozygous for both subtotal C6 and complete C6 deficiency. (jci.org)
- Human deficiency of the fifth complement component (C5D) or complete absence of the sixth component (C6Q0) leads to increased risk of MD, which is often recurrent. (scielo.org.za)
- We strongly recommend diagnostic testing for complement C5 and C6 deficiency in the routine work-up of all MD cases in South Africa. (scielo.org.za)
- 3 C6Q0 subjects have a complete lack of functional C6 - distinct from patients with subtotal C6 deficiency (C6SD), where a small amount of C6 activity remains. (scielo.org.za)
- METHODS: Twenty-eight C6-competent rabbits and 18 rabbits with congenital C6 deficiency were subjected to either 30 min or 2 h of coronary artery occlusion followed by reperfusion. (biomedsearch.com)
- C6 deficiency was confirmed by the complement titration test and immunohistology. (biomedsearch.com)
- Prevalence of congenital or acquired complement deficiency in patients with sporadic meningocococcal disease. (nih.gov)
- Complement deficiency predisposes for meningitis due to nongroupable meningococci and Neisseria-related bacteria. (nih.gov)
- Systemic meningococcal infections in patients with acquired complement deficiency. (nih.gov)
- Mutations in this gene cause complement component 5 deficiency, a disease where patients show a propensity for severe recurrent infections. (wikipedia.org)
- Defects in C7 are a cause of complement component 7 deficiency (C7D) [MIM:610102]. (abcam.com)
- Defects in C9 are a cause of complement component 9 deficiency (C9D) [MIM:613825]. (abcam.com)
- In addition, Alexion has two highly innovative enzyme replacement therapies for patients with life- threatening and ultra-rare metabolic disorders, hypophosphatasia (HPP) and lysosomal acid lipase deficiency (LAL-D). As the leader in complement biology for over 20 years, Alexion focuses its research efforts on novel molecules and targets in the complement cascade, and its development efforts on the core therapeutic areas of hematology, nephrology, neurology, and metabolic disorders. (businesswire.com)
- 71 Complement component 6 deficiency: A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis. (malacards.org)
- C6 Deficiency, also known as complement component 6 deficiency , is related to meningitis and oculocutaneous albinism , and has symptoms including abnormality of metabolism/homeostasis , complement deficiency and recurrent meningococcal disease . (malacards.org)
- An important gene associated with C6 Deficiency is C6 (Complement C6), and among its related pathways/superpathways are Innate Immune System and IL-17 Family Signaling Pathways . (malacards.org)
- When biochemical analysis reveals the causal abnormality of the complement deficiency (CD), molecular mechanisms remains frequently undetermined. (frontiersin.org)
- Here, using direct sequencing analysis of the coding region we report the pathogenic variants spectrum that underlie the total or subtotal complement deficiency in 212 patients. (frontiersin.org)
- Results from this large genetic investigation provide evidence of a restricted number of molecular mechanisms leading to complement deficiency and describe the clinical potential adverse events of anti-complement therapy. (frontiersin.org)
- Restricted number of molecular mechanisms leading to complement deficiency. (frontiersin.org)
- Severe or multiple infections-mainly meningococcal infections-or severe autoimmune diseases (AID) in particular with childhood onset are the main features associated with complement deficiency (CD) ( 6 - 10 ). (frontiersin.org)
- The different roles of the complement system are illustrated by complement deficiencies, which increase susceptibility to bacterial infections and/or autoimmune diseases, for example, C3 deficiency is generally associated with increased risk of bacterial infections, while deficiency of late complement component C5-C9 is associated with Neisseria infections in particular ( 4 ). (frontiersin.org)
- There is wide variation of infections associated with complement deficiency depending on which complement protein and activation pathway is affected(Skattum et al 2011). (pediatriconcall.com)
- Deficiency of C5, C6, C7 and C8 has less than 1000 reported cases of Neisserial meningitis and sepsis, while C9 deficiencies is more common in Japanese populations with prevalence around 1:1000 ( Fukumori et al 1989)(Nagata et al 1989). (pediatriconcall.com)
- Complement deficiency or blockade attenuates T cell-mediated autoimmunity and delays allograft rejection in mice. (springer.com)
- Cephalosporins are often used for treatment of N meningitidis infection in patients with complement deficiency. (medscape.com)
- Skattum L, van Deuren M, van der Poll T, Truedsson L. Complement deficiency states and associated infections. (medscape.com)
- Molecular basis of complement factor I deficiency in Tunisian atypical hemolytic and uremic syndrome Patients. (medscape.com)
- Arnold DF, Roberts AG, Thomas A, Ferry B, Morgan BP, Chapel H. A novel mutation in a patient with a deficiency of the eighth component of complement associated with recurrent meningococcal meningitis. (medscape.com)
- C5 Complement Deficiency in a Saudi Family, Molecular Characterization of Mutation and Literature Review. (medscape.com)
- Invasive meningococcal disease in three siblings with hereditary deficiency of the 8(th) component of complement: evidence for the importance of an early diagnosis. (medscape.com)
- Complement C4 deficiency--a plausible risk factor for non-tuberculous mycobacteria (NTM) infection in apparently immunocompetent patients. (medscape.com)
- The Brief Case: Meningococcemia Leading to a Diagnosis of Complement Deficiency in a 23-Month-Old. (medscape.com)
- aHUS is a chronic, ultra-rare, and life-threatening disease in which a lifelong and permanent genetic deficiency in one or more complement regulatory genes causes chronic uncontrolled complement activation, resulting in complement-mediated thrombotic microangiopathy (TMA), the formation of blood clots in small blood vessels throughout the body. (alxn.com)
- In three models of nonproteinuric primary or secondary tubulointerstitial injury, namely unilateral ureteral obstruction (UUO), cyclosporine A nephropathy, and streptozotocin-induced diabetic nephropathy, genetic C6 deficiency did not affect the severity of the diseases ( 15 ). (asnjournals.org)
- RATIONALE: Late complement deficiency increases susceptibility to meningococcal disease and recurrent infections. (bvsalud.org)
- In Korea, 5 case reports have described meningococcal disease with complement deficiency. (bvsalud.org)
- However, C6 deficiency has not been described previously. (bvsalud.org)
- DIAGNOSIS: He was diagnosed with meningococcal meningitis with C6 deficiency. (bvsalud.org)
- LESSONS: Screening for complement deficiency is necessary in patients with a history of recurrent meningococcal infections to provide appropriate care and prevent recurrent infections. (bvsalud.org)
- Defects in C7 are a cause of complement component 7 deficiency (C7D). (sinobiological.com)
- A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis. (abcam.com)
- Once activated by one of the three main pathways (i.e., the classical, lectin, or alternative pathway), C3, the central component of the complement system, is cleaved by C3 convertases to release C3a and to deposit C3b on the surface of the complement activation site. (jimmunol.org)
- Depending on the nature of complement activators, the classic pathway, the alternative pathway, or the more recently discovered lectin pathway is activated predominantly to produce C3 convertase. (medscape.com)
- Binding of factor H to C3b increases its inactivation by factor I. Properdin stabilizes it, preventing its inactivation by factors H and I. The alternate pathway does not result in a truly nonspecific activation of complement because it requires specific types of compounds for activation. (medscape.com)
- Properdin is a serum glycoprotein that up-regulates the alternative pathway of complement by stabilizing the C3b-Bb complex. (embl.de)
- Three biochemical pathways activate the complement system: the classical complement pathway , the alternative complement pathway , and the lectin pathway . (wikipedia.org)
- The classical complement pathway typically requires antigen-antibody complexes for activation (specific immune response), whereas the alternative pathway can be activated by spontaneous C3 hydrolysis, foreign material, pathogens, or damaged cells. (wikipedia.org)
- the sequence of reactions, each being the catalyst for the next, that leads to the terminal complement pathway and cell lysis. (thefreedictionary.com)
- Complement activation on keratinocytes with persistent intracellular bacteria was found with sera deficient/depleted of the complement components C1q, Mannan-binding lectin, or complement factor B, demonstrating involvement of more than one complement activation pathway. (frontiersin.org)
- In a manner that requires activation of the alternative complement pathway, passive transfer of antibodies to mouse MPO (anti-MPO) induces a mouse model of ANCA NCGN that closely mimics human disease. (asnjournals.org)
- 3 Alternative complement pathway activation is pivotal in the pathogenesis of anti-MPO NCGN in mice. (asnjournals.org)
- 4 - 6 The relevance of alternative complement pathway activation to human AAV is supported by immunohistochemical demonstration of alternative complement pathway components at sites of AAV 7 , 8 and by correlation of plasma alternative complement pathway activation fragments with AAV disease activity. (asnjournals.org)
- 5 , 6 ANCA-activated neutrophils activate the alternative complement pathway. (asnjournals.org)
- Human neutrophils activated by human ANCA release factors that activate the alternative complement pathway. (asnjournals.org)
- The immune cell-derived, alternative pathway complement components activate spontaneously, yielding local, but not systemic, production of C3a and C5a. (springer.com)
- The alternative pathway of the complement system is an innate component of the immune system 's natural defense against infections. (wikipedia.org)
- The alternative pathway is one of three complement pathways that opsonize and kill pathogens. (wikipedia.org)
- After the creation of C5 convertase (either as (C3b) 2 BbP or C4b2a3b from the classical pathway), the complement system follows the same path regardless of the means of activation (alternative, classical, or lectin). (wikipedia.org)
- The primary cases are generally the consequence of perturbations in the regulation of the alternative pathway of complement with constitutive activation of this cascade. (renalandurologynews.com)
- MPGN is one of a group of rare disorders that includes atypical hemolylic uremic syndrome (HUS) and macular degeneration that are characterized by abnormal activation of the alternative pathway of complement. (renalandurologynews.com)
- Mice deficient for classical complement pathway (CCP) factor C4 are resistant to antibody and complement mediated experimental autoimmune myasthenia gravis (EAMG). (springer.com)
- Furthermore, CCP inhibition preserves alternative complement pathway activation, which is required for host defense against microorganisms. (springer.com)
- Complement activation is a major effector pathway in NMO. (springermedizin.de)
- The Alternative Pathway (AP) is initiated by fragments of the complement component C3. (primaryimmune.org)
- This time-limitation is another control mechanism for the complement pathway. (primaryimmune.org)
- The Terminal Pathway (TP) is the final set of steps in the complement activation process that forms a membrane lesion or hole (membrane attack complex or MAC) that kills susceptible bacteria or other cells that activate complement on their surfaces. (primaryimmune.org)
- While all three MAbs elicited similar C1q-dependent C4b deposition on live bacteria (classical complement pathway), only those antibodies that inhibited binding of fH to fHbp (JAR 3 and JAR 5) had bactericidal activity with human complement. (asm.org)
- In the alternate pathway complement C3 undergoes spontaneous cleavage resulting in complement B binding to C3b. (sigmaaldrich.com)
- It has been suggested by several labs, including our own, that B. burgdorferi binds FH/FHL-1 on its surface to inhibit the activation of the alternative pathway of complement and prevent its destruction during the earliest stages of mammalian infection ( 4 , 10 , 40 , 48 , 71 ). (asm.org)
- Pathway analysis implicated the complement and coagulation cascade. (deepdyve.com)
- The complement system has four major function, including lysis of infectious organisms, activation of inflammation, opsonization and immune clearance.There are three different complement pathways, the classical complement pathway, the alternative complement pathway, and the mannose-binding lectin pathway. (sinobiological.com)
- The classic complement pathway is triggered when antibody-antigen complex interact with C1-complex, which consists of C1q, two molecules of C1r, and two molecules of C1s. (sinobiological.com)
- The alternative complement pathway begins with the activation of C3 and requires factor B and factor D. All three pathways merge at C3, which is then converted into C3a and C3b. (sinobiological.com)
- The membrane attack complex is initiated when the complement protein C5 convertase cleaves C5 into C5a and C5b. (wikipedia.org)
- C5b forms the first part of the complement membrane attack complex. (wikipedia.org)
- The C5b macromolecular cleavage product can form a complex with the C6 complement component, and this complex is the basis for formation of the membrane attack complex, which includes additional complement components. (wikipedia.org)
- The complement system is a part of the immune system that enhances (complements) the ability of antibodies and phagocytic cells to clear microbes and damaged cells from an organism, promotes inflammation, and attacks the pathogen's cell membrane . (wikipedia.org)
- The end result of this complement activation or complement fixation cascade is stimulation of phagocytes to clear foreign and damaged material, inflammation to attract additional phagocytes, and activation of the cell-killing membrane attack complex . (wikipedia.org)
- Following the complement cascade, C5b with C6-C9 forms the "membrane attack complex" (MAC), capable of lysing infected cells and pathogens [ 17 , 18 ]. (hindawi.com)
- C5b with C6, C7, C8, and C9 form the membrane attack complex that initiates cell lysis. (thefreedictionary.com)
- The cleavage product C5B joins C6, C7, C8 and multiple copies of C9 to form the Membrane Attack Complex, which forms a channel for water to flood into the target cell, leading to osmotic lysis. (wikipathways.org)
- mmFATP3, -5, and -6 did not complement the transport function even though each was localized to the yeast plasma membrane. (elsevier.com)
- 6 , 12 Cleavage of C5 also releases C5b, which joins with C6 to initiate the membrane attack complex (MAC). (asnjournals.org)
- C5b binds sequentially to C6, C7, C8 and then to multiple molecules of C9 to form membrane attack complex . (wikipedia.org)
- The hydrophobic anchor of C7 in the membrane has a high affinity for phospholipids which ensures strong binding while other complement constituents of the MAC are brought together. (davidson.edu)
- These complement components form the final membrane attack complex (MAC). (thefreedictionary.com)
- A complex of complements C5b,C6, C7, and C8 mediates the polymerization of up to eighteen C9 molecules into a tube-like membrane attack complex that is inserted into the plasma membrane of an unwanted organism such as of gram-negative bacteria and viral infected cells. (sigmaaldrich.com)
- Activation of C5 by a C5 convertase initiates the spontaneous assembly of the late complement components, C5-C9, into the membrane attack complex. (reportbuyer.com)
- Ultimately, it is thought that the inactivation of C3b to iC3b inhibits the deposition of terminal complement components, which then prevents the formation of the bactericidal membrane attack complex. (asm.org)
- CD59 is a membrane-bound protein that is present in many types of cells and binds to homologous complement factors C8 and C9 of the terminal complement system. (davidson.edu)
- The CD59 protein is a complement system regulator that can inhibit the formation of the membrane attack complex (MAC). (davidson.edu)
- Complement component 7 serves as a membrane anchor. (sinobiological.com)
- The complement system is a proteolytic cascade in blood plasma and a mediator of innate immunity, a nonspecific defense mechanism against pathogens. (nih.gov)
- C6 is a component of complement cascade. (thermofisher.com)
- Terminal complement components comprise the final 5 components of the complement cascade (C5 to C9). (scielo.org.za)
- Cleavage of C5 by C5 convertase is the last enzymatic step in the complement activation cascade leading to the formation of the cytolytic proteolytically activated form of C5 (C5b)-9 complex. (jimmunol.org)
- Cleavage of C5, the fifth component of complement, by the serine protease C5 convertase is an important enzymatic reaction that occurs during the activation cascade. (jimmunol.org)
- An end product of the activated complement cascade, MAC has been shown to play a role in neurodegeneration. (businesswire.com)
- The complement system functions as an interactive sequence, with one reaction leading to another in the form of a cascade. (medscape.com)
- The complement system is a biochemical cascade that helps, or complements, the ability of antibodies to clear pathogens from an organism. (wikipathways.org)
- The complement cascade is activated in normal pregnancy, and excessive complement activation propagates the systemic inflammatory response in severe preeclampsia. (ahajournals.org)
- The complement system is a complex cascade involving proteolytic cleavage of serum glycoproteins often activated by cell receptors. (sigmaaldrich.com)
- Ischaemic infarction may also cause initiation of the complement cascade. (sigmaaldrich.com)
- 0.05) for the terminal steps of the complement cascade (complement component C6) and other bacteriolytic processes (lysozyme type II) potentially underlying increased disease susceptibility after pathogen challenge. (nih.gov)
- C6 knock-out mice are protected from thrombophilia mediated by antiphospholipid antibodies. (nih.gov)
- Our Complement C6 Antibodies can be used in a variety of model species: Human, Mouse. (novusbio.com)
- Choose from our Complement C6 polyclonal antibodies and browse our Complement C6 monoclonal antibody catalog. (novusbio.com)
- The complement system can be recruited and brought into action by antibodies generated by the adaptive immune system . (wikipedia.org)
- In the early 20th century, this controversy was resolved when it became understood that complement can act in combination with specific antibodies, or on its own in a non-specific way. (wikipedia.org)
- In contrast, in a discordant combination complement activation by preexisting antibodies in the recipient or by the vascular endothelium of the donor organ causes HAR of the xenograft . (springer.com)
- Peerschke EI, Yin W, Alpert DR, Roubey RA, Salmon JE, Ghebrehiwet B. Serum complement activation on heterologous platelets is associated with arterial thrombosis in patients with systemic lupus erythematosus and antiphospholipid antibodies. (medscape.com)
- Soliris ® (eculizumab) is being investigated in relapsing NMOSD, a life-threatening, ultra-rare, autoimmune neurological disorder, in which complement activation by antibodies against aquaporin-4 on astrocyte cell surfaces causes damage in the central nervous system, including the spinal cord and optic nerve. (alxn.com)
- While some fHbp-specific monoclonal antibodies (MAbs) block binding of fH to fHbp, the stoichiometry of blocking in the presence of high serum concentrations of fH and its effect on complement-mediated bactericidal activity are unknown. (asm.org)
- In preclinical studies with mice, antibodies elicited by fHbp vaccines bound to the bacterial surface, activated complement-mediated bactericidal activity directly ( 8 , 16 , 17 , 33 , 47 ), and inhibited binding of fH to fHbp ( 8 , 30 , 47 ). (asm.org)
- Alexion has been a pioneer in complement biology since the development of Soliris, the world's first complement inhibitor, which has demonstrated the significant impact that C5 inhibition can have on several diseases caused by uncontrolled complement activation," said John Orloff, M.D., Executive Vice President and Head of Research & Development at Alexion. (businesswire.com)
- We believe that C6 inhibition has the potential to treat multiple neurodegenerative disorders, and we look forward to working with Alexion, a partner with a worldwide leading position in complement biology, to realize the possibilities of this approach. (businesswire.com)
- C6 inhibition prevents the formation of the MAC following complement activation which may allow treatment of a variety of central nervous system disorders. (businesswire.com)
- Alexion is the global leader in complement inhibition and has developed and commercializes the first and only approved complement inhibitor to treat patients with paroxysmal nocturnal hemoglobinuria (PNH), atypical hemolytic uremic syndrome (aHUS), and anti-acetylcholine receptor (AchR) antibody-positive generalized myasthenia gravis (gMG). (businesswire.com)
- Complement Pharma is a biotech company focusing on complement inhibition to modify neurodegenerative disease progression. (businesswire.com)
- The Company was founded in 2017 by Prof. Frank Baas and Robert Jan Lamers with the goal of translating the opportunities of C6 complement inhibition into clinical products for patients with acute and chronic neurodegenerative diseases. (businesswire.com)
- Complement inhibition is an intriguing treatment option for patients with severe preeclampsia. (ahajournals.org)
- Terminal complement inhibition decreases early acute humoral rejection in sensitized renal transplant recipients. (springer.com)
- Medof ME, Kinoshita T, Nussenzweig V. Inhibition of complement activation on the surface of cells after incorporation of decay-accelerating factor (DAF) into their membranes. (springer.com)
- Therefore, CCP inhibition might constitute a specific treatment approach for not only myasthenia gravis but also other complement mediated autoimmune diseases. (springer.com)
- Biesecker, G. and Gomez, C.M. (1989) Inhibition of acute passive transfer experimental autoimmune myasthenia gravis with Fab antibody to complement C6. (springer.com)
- Inhibition of binding of fH to the bacterial surface would be expected to increase susceptibility of the bacteria to complement-mediated bacteriolysis. (asm.org)
- Conversely, inhibition of the complement regulatory protein Crry aggravated tubular damage ( 13 ). (asnjournals.org)
- See reference mRNA sequence for the C6 gene (NM_016704.2). (nih.gov)
- See 4 reference sequence protein isoforms for the C6 gene. (nih.gov)
- To determine the frequency of specific disease-associated C5 and C6 gene mutations in patients presenting with MD in the Western Cape. (scielo.org.za)
- The structural similarities of C6 and C7 suggest their evolution from a common ancestral gene. (elsevier.com)
- Complement component 5 is a protein that in humans is encoded by the C5 gene. (wikipedia.org)
- The protein encoded by this gene is the fifth component of complement, which plays an important role in inflammatory and cell killing processes. (fishersci.com)
- Complement C5 (C3 And PZP Like Alpha 2 Macroglobulin Domain Containing Protein 4 or C5) - Complement component 5 is a protein is encoded by the C5 gene. (reportbuyer.com)
- Replication-deficient human adenovirus type 5 (Ad5) can be produced to high titers in complementing cell lines, such as PER.C6, and is widely used as a vaccine and gene therapy vector. (asm.org)
- Recently, we identified the gene that encoded complement factor C5 as a quantitative trait gene that modifies the course of hepatic fibrosis ( 16 ). (asnjournals.org)
- Download the full report: https://www.reportbuyer.com/product/4368352 Summary Complement C5 (C3 And PZP Like Alpha 2 Macroglobulin Domain Containing Protein 4 or C5) - Complement component 5 is a protein is encoded by the C5 gene. (medindia.net)
- 2010) Complement factor 7 gene mutations in relation to meningococcal infection and clinical recurrence of meningococcal disease. (sinobiological.com)
- Influence of the terminal complement-complex on reperfusion injury, no-reflow and arrhythmias: a comparison between C6-competent and C6-deficient rabbits. (biomedsearch.com)
- Using fluorescence microscopy, we found that persistent SA, surviving intracellularly in keratinocytes, caused activation of the complement system with formation of the terminal complement complex (TCC) at the cell surface. (frontiersin.org)
- A fluid phase form of the MAC, called the Terminal Complement Complex (TCC) can be found in the circulation after complement activation occurs and makes a useful laboratory marker for complement activation. (primaryimmune.org)
- 2009) C7 is expressed on endothelial cells as a trap for the assembling terminal complement complex and may exert anti-inflammatory function. (sinobiological.com)
- In the first phase, a series of specific interactions leads to formation of intrinsic complement proteinase, termed C3 convertase. (medscape.com)
- The formation of a C3 convertase can also be prevented when a plasma protease called complement factor I cleaves C3b into its inactive form, iC3b. (wikipedia.org)
- Each of the three pathways of the complement system results in the formation of C3 convertase on the pathogen surface. (davidson.edu)
- The two activated C1s subunits are then able to catalyze the assembly of the C3 convertase (complement C4b2a) from complements C2 and C4. (sigmaaldrich.com)
- Both fragments play a vital role in killing microorganisms and in eliminating targets of complement action. (jimmunol.org)
- Biologically active fragments and protein complexes derived from complement activate and damage the endothelial cells of the graft, and recruit and activate recipient blood cells, resulting in interstitial edema, hemorrhage, and thrombosis, which ultimately destroy the graft within minutes or a few hours of revascularization. (springer.com)
- Locally produced complement fragments C5a and C3a provide both costimulatory and survival signals to naive CD4+ T cells. (springer.com)
- Complement activation also results in the formation of many biologically active complement fragments that act as anaphylatoxins, opsonins, or chemotactic factors. (thefreedictionary.com)
- Freshly activated C5b binds to C6 to form a C5b-6 complex, then to C7 forming the C5b-6-7 complex. (wikipedia.org)
- Another complement protein, C6, binds to C5b. (wikipedia.org)
- CP010 is a humanized monoclonal antibody in preclinical development that binds to C6 in circulation to inhibit its function throughout the body by preventing MAC formation in both the periphery and the central nervous system. (businesswire.com)
- CP010 is a humanized monoclonal antibody that binds to C6 in circulation to inhibit its function throughout the body by preventing MAC formation. (foleyhoag.com)
- If the serum contains antibody, i.e. is positive, an antibody-antigen complex is formed which also binds (fixes) complement. (thefreedictionary.com)
-  Complement factor H preferentially binds to vertebrate cells (because of affinity for sialic acid residues), allowing preferential protection of host (as opposed to bacterial) cells from complement-mediated damage. (wikipedia.org)
- When an IgG1 anti-fHbp MAb binds to sparsely exposed fHbp on the bacterial surface, there appears to be insufficient complement activation for bacteriolysis unless fH binding also is inhibited. (asm.org)
Pathways of the complement system1
- All three pathways of the complement system (classical, lectin and alternative pathways) initiate the formation of MAC. (wikipedia.org)
- NEW HAVEN, Conn., & AMSTERDAM--( BUSINESS WIRE )--Alexion Pharmaceuticals, Inc. (NASDAQ:ALXN) and Complement Pharma today announced a partnership to co-develop the preclinical C6 complement inhibitor CP010 for neurodegenerative disorders. (businesswire.com)
- Foley Hoag LLP recently advised global biopharmaceutical company Alexion Pharmaceuticals, Inc. (NASDAQ:ALXN) in a partnership with Complement Pharma to co-develop a pre-clinical C6 complement inhibitor for neurodegenerative disorders. (foleyhoag.com)
- Alexion and Complement will collaborate on the preclinical and Phase 1 studies and manufacturing for C6 complement inhibitor, CP010. (foleyhoag.com)
- Complement lysis inhibitor and prototype domain in a new protein superfamily. (springer.com)
- The use of an inhibitor of the extracellular signal-regulated kinase (ERK) abrogated the complement-induced reduction in intracellular bacterial load. (frontiersin.org)
- Complement deficiencies in patients over ten years old with meningococcal disease due to uncommon serogroups: Comment. (nih.gov)
- Low prevalence of complement deficiencies among patients with meningococcal disease in Norway. (nih.gov)
- Prevalence of complement deficiencies in children with systemic meningococcal infections. (nih.gov)
- Thus, clinical indications for possible complete complement deficiencies include, among others, recurrent mild or serious bacterial infections as well as autoimmune diseases (AID). (frontiersin.org)
- Therefore, complement deficiencies disorders impair the immune system's ability to defend the body against foreign or abnormal cells that invade or attack it ( 3 - 5 ). (frontiersin.org)
- Some complement deficiencies caries risk of infection, other mainly associated with autoimmune diseases. (pediatriconcall.com)
- Some genetic and molecular defects has been identified to be associated complement deficiencies. (pediatriconcall.com)
- Complement deficiencies and associated genetic disorders and clinical manifestations is summarized in Table 1. (pediatriconcall.com)
- Table 1 - Complement deficiencies and clinical associations. (pediatriconcall.com)
- Grumach AS, Kirschfink M. Are complement deficiencies really rare? (medscape.com)
- Complement deficiencies in systemic lupus erythematosus. (medscape.com)
- Complement genetics, deficiencies, and disease associations. (medscape.com)
- There are deficiencies of each of the individual components of complement. (primaryimmune.org)
- Patients with complement deficiencies encounter clinical problems that depend on the role of the specific complement protein in normal function. (primaryimmune.org)
- Finally, the C6 status seems to influence the susceptibility to ventricular arrhythmias after coronary artery occlusion, independent of reperfusion. (biomedsearch.com)
- Factor H, a regulator of complement activity, is a major determinant of meningococcal disease susceptibility in UK Caucasian patients. (nih.gov)
- The complement system combats infections by detection of microbial intruders followed by lysis or opsonization to facilitate microbial clearance. (frontiersin.org)
- The complement system consists of a series of plasma protein interactions which mediate direct attack on pathogens (opsonization) and induce inflammation. (davidson.edu)
Role of complement5
- However, the role of complement in CIPN remains unclear. (jimmunol.org)
- Our findings demonstrate a previously unknown but pivotal role of complement in CIPN and suggest that complement may be a new target for the development of novel therapeutics to manage this painful disease. (jimmunol.org)
- Our understanding of the role of complement in many disorders continues to increase and many neurodegenerative diseases show activation of the complement system, even in early stages of disease," said Frank Baas, M.D., Ph.D., Chief Scientific Officer at Complement Pharma. (businesswire.com)
- Our aim is to evaluate the role of complement components in acute liver failure (ALF) caused by viral hepatitis, involving virus-induced ALF in human subjects using peripheral blood, samples of liver tissues, and ex vivo assays. (hindawi.com)
- Role of complement in the induction of immunological responses. (springer.com)
- OBJECTIVE: The complement system has been suggested to play a role in reperfusion injury which may result from an enhanced destruction of myocardial tissue or from an impairment of reflow. (biomedsearch.com)
- Complement component 5 is involved in the complement system. (wikipedia.org)
- Complete primary structure and functional characterization of the sixth component of the human complement system. (wikipedia.org)
- Complement, a key component of the innate immune system, has been associated with neuroinflammation, a potentially important trigger of some types of neuropathic pain. (jimmunol.org)
- Complement is an important part of the innate immune system, which plays a primary role in protecting the host from infections ( 6 ). (jimmunol.org)
- This collaboration provides an exciting opportunity to expand on our more than two decades of complement expertise to potentially treat additional diseases by targeting C6, a different component of the complement system. (businesswire.com)
- Reid K. B. M., The complement system , in: B. D. Hames and D. M. Glover (eds. (wikipedia.org)
- If you drive a C6 Corvette, you drive one of those cars that just needs a little bit more out of your brake system. (southerncarparts.com)
- Here at Southern Car Parts, we have the best C6 brakes, rotors, calipers and other brake accessories to help you give your Corvette the kind of brake system it deserves. (southerncarparts.com)
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- Ehrlich introduced the term "complement" as part of his larger theory of the immune system. (wikipedia.org)
- Ehrlich, therefore, named this heat-labile component "complement," because it is something in the blood that "complements" the cells of the immune system. (wikipedia.org)
- The complement system plays an important role in innate immunity inducing liver diseases as well as signaling immune cell activation in local inflammation regulating immunomodulatory effects such as liver damage and/or liver regeneration. (hindawi.com)
- In summary, our data suggest that the complement system may be involved in liver dysfunction in viral-induced acute liver failure cases using ex vivo assays. (hindawi.com)
- In ALF, extensive liver injury can be caused by a variety of molecules of the innate immune system, and the complement system plays an important role in this process [ 9 , 10 ]. (hindawi.com)
- Nevertheless, the function of the complement system is associated with processes of development, degeneration, and regeneration of multiple organs [ 12 - 14 ]. (hindawi.com)
- Here, we assessed the toxicological effects of the complement system in the peripheral blood of patients during acute liver failure. (hindawi.com)
- The complement system is known to be activated by the immunoglobulins IgM and IgG. (thefreedictionary.com)
- In the first, also referred to as the test system, antigen is mixed usually with serial dilutions of a test serum in the presence of complement. (thefreedictionary.com)
- Dalmasso, A.P. The complement system in xenotransplantation. (springer.com)
- Frank, M.M., Complement system. (springer.com)
- Cytolytic lymphocyte clones and complement as effectors of the immune system. (springer.com)
- The complement system is crucial for defense against pathogens and the removal of dying cells or immune complexes. (frontiersin.org)
- The complement system is a key component of innate immunity and contributes to the elimination of pathogens, dying host cells and abnormal molecular structures ( 1 , 2 ). (frontiersin.org)
- The complement system is an ancient part of the innate immune system important for both tissue homeostasis and host defense. (frontiersin.org)
- However, bacteria like Staphylococcus aureus (SA) possess elaborative mechanisms for evading both the complement system and other parts of the immune system. (frontiersin.org)
- These data bridge the roles of the complement system in tissue homeostasis and innate immunity and illustrate a novel mechanism by which the complement system combats persistent intracellular bacteria in epithelial cells. (frontiersin.org)
- In tissue homeostasis, the complement system plays a role as an intricate surveillance system by detecting altered host cells and facilitates clearance of dead or altered cells ( 1 , 3 ). (frontiersin.org)
- Complement: a key system for immune surveillance and homeostasis. (springer.com)
- Degn SE, Jensenius JC, Thiel S. Disease-causing mutations in genes of the complement system. (medscape.com)
- Dysregulation of the complement system has been implicated in several diseases and pathologies. (wikipedia.org)
- PNH is an ultra-rare blood disorder in which chronic, uncontrolled activation of complement, a component of the normal immune system, results in hemolysis (destruction of the patient's red blood cells). (alxn.com)
- The primary functions of the complement system are to protect from infection, to remove particulate substances, (like damaged or dying cells, microbes or immune complexes) and to help modulate adaptive immune responses. (primaryimmune.org)
- As part of the innate immune system, complement acts immediately to start the process of removal and resolution of the problem. (primaryimmune.org)
- Complement works with the inflammatory cells of the innate immune system and those of adaptive or acquired immunity. (primaryimmune.org)
- A unique feature of the AP is the presence of the only positive regulator in the complement system, Properdin. (primaryimmune.org)
- It activates C3 of the complement system and probably supports inflammation in other ways as well. (biology-pages.info)
- Complement component 7 is a component of the complement system. (sinobiological.com)
- In several models of autoimmune diseases, the overactivation of the complement system (CS) is the cause of disease exacerbation. (scielo.br)
- Our findings indicate that the complement and coagulation system is dysregulated in the blood during childhood before the development of the PD. (deepdyve.com)
- Activation of complement via the innate and the adaptive immune systems is vital to the body's defenses in fighting infections. (jimmunol.org)
- meningococcal or pneumococcal infections and auto-immune diseases are adverse events of anti-complement therapy. (frontiersin.org)
- Central to innate immunity, complement activation is heightened in pregnancy, 1 in part, to facilitate normal clearance of fetoplacental material, including apoptotic blebs, 2 circulating fetal DNA, 3 and immune complexes. (ahajournals.org)
- Functional complement C1q abnormality leads to impaired immune complexes and apoptotic cell clearance. (medscape.com)
- C2 and C4 also participate in the LP. The LP is thought to be the most evolutionarily primitive of the complement pathways and the first to react before the adaptive immune response occurs. (primaryimmune.org)
- Complement C3b may be an opsonin for antigen-antibody complexes which helps prevent damage from the formation of large, insoluble immune aggregates. (sigmaaldrich.com)
- Complement inhibitors are being studied as potential therapeutics for immune diseases and Alzheimer's. (sigmaaldrich.com)
- Borrelia burgdorferi has developed efficient mechanisms for evading the innate immune response during mammalian infection and has been shown to be resistant to the complement-mediated bactericidal activity of human serum. (asm.org)
Classical and alternative complement signalin1
- 6 The burden of fetoplacental debris becomes exaggerated in severe preeclampsia, 7 - 10 propagating a systemic inflammatory response and placing strain on both classical and alternative complement signaling pathways ( Figure 1 ) as early as the first trimester. (ahajournals.org)
- and producing a recombinant nucleic acid molecule by the action of said nucleic acid polymerase on said precursor molecule in said complementing cell. (freepatentsonline.com)
- 5. The method according to claim 1, wherein said precursor molecule lacks overlapping sequences with the nucleic acid of the complementing cell into which it is introduced, said overlapping sequences otherwise enabling homologous recombination leading to replication competent virus in the complementing cell. (freepatentsonline.com)
- C5a is also a potent chemokine which stimulates the locomotion of polymorphonuclear leukocytes and directs their migration toward sites of inflammation.Complement C5 (C3 And PZP Like Alpha 2 Macroglobulin Domain Containing Protein 4 or C5) pipeline Target constitutes close to 31 molecules. (medindia.net)
- It also reviews key players involved in Complement C5 (C3 And PZP Like Alpha 2 Macroglobulin Domain Containing Protein 4 or C5) targeted therapeutics development with respective active and dormant or discontinued projects.The report is built using data and information sourced from proprietary databases, company/university websites, clinical trial registries, conferences, SEC filings, investor presentations and featured press releases from company/university sites and industry-specific third party sources. (medindia.net)
Composed of the complement components1
- The MAC is composed of the complement components C5b, C6, C7, C8 and several C9 molecules. (wikipedia.org)
- Complement C6 antibody LS-C123308 is an unconjugated mouse monoclonal antibody to human Complement C6. (lsbio.com)
Preclinical and Phase 1 studies1
- Complement Pharma will be responsible for conducting preclinical and Phase 1 studies and for manufacturing CP010. (businesswire.com)
- Genetically determined molecular weight differences in murine complement component C6. (nih.gov)
- C3d of complement as a molecular adjuvant: bridging innate and acquired immunity. (springer.com)
- In addition, we have identified the minimal sequence of the Ad35-E1B region (molecular weight, 55,000 [55K]), pivotal for complementation of fully E1-lacking Ad35 vector on PER.C6 cells. (asm.org)
- A sandwich ELISA for quantitative measurement of Porcine Complement component C6(C6) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. (antibody-antibodies.com)
- Urine and blood samples were collected on the day of enrollment, with complement activation (C3a, C5a, and C5b-9) measured by ELISA. (ahajournals.org)
Antibody to complement2
- 1991). "Complete cDNA sequence of human complement pro-C5. (wikipedia.org)
- We thus predicted that the abnormal C6 molecule might be carboxy-terminally truncated and sought a mutation in an area approximately 14% from the carboxy-terminal end of the coding sequence. (jci.org)
- After stable insertion of the 55K sequence into PER.C6 cells a cell line was obtained (PER.C6/55K) that efficiently transcomplements both Ad5 and Ad35 vectors. (asm.org)
- In this chapter, the structure and function of the complement receptors on the macrophage and related cells are described. (springer.com)
- C5a, the smaller fragment, is a potent chemotactic and spasmogenic anaphylatoxin that mediates inflammatory responses by stimulating platelets, endothelial cells, eosinophils, neutrophils, and phagocytes to the site of complement activation ( 4 , 5 ). (jimmunol.org)
- Complements C3a and C4b are opsonins that bridge phagocytes to microorganisms. (sigmaaldrich.com)
- The following antibody was used in this experiment: Complement C6 Polyclonal Antibody from Thermo Fisher Scientific, catalog # PA1-40290, RRID AB_2067307. (thermofisher.com)
- We investigated the influence of the C5b-9 complement complex on infarct size, reflow and arrhythmogenesis. (biomedsearch.com)
- CONCLUSIONS: These results suggest that the lytic C5b-9 complement complex leads to reperfusion injury in the early phase (30 min) of ischaemia, resulting in a larger infarct. (biomedsearch.com)
- C5 convertases of the alternative and classical pathways of complement are complex serine proteases that are made up of two or more subunits ( 1 , 6 , 7 , 8 , 9 , 10 , 11 ). (jimmunol.org)
- C5b begins the assembly of complement components by binding C6 and C7 to its C5-C345C domain, creating a C5b-7 complex which remains loosely associated with C3b (Dalmasso, 1998). (davidson.edu)
- complement fixation the combining of complement with the antigen-antibody complex , rendering the complement inactive, or fixed. (thefreedictionary.com)
- The C5b-C6 complex is the foundation upon which the lytic complex is assembled. (reportbuyer.com)
- IgE mediated eosinophil production is induced by compounds released by basophils and mast cells , including eosinophil chemotactic factor of anaphylaxis , leukotriene B4 and serotonin mediated release of eosinophil granules occur, complement complex (C5-C6-C7), interleukin 5 , and histamine (though this has a narrow range of concentration). (wikipedia.org)
- Mollnes, Klos, Tschopp: Identification of a human C5 beta-chain epitope exposed in the native complement component but concealed in the SC5b-9 complex. (antikoerper-online.de)
- Dalmasso, A.P. Complement in the pathophysiology and diagnosis of human diseases. (springer.com)
- Ehrlich's term for the thermolabile substance, normally present in serum, that is destructive to certain bacteria and other cells sensitized by a specific complement-fixing antibody. (thefreedictionary.com)
- Complement C6 antibody was raised against purified human C6. (lsbio.com)
- Recognizes human C6. (lsbio.com)
- Complement C6 antibody was raised against synthetic peptide derived from human C6. (lsbio.com)
- A new method for the isolation of C6 and C7 by affinity chromatography of human serum with anti-C6 and anti-C7 coupled to Sepharose is described. (elsevier.com)
- Muller-Eberhard, H. J. / Structural similarities between C6 and C7 of human complement . (elsevier.com)
- Lyophilized fluorescein-5-isothiocyanate (FITC "Isomer I")-conjugated goat IgG fraction to human complement C4 and buffer salts. (mpbio.com)
- FITC-Conjugated Goat IgG Fraction to Anti-Human Complement C4 is used as a reagent in immunofluorescence assays (IFA), cell staining (fluorescent microscopy and cell sorting), tissue staining, and blot immunostaining. (mpbio.com)
- Binding of the complement-downregulating protein factor H (fH) to the surface of the meningococcus is important for survival of the organism in human serum. (asm.org)
- MAb502, which did not inhibit fH binding, had complement-mediated bactericidal activity only when tested with fH-depleted human complement. (asm.org)
- The combined data lead us to conclude that the CspA-mediated binding of human FH confers serum resistance by directly inhibiting complement deposition on the surface of B. burgdorferi . (asm.org)
- In fact, with regard to the major borrelial genospecies that cause Lyme disease, B. burgdorferi and Borrelia afzelii are resistant to the complement-mediated bactericidal activity of serum, while most strains of Borrelia garinii are killed by human serum ( 4 , 7 , 10 , 48 ). (asm.org)
- Many human pathogens, including serum-resistant B. burgdorferi , evade complement destruction by binding complement factor H (FH) and FH-like protein 1 (FHL-1) ( 4 , 29 , 32 , 40 , 55 ). (asm.org)
- All human adenovirus types, Ad1 through Ad51 (a kind gift from Jan de Jong, University of Rotterdam, Rotterdam, The Netherlands), were inoculated on PER.C6 cells. (asm.org)
- Next, we generated E1-lacking Ad35 viruses carrying marker genes using newly developed PER.C6/55K, Ad35 packaging cells. (asm.org)
- CFHR5 (Complement Factor H-Related protein 5) is able to bind to act as a cofactor for factor I, has decay accelerating activity and is able to bind preferentially to C3b at host surfaces. (wikipedia.org)