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.
Complement component C8gamma is expressed in human fetal and adult kidney independent of C8alpha. (1/110)Human complement component C8gamma is an unusual complement factor since it shows no homology to other complement proteins but is a member of the lipocalin superfamily. So far, it has been found exclusively in plasma, covalently linked to C8alpha by disulfide bridging. We have used dot blot and Northern blot analyses of a large number of different human tissues to survey systematically the expression pattern of C8gamma. Our experiments clearly showed that besides in liver, this gene is also expressed in fetal and adult kidney. Renal expression of C8gamma is not dependent on C8alpha expression, since we could not detect C8alpha expression in kidney. Thus its physiological function is not restricted to a specific action in association with complement components. As a prerequisite for further characterization of the structure and binding activities of the uncomplexed C8gamma, we have expressed the encoding cDNA in Escherichia coli. To increase the probability for proper folding of the characteristic intramolecular disulfide bridge the recombinant protein was produced by secretion to the periplasm. (+info)
Increased ion permeability of planar lipid bilayer membranes after treatment with the C5b-9 cytolytic attack mechanism of complement. (2/110)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)
The role of Fcgamma receptor polymorphisms and C3 in the immune defence against Neisseria meningitidis in complement-deficient individuals. (3/110)Individuals with either a late (C5-9) complement component deficiency (LCCD) or properdin deficiency are at increased risk to develop meningococcal disease, often due to serogroups W135 and Y. Anti-meningococcal defence in both LCCD persons and properdin-deficient individuals without bactericidal antibodies depends mainly on phagocytosis. Three types of opsonin receptors are involved in phagocytosis by polymorphonuclear cells (PMN). These represent the polymorphic FcgammaRIIa (CD32) and FcgammaRIIIb (CD16b) receptors, and the C3 receptor CR3 (CD11b/CD18). When the distribution of FcgammaRIIa and FcgammaRIIIb allotypes was assessed in 15 LCCD and in 15 properdin-deficient patients with/without previous meningococcal disease, we found the combination of FcgammaRIIa-R/R131 with FcgammaRIIIb-NA2/NA2 allotypes to be associated with previous meningococcal disease (odds ratio 13.9, Fisher's test P = 0.036). No such relation was observed in the properdin-deficient patients. The importance of FcgammaRIIa allotypes was also demonstrated using in vitro phagocytosis assays. PMN from FcgammaRIIa-R/R131 homozygous donors internalized IgG2 opsonized meningococci W135 significantly (P < 0.05) less than PMN from FcgammaRIIa-H/H131 donors. When properdin-deficient serum was tested, it was observed that reconstitution with properdin resulted in enhanced PMN phagocytosis of the W135 meningococci (P = 0.001). This enhanced phagocytosis was parallelled by an increase in C3 deposition onto the opsonized meningococci W135 (r = 0.6568, P = 0. 01). We conclude that the occurrence of meningococcal disease in LCCD patients is associated with certain FcgammaR allotypes. Properdin-deficient individuals are susceptible to meningococcal disease because of an insufficient C3 deposition on the surface of meningococci, resulting in insufficient phagocytosis. (+info)
Free radicals upregulate complement expression in rabbit isolated heart. (4/110)Both free radicals and complement activation can injure tissue. Our study determined whether free radicals alter complement production by the myocardium. Isolated hearts from New Zealand White rabbits were perfused on a Langendorff apparatus and exposed to xanthine (X; 100 microM) plus xanthine oxidase (XO; 8 mU/ml) (X/XO). The free radical-generating system significantly (P < 0.05) increased C1q and also increased C1r, C3, C8, and C9 transcription compared with controls. Immunohistological examination revealed augmented membrane attack complex deposition on X/XO-treated tissue. X/XO-treated hearts also exhibited significant (P < 0.05) increases in coronary perfusion pressure and left ventricular end-diastolic pressure and a decrease in left-ventricular developed pressure. N-(2-mercaptopropionyl)-glycine (3 mM), in conjunction with the superoxide dismutase mimetic SC-52608 (100 microM), significantly (P < 0.05) reduced the upregulation of C1q, C1r, C3, C8, and C9 mRNA expression elicited by X/XO. The antioxidants also ameliorated the deterioration in function caused by X/XO. Local complement activation may represent a mechanism by which free radicals mediate tissue injury. (+info)
CD59 protects rat kidney from complement mediated injury in collaboration with crry. (5/110)BACKGROUND: As previously reported, the membrane-bound complement regulator at the C3 level (Crry/p65) is important in maintaining normal integrity of the kidney in rats. However, the role of a complement regulator at the C8/9 level (CD59) is not clear, especially when activation of complement occurs at the C3 level. The aim of this work was to elucidate the in vivo role of CD59 under C3 activating conditions. METHODS: Two monoclonal antibodies, 5I2 and 6D1, were used to suppress the function of Crry and CD59, respectively. In order to activate alternative the pathway of complement, the left kidney was perfused with 5I2 and/or 6D1 and was recirculated. RESULTS: In the kidneys perfused with 5I2 alone, deposition of C3 and membrane attack complex (MAC) was observed in the peritubular capillaries, vasa recta, and tubular basement membranes. Cast formation, tubular dilation and degeneration, and cellular infiltration were observed at days 1 and 4, and they recovered by day 7. Further suppression of CD59 by 6D1 significantly enhanced the deposition of MAC and worsened the already exacerbated tubulointerstitial injury. These effects of 6D1 were dose dependent. Perfusion with 6D1 alone did not induce histologic damage or MAC deposition in the tubulointerstitium. CONCLUSIONS: In rats, CD59 maintains normal integrity of the kidney in collaboration with Crry in rats against complement-mediated damage in vivo. (+info)
Hypochlorite-induced alterations to canine serum complement. (6/110)Changes in the concentration of the components of complement produced by NaOC1 both in vitro and in vivo are recorded. C1, C4 and C7 are particularly sensitive to this oxidizing agent, although all components decrease at high concentrations of NaOC1. Following oxidation, complement componenets return rapidly to normal. Data are presented to indicate that part of this repair mechanism is due to the action of reducing agents such as ascorbic acid and part is due to the synthesis of the individual components. The unique sensitivity of complement components to oxidation make this treatment of potential value in suppressing the inflammatory response. (+info)
Novel mechanism of antibody-independent complement neutralization of herpes simplex virus type 1. (7/110)The envelope surface glycoprotein C (gC) of HSV-1 interferes with the complement cascade by binding C3 and activation products C3b, iC3b, and C3c, and by blocking the interaction of C5 and properdin with C3b. Wild-type HSV-1 is resistant to Ab-independent complement neutralization; however, HSV-1 mutant virus lacking gC is highly susceptible to complement resulting in > or =100-fold reduction in virus titer. We evaluated the mechanisms by which complement inhibits HSV-1 gC null virus to better understand how gC protects against complement-mediated neutralization. C8-depleted serum prepared from an HSV-1 and -2 Ab-negative donor neutralized gC null virus comparable to complement-intact serum, indicating that C8 and terminal lytic activity are not required. In contrast, C5-depleted serum from the same donor failed to neutralize gC null virus, supporting a requirement for C5. EDTA-treated serum did not neutralize gC null virus, indicating that complement activation is required. Factor D-depleted and C6-depleted sera neutralized virus, suggesting that the alternative complement pathway and complement components beyond C5 are not required. Complement did not aggregate virus or block attachment to cells. However, complement inhibited infection before early viral gene expression, indicating that complement affects one or more of the following steps in virus replication: virus entry, uncoating, DNA transport to the nucleus, or immediate early gene expression. Therefore, in the absence of gC, HSV-1 is readily inhibited by complement by a C5-dependent mechanism that does not require viral lysis, aggregation, or blocking virus attachment. (+info)
Human complement protein C8 gamma. (8/110)Human C8 gamma is a 22 kDa subunit of complement component C8, which is one of five components (C5b, C6, C7, C8, C9) that interact to form the cytolytic membrane attack complex (MAC) of complement. C8 contains three nonidentical subunits (alpha, beta, gamma) that are products of different genes. These subunits are arranged asymmetrically to form a disulfide-linked C8 alpha-gamma dimer that is noncovalently associated with C8 beta. C8 alpha and C8 beta are homologous to C6, C7 and C9 and together these proteins comprise what is referred to as the 'MAC protein family'. By comparison, C8 gamma is distinct in that it belongs to the lipocalin family of small, secreted proteins which have the common ability to bind small hydrophobic ligands. While specific roles have been identified for C8 alpha and C8 beta in the formation and function of the MAC, a function for C8 gamma and the identity of its ligand are unknown. This review summarizes the current status of C8 gamma structure and function and the progress made from efforts to determine its role in the complement system. (+info)
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.. [^] ...
Complement deficiencies - Medical information | BMJ Best Practice
Complement proteins moderate the actions of specific antibodies, aid the processing and removal of immune complexes, and modify T-cell and B-cell responses. Complement deficiencies can be inherited, or acquired as a result of infection (e.g., recurrent meningococcal or disseminated gonococcal inf...
Publications EDYSAN « Edysan
Publications : 2012 - 2013 - 2014 - 2015 - 2016 - 2017 - 2018 - 2019. PUBLICATIONS INTERNATIONALES 2020. Almoussawi, Ali, Jonathan Lenoir, Aurélien Jamoneau, Tarek Hattab, Safaa Wasof, Emilie Gallet‐Moron, Carol X. Garzon‐Lopez, Fabien Spicher, Ahmad Kobaissi, and Guillaume Decocq. 2020. Forest Fragmentation Shapes the Alpha-Gamma Relationship in Plant Diversity. Journal of Vegetation Science 31(1):63-74. doi: https://doi.org/10.1111/jvs.12817. IF 2.698. Almoussawi, Ali, Jonathan Lenoir, Fabien Spicher, Frederic Dupont, Olivier Chabrerie, Deborah Closset-Kopp, Boris Brasseur, Ahmad Kobaissi, Frederic Dubois, and Guillaume Decocq. 2020. Direct Seeding Associated with a Mixture of Winter Cover Crops Decreases Weed Abundance While Increasing Cash-Crop Yields. Soil & Tillage Research 200:104622. doi: 10.1016/j.still.2020.104622. IF 4.601. Castañeda, Irene, Diane Zarzoso-Lacoste and Elsa Bonnaud. 2020. Feeding behaviour of red fox and domestic cat populations in suburban areas in the south ...
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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. ...
Gamma motoneurons regulate how sensitive the stretch reflex is by tightening or relaxing the fibers within the spindle. There are several theories as to what may trigger gamma motoneurons to increase the reflexs sensitivity. For example, alpha-gamma co-activation might keep the spindles taut when a muscle is contracted, preserving stretch reflex sensitivity even as the muscle fibers become shorter. Otherwise the spindles would become slack and the reflex would cease to function. ...
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.
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 ...
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 ...
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
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 ...
Human Complement C2 ELISA Kit (ab154132) | Abcam
Human Complement C2 ELISA Kit is a Sandwich (quantitative) ELISA for the measurement of Human Complement C2 in Human Cell culture supernatant, Saliva, Milk, Serum, Plasma, Cerebral Spinal Fluid…
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 ...
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).. ...
Secreted Aspergillus fumigatus protease Alp1 degrades human complement proteins C3, C4, and C5. - Leibniz-Institut für...
The opportunistic human pathogenic fungus Aspergillus fumigatus is a major cause of fungal infections in immunocompromised patients. Innate immunity plays an important role in the defense against infections. The complement system represents an essential part of the innate immune system. This cascade system is activated on the surface of A. fumigatus conidia and hyphae and enhances phagocytosis of conidia. A. fumigatus conidia but not hyphae bind to their surface host complement regulators factor H, FHL-1, and CFHR1, which control complement activation. Here, we show that A. fumigatus hyphae possess an additional endogenous activity to control complement activation. A. fumigatus culture supernatant efficiently cleaved complement components C3, C4, C5, and C1q as well as immunoglobulin G. Secretome analysis and protease inhibitor studies identified the secreted alkaline protease Alp1, which is present in large amounts in the culture supernatant, as the central molecule responsible for this ...
Fluorescein-Conjugated Goat F(Ab)2 Fragment To Human Complement C3
Fluorescein-Conjugated Goat F(ab)|sub|2|/sub| Fragment to Human Complement C3 is the lyophilized powder of fluorescein-5-isothiocyanate (FITC Isomer I)-conjugated goat F(ab)|sub|2|/sub| fragment to human complement C3 and buffer salts.
PB11 competition: satisfaction and optimization track competition: execution trace
runsolver Copyright (C) 2010-2011 Olivier ROUSSEL This is runsolver version 3.3.0 (svn: 977) This program is distributed in the hope that it will be useful, but WITHOUT ANY WARRANTY; without even the implied warranty of MERCHANTABILITY or FITNESS FOR A PARTICULAR PURPOSE. See the GNU General Public License for more details. command line: BIN/runsolver --timestamp -w /tmp/evaluation-result-3452688-1307087324/watcher-3452688-1307087324 -o /tmp/evaluation-result-3452688-1307087324/solver-3452688-1307087324 -C 1800 -W 1900 -M 15500 HOME/scip-184.108.40.206.linux.x86_64.gnu.opt.spx -f HOME/instance-3452688-1307087324.opb -t 1800 -m 15500 running on 4 cores: 0,2,4,6 Enforcing CPUTime limit (soft limit, will send SIGTERM then SIGKILL): 1800 seconds Enforcing CPUTime limit (hard limit, will send SIGXCPU): 1830 seconds Enforcing wall clock limit (soft limit, will send SIGTERM then SIGKILL): 1900 seconds Enforcing VSIZE limit (soft limit, will send SIGTERM then SIGKILL): 15872000 KiB Enforcing VSIZE limit (hard ...
GRP75/HSPA9B/Mortalin Antibody (OTI9F8) (NBP1-47801): Novus Biologicals
Mouse Monoclonal Anti-GRP75/HSPA9B/Mortalin Antibody (OTI9F8). Immortalization Marker. Validated: WB, Flow, ICC/IF, IHC, IHC-P. Tested Reactivity: Human, Mouse, Rat, and more. 100% Guaranteed.
Stretch reflex - Wikipedia
The stretch reflex (myotatic reflex) is a muscle contraction in response to stretching within the muscle. It is a monosynaptic reflex which provides automatic regulation of skeletal muscle length. When a muscle lengthens, the muscle spindle is stretched and its nerve activity increases. This increases alpha motor neuron activity, causing the muscle fibers to contract and thus resist the stretching. A secondary set of neurons also causes the opposing muscle to relax. The reflex functions to maintain the muscle at a constant length. Gamma motoneurons regulate how sensitive the stretch reflex is by tightening or relaxing the fibers within the spindle. There are several theories as to what may trigger gamma motoneurons to increase the reflexs sensitivity. For example, alpha-gamma co-activation might keep the spindles taut when a muscle is contracted, preserving stretch reflex sensitivity even as the muscle fibers become shorter. Otherwise the spindles would become slack and the reflex would cease ...
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.
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).
Human Complement C4 ELISA Kit | Abcam
Abcams Complement C4 ELISA Kit (ab108825) suitable for Cell culture supernatant, Saliva, Milk, Urine, Cerebral Spinal Fluid in human. Reliably quantify 0.07…
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. ...
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. …
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 ...
Membrane attack complex inhibitor CD59a protects against focal cerebral ischemia in mice | Journal of Neuroinflammation | Full...
The complement system is a crucial mediator of inflammation and cell lysis after cerebral ischemia. However, there is little information about the exact contribution of the membrane attack complex (MAC) and its inhibitor-protein CD59. Transient focal cerebral ischemia was induced by middle cerebral artery occlusion (MCAO) in young male and female CD59a knockout and wild-type mice. Two models of MCAO were applied: 60 min MCAO and 48 h reperfusion, as well as 30 min MCAO and 72 h reperfusion. CD59a knockout animals were compared to wild-type animals in terms of infarct size, edema, neurological deficit, and cell death. CD59a-deficiency in male mice caused significantly increased infarct volumes and brain swelling when compared to wild-type mice at 72 h after 30 min-occlusion time, whereas no significant difference was observed after 1 h-MCAO. Moreover, CD59a-deficient mice had impaired neurological function when compared to wild-type mice after 30 min MCAO. We conclude that CD59a protects against ischemic
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.
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. ...
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 ...
[A type of glomerulonephritis that is characterized by the accumulation of immune deposits ( COMPLEMENT MEMBRANE ATTACK COMPLEX) on the outer aspect of the GLOMERULAR BASEMENT MEMBRANE. It progresses from subepithelial dense deposits, to basement membrane reaction and eventual thickening of the basement membrane., A slowly progressive inflammation of the glomeruli characterized by immune complex deposits at the glomerular basement membrane, resulting in a thickened membrane, and nephrotic syndrome.]
Native Human Complement 4c | Cell Sciences
Human Complement C4c (C4c) is used as an immunogen and is the inactive form of Human Complement C4. C4c interacts with complement C1 and complement C2 to form complement C3 convertase of the classic activation pathway. Systemic lupus erythematosus is ofte
Meningococcal Infections | Childrens Hospital of Philadelphia
Meningococcal infections are caused by a bacteria called Neisseria meningitidis. The most common forms of meningococcal infections include meningitis and meningococcemia . Meningococcal infections are uncommon, but can be fatal.
Cefuroxime-induced immune hemolysis<...
TY - JOUR. T1 - Cefuroxime-induced immune hemolysis. AU - Malloy, Colleen Ann. AU - Kiss, Joseph E.. AU - Challapalli, Malliswari. PY - 2003/7/1. Y1 - 2003/7/1. N2 - Drug-induced immune hemolytic anemia (IHA) is rare but is being reported with increasing frequency. The most commonly cited cause of drug-induced IHA has been receipt of second and third generation cephalosporin antibiotics. We report the first case of IHA associated with cefuroxime administration.. AB - Drug-induced immune hemolytic anemia (IHA) is rare but is being reported with increasing frequency. The most commonly cited cause of drug-induced IHA has been receipt of second and third generation cephalosporin antibiotics. We report the first case of IHA associated with cefuroxime administration.. UR - http://www.scopus.com/inward/record.url?scp=0041743122&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=0041743122&partnerID=8YFLogxK. U2 - 10.1016/S0022-3476(03)00210-5. DO - ...
Tory). The regulatory unit consists of EGF (red), TS1, TS2, and TS3 modules (blue). The upper unit includes the LR Propionylpromazine (hydrochloride) site module (magenta) and also the upper fragment of MACPF, such as the linchpin helix (red). The lower unit includes the reduce fragment of MACPF like CH1CH2 (green) and CH3 (orange). Glycosylation sites are shown as brown sticks. Two disulfide bonds linking TS3 to MACPF and EGF are shown as yellow balls. B, comparison of C6 (lacking CCPs and FIMs) with perforin (PDB code 2NSJ) along with a member of your CDC family members, PFO (PDB code 1PFO). The domains of PFO are designated D1 to D4. D1 and D3 are analogous to the upper and lower domains of C6. The linchpin helices (in orange) as well as the EGF domains (in red) of C6 and perforin have some functional analogy with domain D2 of PFO, but PFO and perforin lack the regulatory functions supplied by the auxiliary domains of C6. D4 might be identified with TS1 of C6 on structural and possibly ...
Peitsch MC, Tschopp J, Jenne DE, Haefliger JA (1991). "Structural and functional characterization of complement C8 gamma, a ... complement component C8 gamma chain; crustacyanin; epididymal-retinoic acid binding protein (E-RABP); insectacyanin; odorant ...
Power, LM; Chua, SC Jr; Leibel, RL (1994). "Locus D1S21 contains exonic sequence from the C8 beta component of complement". ...
... proteins of the complement system (C6, C7, C8α, C8β and C9) and perforin (PF). Members of this protein family are pore-forming ... "Identity of the segment of human complement C8 recognized by complement regulatory protein CD59". J. Biol. Chem. 270 (34): ... Complement proteins C6-C9 all contain a MACPF domain and assemble into the membrane attack complex. C6, C7 and C8β appear to be ... The binding site on C8α is known, however, the precise role of C8γ in the MAC remains to be understood. Deficiency of C9, or ...
Hadders, M. A.; Beringer, D. X.; Gros, P. (2007). "Structure of C8 -MACPF Reveals Mechanism of Membrane Attack in Complement ... This protein is part of the oldest part of the immune system that is present in the human body, the complement system. Using ... Among his findings are the molecular mechanisms that underlie key steps in the human immune defense by the complement system, ... such as the mode of action of complement component 3 and component 8. Other research in his lab has focussed on the adhesion of ...
"Structure of human C8 protein provides mechanistic insight into membrane pore formation by complement". J Biol Chem. 286 (20): ... the first crystal structure of a protein containing this type of glycosylation was determined-that of human complement ...
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 ...
... the four genes for complement C8 gamma chain, prostaglandin-D-synthase, oncogene-24p3, and progestagen-associated endometrial ...
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 ...
Ninomiya H, Sims PJ (1992). "The human complement regulatory protein CD59 binds to the alpha-chain of C8 and to the "b"domain ... When complement activation leads to deposition of C5b678 on host cells, CD59 can prevent C9 from polymerizing and forming the ... 1992). "Complement regulatory proteins at the feto-maternal interface during human placental development: distribution of CD59 ... Bohana-Kashtan O, Ziporen L, Donin N, Kraus S, Fishelson Z (July 2004). "Cell signals transduced by complement". Mol. Immunol. ...
Ninomiya H, Sims PJ (July 1992). "The human complement regulatory protein CD59 binds to the alpha-chain of C8 and to the "b" ...
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+C8 at the US National Library of Medicine Medical Subject Headings (MeSH) v t e. ... C8 is a heterotrimer; it consists of three different subunits. These are called C8 alpha, beta and gamma chains, encoded by the ... "Structure of human C8 protein provides mechanistic insight into membrane pore formation by complement". The Journal of ...
Complement membrane attack complex
C8 is a complex made of the two proteins C8-beta and C8 alpha-gamma. ... Polymerization: C8-C9. Similar hydrophobic sites on C8 and C9 molecules are exposed when they bind to the complex, so ... MAC is composed of a complex of four complement proteins (C5b, C6, C7, and C8) that bind to the outer surface of the plasma ... C8 alpha-gamma has the hydrophobic area that inserts into the bilayer. C8 alpha-gamma induces the polymerization of 10-16 ...
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
"Evidence that C5b recognizes and mediates C8 incorporation into the cytolytic complex of complement". J. Immunol. 139 (6): 1960 ... 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 ... 2008). "Structure of and influence of a tick complement inhibitor on human complement component 5". Nat Immunol. 9 (7): 753-60 ...
Terminal complement pathway deficiency
... and C8. (While C9 is part of the MAC, and deficiencies have been identified, it is not required for cell lysis.) People with ... 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. ...
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 ...
C8, and polymeric C9. MAC is the cytolytic endproduct of the complement cascade; it forms a transmembrane channel, which ... 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 ...
... three proteins involved in the complement system (part of the immune system) Cervical spinal nerve 8 in human anatomy An octyl- ... Citroën C8, a brand of minivan LSWR C8 class, a London and South Western Railway locomotive Sauber C8, a 1985 racing car Spyker ... C8, C08, C.VIII or C-8 may refer to: AEG C.VIII, a World War I German armed reconnaissance aircraft AGO C.VIII, a World War I ... C8, a sportscar produced by car manufacturer Spyker Cars C-8 (Cercanías Madrid) Eighth generation Chevrolet Corvette (C8) ...
Complement component 1s
... (EC 220.127.116.11, 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 ...
CD59 - 维基百科，自由的百科全书
The human complement regulatory protein CD59 binds to the alpha-chain of C8 and to the "b"domain of C9. J. Biol. Chem. 1992, ... regulation of complement activation. • cell activation. • neutrophil degranulation. • regulation of complement-dependent ... complement binding. 細胞組分. • 囊泡. • endoplasmic reticulum membrane. • 膜. • 粘着斑. • Golgi membrane. • 细胞膜. • 細胞外區域. • cell surface ... Entrez Gene: CD59 molecule, complement regulatory protein.. *^ 7.0 7.1 Maio M, Brasoveanu LI, Coral S, Sigalotti L, Lamaj E, ...
Autoimmune hemolytic anemia
Cases may also arise with complement alone or with IgA, IgM or a combination of these three antibody classes and complement. ... C8, C9) either can form the membrane attack complex (MAC) or can bind the antibody, aiding phagocytosis by macrophages (C3b). ... 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 ...
Complement:. 146. Armament:. *As built:. *3 x 152 mm (6.0 in) naval guns (3x1) ...
Complement amounted to 30. Along with the rest of the Romanian Navy, the class saw service during the Romanian Campaign of the ...
The public system is complemented by numerous private clinics. Iași has the second-worse air quality in Romania, after ...
For decades, the two complemented each other within the same integrated factory. Until 1855, the works belonged to the Treasury ...
The C1 complement complex binds to these antibodies resulting in its activation via cross proteolysis. This activated C1 ... C5b associates with C6, C7, C8, and C9, all of which form a complex that results in a pore through the pathogen's membrane. ... 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 ...
Ștefan Vodă District
Forests of the district are complemented by tree species such as oak, ash, hornbeam, linden, maple, walnut and others. From ...
Komplemendisüsteem - Vikipeedia
Zipfel, P. F., Hallström, T., & Riesbeck, K. (2013). Human complement control and complement evasion by pathogenic microbes- ... C8 ja polümeerset C9 valku. Membraaniründe kompleks on komplemendisüsteemi ahelreaktsioonide tsütolüütiline lõpp-produkt, ... 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. ...
Corrocher R, Tedesco F, Rabusin P, De Sandre G (1975). "Effect of human erythrocyte stromata on complement activation". Br. J. ...
p. c8. Jewel T, one of the big guys in the limited-assortment discount grocery business, will open eight stores Wednesday in ... 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- ...
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 ( ... Sîstema temamker an jî sîstema tewawker (bi îngilîzî: complement system) beşek ji sîstema bergiriya giştî ye. Sîstema ... 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 ...
... 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 ...
Sistem komplementa - Wikipedija, prosta enciklopedija
Označujemo jih z veliko črko C (kratica za complement) in številko. Ko signal sproži spremembo enega od začetnih proencimov v ... Tako aktivirana C5 sproži nastanek kompleksa iz molekul C5b, C6, C7 in C8, ki se vrine v membrano bakterije. Tam sproži ...
... complement receptors, Fc receptors, B cell receptors and T cell receptors. ...
HMS Temeraire (1798)
Essex Record Office: D/DGu/C8. as cited in Willis. The Fighting Temeraire. p. 192. Adkin, Mark (2007). The Trafalgar Companion ... Vice-Admiral Mitchell was granted extraordinary powers regarding the death sentence and Temeraire's complement of marines was ... and provided men from her sailor and marine complement to crew batteries and gunboats. Men from Temeraire were heavily involved ...
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 ...
It is innervated by the anterior interosseus branch of the median nerve (C7-C8) It is a persistence of one of the former ... highly functional adaptation be followed by a series of more complex ones that complement it. With Homo habilis, an advanced ... It is innervated by the deep branch of the ulnar nerve (C8-T1). The first dorsal interosseous, one of the central muscles of ... It is innervated by the deep branch of the radial nerve (C7-C8). The extensor pollicis longus (EPL) originates on the dorsal ...
St. Xavier High School (Ohio)
p. C8. Archived from the original on January 23, 2008. Retrieved January 8, 2007. "OHSAA 2003 Boys CC State Results". Ohio High ... Later, a tuition-free elementary school division opened to complement the college. In 1844, the school's elementary division ...
Royal Canadian Mounted Police
Colt Canada C8 IUR (Integrated Upper Receiver ) 5.56mm NATO. The semi-automatic C8 IUR was adopted for general use in October, ... to complement the Mounted Police capbadge and avoid using a Canadian coin that would deface the monarch. The RCMP last issued ... "RCMP Adopts C8 Carbine - Soldier Systems Daily". "Under Fire: The carbine". Global News. "EXCLUSIVE: All RCMP cadets now ... Remington 870 12-gauge shotgun Colt Canada C7 rifle (5.56mm NATO) Colt Canada C8 carbine (5.56mm NATO) - Adopted by ERT in ...
Kenneth D. Taylor
p. C8. "Escape from Iran: The Canadian Caper (1981)". Knelman, Martin (September 13, 2012). "TIFF 2012: How Canadian hero Ken ... "The involvement of the CIA complemented efforts of the Canadian embassy to free the six held in Tehran. To this day the story ...
108 (C8): 1978-2012. Bibcode:2003JGRC..108.3277K. doi:10.1029/2001jc001031.CS1 maint: multiple names: authors list (link) ... Expanding this atmospheric source of iron could complement ship-based deployment. One proposal is to boost the atmospheric iron ...
District of Budapest is András Pikó (member of c8 - NGO in VIII district - supported by Momentum). The District Assembly, ... complemented by the Kempelen Farkas Student Information and Resource Centre. The Piarist Secondary School, the main building of ...
List of MeSH codes (D12.776.124)
... complement c6 MeSH D12.776.124.486.274.650 - complement c7 MeSH D12.776.124.486.274.750 - complement c8 MeSH D12.776.124.486. ... complement c1 MeSH D12.776.124.486.274.050.270 - complement c1q MeSH D12.776.124.486.274.050.280 - complement c1r MeSH D12.776. ... complement c2 MeSH D12.776.124.486.274.150.500 - complement c2a MeSH D12.776.124.486.274.150.750 - complement c2b MeSH D12.776. ... complement c3c MeSH D12.776.124.486.274.250.260.750 - complement c3d MeSH D12.776.124.486.274.350 - complement c4 MeSH D12.776. ...
HMS Norham Castle (K447)
Brown states the complement as 99 and Johnston states the complement of Canadian ships at 112 (7 officers and 105 ratings). Mk ... Following that, the ship joined the Mid-Ocean Escort Force as a member of the escort group C-8 for convoy escort duty in the ... The ships had a complement of 120. The ships were powered by two Admiralty three-drum boilers which created 2,750 indicated ...
Speed 2: Cruise Control
p. C-8. Ryan, James (May 4, 1997). "Look, Ma, No Pixels: Plastic Triumphs On the Set". The New York Times. Archived from the ... To complement the film's Caribbean setting, the soundtrack consists of mostly reggae music. De Bont wanted musicians to appear ...
Spoleto Festival USA
p. C8. Retrieved May 24, 2013. Jerry R. Sanders (Sep 28, 1976). "Stern to Head Spoleto". Charleston News & Courier. p. 1A. ... Piccolo Spoleto is "the perfect complement to the international scope of its parent festival and its 700 events in 17 days ...
Complement component C8 beta chain - DrugBank
Complement component C8 beta chain. Details. Name. Complement component C8 beta chain. Synonyms. *Complement component 8 ... complement activation / complement activation, alternative pathway / complement activation, classical pathway / cytolysis / ... Haefliger JA, Tschopp J, Nardelli D, Wahli W, Kocher HP, Tosi M, Stanley KK: Complementary DNA cloning of complement C8 beta ... Dewald G, Hemmer S, Nothen MM: Human complement component C8. Molecular basis of the beta-chain polymorphism. FEBS Lett. 1994 ...
C8B - Complement component C8 beta chain precursor - Homo sapiens (Human) - C8B gene & protein
Complement component C8 beta chainAdd BLAST. 537. Amino acid modifications. Feature key. Position(s). DescriptionActions. ... "Human complement component C8. Molecular basis of the beta-chain polymorphism.". Dewald G., Hemmer S., Noethen M.M.. FEBS Lett ... Belongs to the complement C6/C7/C8/C9 family.Curated. Keywords - Domaini. EGF-like domain, Repeat, Signal. Phylogenomic ... IPR037566 Complement_C8_beta. IPR036055 LDL_receptor-like_sf. IPR023415 LDLR_class-A_CS. IPR002172 LDrepeatLR_classA_rpt. ...
RCSB PDB - 2QOS: Crystal structure of complement protein C8 in complex with a peptide containing the C8 binding site...
Crystal structure of complement protein C8gamma in complex with a peptide containing the C8gamma binding site on C8alpha: ... Crystal structure of complement protein C8 in complex with a peptide containing the C8 binding site on C8. *DOI: 10.2210/ ... Complement component C8 alpha chain A 11 Homo sapiens Mutation: C164A Gene Name(s): C8A Gene View ... Complement component 8, gamma polypeptide C 173 Homo sapiens Mutation: C40A Gene Name(s): C8G Gene View ...
C8 Complement, Antigen | MLabs
Complement component C8, A chain
... The C8A gene encodes the alpha subunit of the complement component 8. C8 participates in the ... Genomic organization of human complement protein C8 alpha and further examination of its linkage to C8 beta. ... Merritt AD et al. (1976) Chromosome 6: linkage of the eighth component of complement (C8) to the histocompatibility region (HLA ... Evidence for two unlinked genetic loci for the eighth component of human complement (C8). ...
"Human Complement Protein C8: Crystal Structure and Analysis of Binding" by Christopher Langston Cooper
The MAC is comprised of complement proteins C5b, C6, C7, C8 and C9. The 150-kDa C8 protein consists of three non-identical ... The role of C8 within the MAC is to initiate formation of a transmembrane pore consisting of 12-18 molecules of C9. ... Human C8 is part of the membrane attack complex (MAC) that assembles on bacterial cell membranes to form a lethal pore-like ... The MAC is comprised of complement proteins C5b, C6, C7, C8 and C9. The 150-kDa C8 protein consists of three non-identical ...
Complement membrane attack complex - Wikipedia
C8 is a complex made of the two proteins C8-beta and C8 alpha-gamma. ... Polymerization: C8-C9. Similar hydrophobic sites on C8 and C9 molecules are exposed when they bind to the complex, so ... MAC is composed of a complex of four complement proteins (C5b, C6, C7, and C8) that bind to the outer surface of the plasma ... C8 alpha-gamma has the hydrophobic area that inserts into the bilayer. C8 alpha-gamma induces the polymerization of 10-16 ...
C8B gene: MedlinePlus Genetics
... of a protein complex called complement component 8. Learn about this gene and related health conditions. ... Chromosomal assignment of genes encoding the alpha, beta, and gamma subunits of human complement protein C8: identification of ... Complement component 8 aids in a part of the bodys immune response known as the complement system. The complement system is a ... Genetic basis of human complement C8 beta deficiency. J Immunol. 1993 Jun 1;150(11):4943-7. Citation on PubMed ...
MedlinePlus: Genes: C
Single-particle electron microscopy reconstruction of the structure of complement component 8 (C8) at 23 angstrom resolution -...
Single-particle electron microscopy reconstruction of the structure of complement component 8 (C8) at 23 angstrom resolution ... Single-particle electron microscopy reconstruction of the structure of complement component 8 (C8) at 23 angstrom resolution ... Identification of immune complement function as a determinant of adverse SARS-CoV-2 infection outcome ... Deregulated cellular circuits driving immunoglobulins and complement consumption associate with the severity of COVID-19 ...
Crystal Structure of Human Complement Protein C8γ at 1.2 Å Resolution Reveals a Lipocalin Fold and a Distinct Ligand Binding...
Molecular cloning of Limulus alpha 2-macroglobulin
Killing of Neisseria meningitidis by human neutrophils: implications for normal and complement-deficient individuals
... to the killing of 62 strains of meningococci were examined by using C8-depleted or pooled human serum (PHS). The complement- ... The contributions of complement-dependent phagocytosis and serum bactericidal activity (SBA) ... to the killing of 62 strains of meningococci were examined by using C8-depleted or pooled human serum (PHS). The complement- ... susceptibility to complement-dependent phagocytosis and complement-mediated SBA of serogroups B and Y did not correlate. We ...
Low-density lipoprotein (LDL) receptor class A, conserved site (IPR023415) | InterPro | EMBL-EBI
iTRAQ-Based Quantitative Proteomic Analysis Identified HSC71 as a Novel Serum Biomarker for Renal Cell Carcinoma
Complement C9. 984.63. 41.86%. 1.815. P02787. Serotransferrin. 1689.45. 61.46%. 1.775. P07360. Complement component C8 gamma ... A. Narayanasamy, J.-M. Ahn, H.-J. Sung et al., "Fucosylated glycoproteomic approach to identify a complement component 9 ... and complement C9 . However, those candidate biomarkers need further research to confirm the clinical utility of as ...
PROSITE: PDOC00021 (documentation file)
Lipocalin - Wikipedia
Recombinant Human C8G protein (ab113121) | Abcam
IUCr) Acta Crystallographica Section D Volume 63, Part 6, June 2007
Crystal structure of CD59: implications for molecular recognition of the complement proteins C8 and C9 in the membrane-attack ... The crystal structure of the human complement protein CD59 is presented at 2.1 Å resolution. This structure is compared with a ... previous NMR model and the binding region for C8 and C9 is mapped. ...
Table], What is the prevalence of primary immunodeficiency in children and young people with meningococcal disease? - Bacterial...
Hereditary complement deficiency in survivors of meningococcal disease: high prevalence of C7/C8 deficiency in Sephardic ( ... Genetic aspects of complement component C8 in Norwegian meningococcal disease patients. Scandinavian Journal of Infectious ... Complement deficiencies in patients over ten years old with meningococcal disease due to uncommon serogroups: Comment. ... 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
Torso-like functions independently of Torso to regulate Drosophila growth and developmental timing | PNAS
KAKEN - Research Projects | Planning-Coordination Committe on Special Cancer Research (KAKENHI-PROJECT-09042003)
Publications] Kojima,T.: Genetic basis of human complement c8 α-γ deficiency. J.Immunology. 161. 3762-3766 (1998). *. Related ... Publications] Kojima, T.: Genetic basis of human complement c8 α-γ deficiency. J.Immunology. 161. 3762-3766 (1998). *. ... Publications] Kojima, T.: Genetic basis of human complement c8 alpha-gamma deficiency. J.Immunology. 161. 3762-3766 (1998). * ...
34. Infections in immunodeficiency Flashcards by Eva Gonzales | Brainscape
A boy with C8 complement deficiency requires a vaccine. Which vaccine does he need? Which bacterium is he most susceptible to ... Meningococcal vaccine; Neisseria, as there is no formation of membrane attack complex (late complement deficiency). ... bacteria encapsuled species with early complement deficiencies, Neisseria with late compoment deficiencies ...
Immune System Flashcards by Lauren Schnitzen | Brainscape
Complement mediates the induction of granule release from mast cells and recruitment of inflammatory cells into the tissue ... What results from deficiencies in terminal complement C5-C8? Associated with recurrent infections ... Once activates, what is the role of the complement in a type III hypersensitivity reaction? ... Stimulates mast cells to release histamine and activates the complement pathway leading to thromboxane production ...
C6 Gene - GeneCards | CO6 Protein | CO6 Antibody
Complement C6, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene ... Belongs to the complement C6/C7/C8/C9 family.. Family:. * Belongs to the complement C6/C7/C8/C9 family. ... C6 (Complement C6) is a Protein Coding gene. Diseases associated with C6 include Complement Component 6 Deficiency and ... Complement component 6 deficiency (C6D) [MIM:612446]: A rare defect of the complement classical pathway associated with ...
Anti-C7 antibody (ab231047) | Abcam
Rudolph Leibel - Wikipedia
Postmenopausal estrogen and progestin effects on the serum proteome | Genome Medicine | Full Text
ProteinsDeficiencyMembraneCD59Classical pathwaySERUMAntibodyDeficienciesBindsCell lysisProtein C8BindSubunitsGenesLysisConvertaseActivationPolypeptidePhagocytosisCascadeComponent of complementMolecularBacteriaImmuneComplexMeningococcalParticipatesPositive regulator of the alternative complemRegulatorFragmentsInflammationAlternative pathwaysEighth component of human complementHumanPathogensLectinANTIGENTissuesInfectionsInnate immunityTerminal pathwayComponentsActivate
- The complement system is a group of proteins that work together to destroy foreign invaders, trigger inflammation, and remove debris from cells and tissues. (medlineplus.gov)
- Complement component 8 combines with several other complement proteins to form the membrane attack complex (MAC), which inserts itself in the outer membrane of bacterial cells. (medlineplus.gov)
- 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)
- 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)
- C8 is a complex made of the two proteins C8-beta and C8 alpha-gamma. (wikipedia.org)
- The complement system is a network of more than 50 plasma proteins and receptors, which have the role of mediating innate and adaptive host defence mechanisms, whereas they also participate in various (patho)physiological processes. (els.net)
- The primary functions mediated by complement proteins include phagocytosis of foreign elements (bacteria, viruses, particles etc .), cell lysis, inflammation, solubilisation of immune complexes, apoptotic cell clearance and enhancement of humoral immune responses. (els.net)
- Complement is a multiprotein network of plasma proteins and cell surface receptors. (els.net)
- 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)
- Activation of this system leads to a deposition of complement proteins on the bacterial surface, which results in opsonization of pathogens. (jimmunol.org)
- Some pathogens are able to inactivate complement components, whereas others express proteins that mimic the complement inhibitors of the host ( 10 ). (jimmunol.org)
- 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)
- OBJECTIVE We examined the association of urine complement proteins with progression to end-stage renal disease (ESRD) or death in people with type 2 diabetes and proteinuric diabetic kidney disease (DKD). (diabetesjournals.org)
- RESEARCH DESIGN AND METHODS Using targeted mass spectrometry, we quantified urinary abundance of 12 complement proteins in a predominantly Mexican-American cohort with type 2 diabetes and proteinuric DKD ( n = 141). (diabetesjournals.org)
- The association of urine complement proteins with progression to ESRD or death was evaluated using time-to-event analyses. (diabetesjournals.org)
- CONCLUSIONS In a largely Mexican-American cohort with type 2 diabetes and proteinuric DKD, urine abundance of several complement and complement regulatory proteins was strongly associated with progression to ESRD and death. (diabetesjournals.org)
- 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)
- 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)
- 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)
- 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)
- C7 is one of five complement proteins (C5b, C6, C7, C8, and C9)that assemble on pathogen membranes to form the lethal membrane attack complex. (innov-research.com)
- 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)
- Complement proteins C8 and C9 can bind to VTN:C5b:C6:C7 to form soluble C5b-C9 complex in plasma. (reactome.org)
- 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)
- Subsequent studies revealed that T. spiralis worms could bind to complement components [ 8 - 10 ], suggesting that T. spiralis contains proteins that bind to and potentially inhibit complement activation to protect against host complement attack. (biomedcentral.com)
- Mutations cause autosomal recessive complement C8 deficiency type 1. (moldiag.com)
- C8 deficiency in a family with xeroderma pigmentosum. (moldiag.com)
- Several mutations in the C8B gene cause complement component 8 deficiency type II. (medlineplus.gov)
- People with complement component 8 deficiency have a significantly increased risk of developing recurrent infections, particularly by Neisseria meningitidis , which causes meningitis, a serious condition that involves inflammation of the membranes surrounding the brain and spinal cord. (medlineplus.gov)
- C8B gene mutations involved in complement component 8 deficiency are most often of a type called a C to T transition, in which a DNA building block (nucleotide) called cytosine (C) is changed to the nucleotide thymine (T). Most commonly, this change occurs in a region of the gene called exon 9, but it can occur in other regions. (medlineplus.gov)
- Deficiency of the eighth component of complement associated with recurrent meningococcal meningitis--case report and literature review. (medlineplus.gov)
- 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)
- A boy with C8 complement deficiency requires a vaccine. (brainscape.com)
- Mutations in this gene are associated with complement component-6 deficiency. (genecards.org)
- Diseases associated with C6 include Complement Component 6 Deficiency and Immunodeficiency Due To A Late Component Of Complement Deficiency . (genecards.org)
- Defects in C7 are a cause of complement component 7 deficiency (C7D) [MIM:610102]. (abcam.com)
- 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)
- Neisseria meningitidis Serogroup Z Meningitis in a Child With Complement C8 Deficiency and Potential Cross Protection of the MenB-4C Vaccine. (amedeo.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)
- 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)
- Case 6: Deficiency of the C8 Complement Component 7. (taylorfrancis.com)
- Defects in C7 are a cause of complement component 7 deficiency (C7D). (sinobiological.com)
- C8 participates in the formation of the membrane attack complex (MAC) which disrupts bacterial walls, which mediates cell lysis. (moldiag.com)
- Human C8 is part of the membrane attack complex (MAC) that assembles on bacterial cell membranes to form a lethal pore-like structure. (sc.edu)
- The membrane attack complex is initiated when the complement protein C5 convertase cleaves C5 into C5a and C5b. (wikipedia.org)
- C8 alpha-gamma induces the polymerization of 10-16 molecules of C9 into a pore-forming structure known as the membrane attack complex. (wikipedia.org)
- C8 is a constituent of the membrane attack complex. (abcam.com)
- 2011) Structure of human complement C8, a precursor to membrane attack. (els.net)
- The membrane attack complex (MAC) of complement, C5b-9, was also involved in MS and EAE, as shown by the presence of C5b-9 neoantigens in the spinal fluid of MS patients ( 10 ), on the surface of damaged myelin and OLG, and in degraded myelin within macrophages ( 11 ). (jimmunol.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)
- C5b with C6, C7, C8, and C9 form the membrane attack complex that initiates cell lysis. (thefreedictionary.com)
- C5b binds sequentially to C6, C7, C8 and then to multiple molecules of C9 to form membrane attack complex . (wikipedia.org)
- These complement components form the final membrane attack complex (MAC). (thefreedictionary.com)
- C8 is one component of the membrane attack complex, which mediates cell lysis, and it initiates membrane penetration of the complex. (lsbio.com)
- This protein mediates the interaction of C8 with the C5b-7 membrane attack complex precursor. (lsbio.com)
- 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)
- CD59 is a potent inhibitor of the complement membrane attack complex, whereby it binds complement C8 and/or C9 during the assembly of this complex, thereby inhibiting the incorporation of multiple copies of C9 into the complex, which is necessary for osmolytic pore formation. (fishersci.com)
- 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)
- 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)
- The binding specificity between C8 beta and C8 alpha subunits is determined by a cooperative interaction of the N-terminal thrombospondin type 1 module and the membrane attack complex/ perforin (zeige PRF1 Antikörper ) domain. (antikoerper-online.de)
- C8 participates in the formation of Membrane Attack Complex that results in the lysis of cells. (antikoerper-online.de)
- 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)
- Complement component 7 serves as a membrane anchor. (sinobiological.com)
- Previous studies demonstrated that Ts -Pmy bound to complement components C8 and C9 and inhibited the polymerization of C9 during the formation of the membrane attack complex (MAC). (biomedcentral.com)
- CD59 is the key regulator that preserves the autologous cells from terminal effector mechanism of the complement cascade. (fishersci.com)
- The unique species specificity of the bacterial cytolysin intermedilysin is explained by its requirement for the human complement regulator CD59 as the primary receptor. (strath.ac.uk)
- We first looked for regions of homology between domain 4 in intermedilysin and the terminal complement components that bind CD59, C8 and C9. (strath.ac.uk)
- A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis. (abcam.com)
- The components of complement system can be organised into three major pathways: The classical pathway is mainly initiated by the binding of C1q to antigen-antibody complexes, whereas the lectin pathway is triggered by binding of mannose‐binding lectin ( MBL ) or ficolins to glycosylated surfaces on microbial cell walls. (els.net)
- 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 contributions of complement-dependent phagocytosis and serum bactericidal activity (SBA) to the killing of 62 strains of meningococci were examined by using C8-depleted or pooled human serum (PHS). (nih.gov)
- The complement-dependent nature of killing by neutrophils was confirmed by restoring survival to control values by using heated serum. (nih.gov)
- We also examined meningococcal phagocytosis by using serum from a C8-deficient patient. (nih.gov)
- Properdin is a serum glycoprotein that up-regulates the alternative pathway of complement by stabilizing the C3b-Bb complex. (embl.de)
- 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)
- If the serum contains antibody, i.e. is positive, an antibody-antigen complex is formed which also binds (fixes) complement. (thefreedictionary.com)
- Inhibition of the C8/C9 steps of complement lysis by a high density lipoprotein (HDL) of human serum. (yale.edu)
- 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)
- 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)
- The complement system is a complex cascade involving proteolytic cleavage of serum glycoproteins often activated by cell receptors. (sigmaaldrich.com)
- Human Complement C7 ELISA Kit is designed for detection of C7 in human plasma, serum, urine, milk, saliva, and cell culture supernatants. (innov-research.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)
- 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)
- Consistent with this notion, the terminal complement components (C5b, C6, C7, C8, and C9) are deposited more efficiently and abundantly on the surface of serum-sensitive strains of Borrelia spp. (asm.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)
- If antibody was not present in stage 1, then the free complement lyses the sensitized sheep red blood cells. (thefreedictionary.com)
- complement fixation the combining of complement with the antigen-antibody complex , rendering the complement inactive, or fixed. (thefreedictionary.com)
- Complement C8b antibody LS-C208192 is an FITC-conjugated rabbit polyclonal antibody to human Complement C8b. (lsbio.com)
- Complement C3b may be an opsonin for antigen-antibody complexes which helps prevent damage from the formation of large, insoluble immune aggregates. (sigmaaldrich.com)
- Subsequent binding of the antibody to complement C1q subunits of C1 result in catalytically active C1s subunits. (sigmaaldrich.com)
- In this study, a monoclonal antibody against the complement C9 binding domain of Ts -Pmy (mAb 9G3) was produced using hybridoma technology. (biomedcentral.com)
- mAb 9G3 is a specific antibody that binds to the C9 binding domain of Ts -Pmy and interferes with Ts -Pmy's complement-binding activity. (biomedcentral.com)
- 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)
- Complement dysregulation and deficiencies are connected to disease. (els.net)
- 2009) Complement in human diseases: Lessons from complement deficiencies. (els.net)
- 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)
- 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)
- 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)
- 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)
- 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. (wikipedia.org)
- Another complement protein, C6 , binds to C5b. (wikipedia.org)
- C8 binds to the C5B-7 complex, forming the C5B-8 complex. (abcam.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)
- 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)
- Howard OM, Rao AG, Sodetz JM: Complementary DNA and derived amino acid sequence of the beta subunit of human complement protein C8: identification of a close structural and ancestral relationship to the alpha subunit and C9. (drugbank.ca)
- Chromosomal assignment of genes encoding the alpha, beta, and gamma subunits of human complement protein C8: identification of a close physical linkage between the alpha and the beta loci. (medlineplus.gov)
- These fluid phase complexes do not bind to cell membranes and are ultimately scavenged by clusterin and vitronectin, two regulators of complement. (wikipedia.org)
- Similar hydrophobic sites on C8 and C9 molecules are exposed when they bind to the complex, so they can also insert into the bilayer. (wikipedia.org)
- The hydrolysis induces a conformational change that allows factor B to bind and form the alternative complement activation pathway C3 convertase (amplification loop is represented by the bold dotted arrow). (els.net)
- In the presence of vitronectin, the C5b-7 complex is still able to bind C8 and C9 to form C5b-8 and C5b-9 complexes but the latter is nonlytic. (jimmunol.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)
- Recently, properdin, a positive regulator of the alternative pathway of complement, has been shown to bind to surfaces and promote complement activation. (pubmedcentralcanada.ca)
- It is well recognized that B. burgdorferi expresses multiple lipoproteins on its surface that bind the human complement inhibitors factor H and factor H-like protein 1 (FH/FHL-1). (asm.org)
- The 150-kDa C8 protein consists of three non-identical subunits (C8alpha, C8beta, C8gamma), arranged as a disulfide linked C8alpha-gamma heterodimer non-covalently associated with C8beta. (sc.edu)
- Active MAC is composed of the subunits C5b, C6, C7, C8 and several C9 molecules. (wikipedia.org)
- Complement C8b is one of the three subunits of the complement component 8 (C8) protein. (lsbio.com)
- C8 is composed of equimolar amounts of alpha, beta and gamma subunits, which are encoded by three separate genes. (lsbio.com)
- 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)
- C8 beta is one of the three subunits that comprise the component 8 (C8) of the complement system. (antikoerper-online.de)
- Degn SE, Jensenius JC, Thiel S. Disease-causing mutations in genes of the complement system. (medscape.com)
- Complement lysis inhibitor and prototype domain in a new protein superfamily. (springer.com)
- A peptide containing this sequence caused dose-dependent inhibition of intermedilysin-mediated lysis of human erythrocytes and rendered erythrocytes more susceptible to complement lysis. (strath.ac.uk)
- C8 alpha and C8 beta have correspondingly similar roles in MAC -mediated lysis of erythrocytes and bacterial killing. (antikoerper-online.de)
- In the first phase, a series of specific interactions leads to formation of intrinsic complement proteinase, termed C3 convertase. (medscape.com)
- 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)
- 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)
- Complement activation pathways. (els.net)
- 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)
- Complement activation is involved in the initiation of Ab-mediated inflammatory demyelination in experimental autoimmune encephalomyelitis (EAE). (jimmunol.org)
- Nontypeable Haemophilus influenzae (NTHi) commonly causes local disease in the upper and lower respiratory tract and has recently been shown to interfere with both the classical and alternative pathways of complement activation. (jimmunol.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)
- Complement activation also results in the formation of many biologically active complement fragments that act as anaphylatoxins, opsonins, or chemotactic factors. (thefreedictionary.com)
- Complement activation is tightly regulated and designed to kill invading microbes while producing minimal "collateral damage" that could result in the destruction of host tissues. (primaryimmune.org)
- Complement activation is a cascading event like the falling of a row of dominoes. (primaryimmune.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)
- Other deleterious effects of complement activation include, degranulation of neutrophils, basophils and mast cells, unwanted release of the neutrophil products elastase and oxygen radicals, and extracorporeal blood circulation. (sigmaaldrich.com)
- Activation of complement represents one means of natural resistance to infection from a wide variety of potential pathogens. (pubmedcentralcanada.ca)
- Here we studied whether properdin-mediated complement activation occurs on the surface of Chlamydia pneumoniae , an obligate intracellular Gram-negative bacterium that causes 10 to 20% of community-acquired pneumonia. (pubmedcentralcanada.ca)
- The binding of these physiological forms accelerates complement activation on the Chlamydia pneumoniae surface, as measured by C3b and C9 deposition. (pubmedcentralcanada.ca)
- The complement system is a central component of the innate immune response and is involved in many functions, including recognition, opsonization, phagocytosis, and destruction of foreign cells, as well as generation of chemotactic fragments (C3a and C5a) and activation of adaptive immunity ( 30 , 32 , 51 ) Three pathways of complement activation are known: the classical, lectin, and alternative pathways. (pubmedcentralcanada.ca)
- Moreover, the mechanisms involved in alternative pathway-mediated complement activation or its consequences on infection in C. pneumoniae remain unknown. (pubmedcentralcanada.ca)
- Auf www.antikoerper-online.de finden Sie aktuell 31 Complement Component 8, beta Polypeptide (C8B) Antikörper von 13 unterschiedlichen Herstellern. (antikoerper-online.de)
- however, susceptibility to complement-dependent phagocytosis and complement-mediated SBA of serogroups B and Y did not correlate. (nih.gov)
- This finding suggests that vaccinating individuals deficient in late-complement components may shift the burden of host defense from SBA to phagocytosis. (nih.gov)
- What are the two pathways that activate the complement protein cascade? (brainscape.com)
- How does the complement cascade help clear microorganisms? (brainscape.com)
- This gene encodes a component of the complement cascade. (genecards.org)
- The complement system functions as an interactive sequence, with one reaction leading to another in the form of a cascade. (medscape.com)
- Ischaemic infarction may also cause initiation of the complement cascade. (sigmaaldrich.com)
Component of complement1
- Leiner's disease associated with diminished third component of complement. (medscape.com)
- 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)
- C8 H10 N4 O2 is Caffeine in Molecular formula. (e-lactancia.org)
- Human Complement Component 7 (C7) is a single-chain glycoprotein consisting of 821 amino acid residues with a molecular weight of about 97 kDa. (innov-research.com)
- C8 gamma is not required for complement-mediated killing of Gram-negative bacteria. (antikoerper-online.de)
- Complement component 8 aids in a part of the body's immune response known as the complement system. (medlineplus.gov)
- Among its related pathways are Complement and coagulation cascades and Immune response Lectin induced complement pathway . (genecards.org)
- The complement system can be recruited and brought into action by antibodies generated by the adaptive immune system . (wikipedia.org)
- 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 is crucial for defense against pathogens and the removal of dying cells or immune complexes. (frontiersin.org)
- meningococcal or pneumococcal infections and auto-immune diseases are adverse events of anti-complement therapy. (frontiersin.org)
- Functional complement C1q abnormality leads to impaired immune complexes and apoptotic cell clearance. (medscape.com)
- Cytolytic lymphocyte clones and complement as effectors of the immune system. (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 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)
- 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 inhibitors are being studied as potential therapeutics for immune diseases and Alzheimer's. (sigmaaldrich.com)
- During this journey, from circulating cells to endothelial cells, C. pneumoniae encounters the innate immune system, in which the complement system may play a fundamental role in controlling Chlamydia infection. (pubmedcentralcanada.ca)
- The C8B gene provides instructions for making one piece, the beta subunit, of a protein complex called complement component 8. (medlineplus.gov)
- 2009) C7 is expressed on endothelial cells as a trap for the assembling terminal complement complex and may exert anti-inflammatory function. (sinobiological.com)
- Complement participates in innate and adaptive immunity. (els.net)
Positive regulator of the alternative complem1
- higher urine factor H-related protein 2, a positive regulator of the alternative complement pathway, was associated with greater risk of death (HR [95% CI] 1.61 [1.05-2.48]) in fully adjusted models. (diabetesjournals.org)
- A unique feature of the AP is the presence of the only positive regulator in the complement system, Properdin. (primaryimmune.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)
- The Alternative Pathway (AP) is initiated by fragments of the complement component C3. (primaryimmune.org)
- It activates C3 of the complement system and probably supports inflammation in other ways as well. (biology-pages.info)
- All three pathways of the complement system ( classical , lectin and alternative pathways) initiate the formation of MAC. (wikipedia.org)
Eighth component of human complement2
- However, the N-terminal portion (positions 24-105) has no sequence similarity with those of mammalian alpha 2Ms, and it is structurally related to that of the human complement factor C8 chain, consistent with a role for Limulus alpha 2M in host defense. (nih.gov)
- Dalmasso, A.P. Complement in the pathophysiology and diagnosis of human diseases. (springer.com)
- The binding of mAb 9G3 to Ts -Pmy efficiently blocked the binding of Ts -Pmy to human complement C9, resulting in a significant increase in the complement-mediated killing of newborn larvae in vitro and reduced infectivity of T. spiralis larvae in mice passively transferred with the mAb. (biomedcentral.com)
- Therefore, many pathogens have evolved different mechanisms to avoid complement-mediated killing. (jimmunol.org)
- Another frequent strategy used by some pathogens is binding of complement inhibitors such as vitronectin, C4b-binding protein (C4BP), and factor H, which all protect from the complement-mediated attack. (jimmunol.org)
- The alternative pathway is one of three complement pathways that opsonize and kill pathogens. (wikipedia.org)
- Three biochemical pathways activate the complement system: the classical complement pathway , the alternative complement pathway , and the lectin pathway . (wikipedia.org)
- Ehrlich believed that each antigen-specific amboceptor has its own specific complement, whereas Bordet believed that there is only one type of complement. (wikipedia.org)
- Age related macular degeneration (AMD) is now believed to be caused, at least in part, by complement mediated attack on ocular tissues . (wikipedia.org)
- Dysregulation of complement activity has, therefore, been connected to various diseases, including autoimmune conditions, thrombotic pathologies and infections. (els.net)
- 2001). "The complement system and innate immunity" . (wikipedia.org)
- The terminal pathway of the complement system is regulated by vitronectin that is a component of both plasma and the extracellular matrix. (jimmunol.org)
- Hofsteenge J, Blommers M, Hess D, Furmanek A, Miroshnichenko O: The four terminal components of the complement system are C-mannosylated on multiple tryptophan residues. (drugbank.ca)
- Complement components C6, C7, C8 and C9. (ebi.ac.uk)
- Complement C1r/C1s components (1 copy). (yale.edu)
- various complement components may be deficient without serious effects on the host. (thefreedictionary.com)
- Additionally, the deposition of complement components C3, C6, and C5b-9 was enhanced on the surface of the CspA mutant compared to that of the wild-type strain. (asm.org)