Complement C3
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.
Complement C4
Complement C4a
Complement C3a
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.
Complement C1q
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.
Complement C5a
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.
Complement Activation
Complement C4b
Complement C5
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.
Complement C3b
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).
Complement C6
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.
Complement C3c
Complement C3d
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).
Complement C2
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).
Complement C9
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.
Receptors, Complement
Complement C1s
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.
Complement C1r
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.
Complement C7
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.
Complement C8
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.
Complement C1
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).
Complement C5b
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.
Complement C2a
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.
Anaphylatoxins
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.
Antigens, CD55
Complement C3-C5 Convertases, Classical Pathway
Complement C2b
Antigens, CD59
Cobra Venoms
Antigen-Antibody Complex
Steroid 21-Hydroxylase
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.
Immunoglobulin G
Hemolysis
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
Antigens, CD46
Opsonin Proteins
Blood Proteins
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
Phagocytosis
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.
Properdin
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
Macrophage-1 Antigen
Protein Binding
Neutrophils
Base Sequence
Kidney Glomerulus
Serum
Glomerulonephritis, Membranoproliferative
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.
Immunoglobulin M
Schistosoma
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.
Mice, Knockout
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.
Glomerulonephritis
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.
Arteriolosclerosis
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.
Erythrocytes
Autoantibodies
Cells, Cultured
RNA, Messenger
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.
Macrophages
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.)
Immunity, Innate
Peptide Fragments
Mutation
Rabbits
Disease Models, Animal
Cloning, Molecular
Binding Sites
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.
Antigens, CD
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
Mannose-Binding Lectin
Alleles
Antibodies
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.
Glycoproteins
Immunoglobulins
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.
Haptoglobins
DNA
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.
Peptides, Cyclic
Lupus Nephritis
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).
Antibodies, Antinuclear
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
Blotting, Western
Cosmids
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
Biological Markers
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.
Inflammation
Carrier Proteins
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.
Species Specificity
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.
Kidney
Phenotype
Immunologic Factors
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.
Immunohistochemistry
Gene Dosage
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.
Haplotypes
Membrane Proteins
HLA Antigens
Sequence Homology, Nucleic Acid
Gene Expression
Monocytes
Fibrinogen
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.
Exons
B-Lymphocytes
Flow Cytometry
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.
Antibody Formation
Serine Endopeptidases
Streptococcus pneumoniae
Collectins
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.
Restriction Mapping
Genes
DNA Primers
C-Reactive Protein
Genotype
Up-Regulation
Lipopolysaccharides
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)
Steroid Hydroxylases
Blotting, Northern
T-Lymphocytes
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.
DNA, Complementary
Blotting, Southern
Cytokines
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.
Macular Degeneration
Disease Susceptibility
Models, Molecular
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
Cell Membrane
Pedigree
Case-Control Studies
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
Gene Frequency
Guinea Pigs
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.
Escherichia coli
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.
Immunoelectrophoresis
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.
Staphylococcus aureus
Transfection
Liver
Lung
Arthritis, Rheumatoid
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.
Interleukin-6
Protein Conformation
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).
Epithelial Cells
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.
Structure-Activity Relationship
Gene Library
Signal Transduction
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
Hemoglobinuria, Paroxysmal
A condition characterized by the recurrence of HEMOGLOBINURIA caused by intravascular HEMOLYSIS. In cases occurring upon cold exposure (paroxysmal cold hemoglobinuria), usually after infections, there is a circulating antibody which is also a cold hemolysin. In cases occurring during or after sleep (paroxysmal nocturnal hemoglobinuria), the clonal hematopoietic stem cells exhibit a global deficiency of cell membrane proteins.
Polymorphism, Single Nucleotide
Immune Complex Diseases
Group of diseases mediated by the deposition of large soluble complexes of antigen and antibody with resultant damage to tissue. Besides SERUM SICKNESS and the ARTHUS REACTION, evidence supports a pathogenic role for immune complexes in many other IMMUNE SYSTEM DISEASES including GLOMERULONEPHRITIS, systemic lupus erythematosus (LUPUS ERYTHEMATOSUS, SYSTEMIC) and POLYARTERITIS NODOSA.
Combined genetic deficiency of C6 and C7 in man. (1/83)
By routine screening of sera, a subject was discovered who showed a sub-total deficiency of C6 and C7. No clinical disease was associated with this deficiency which was transmitted through the subject's family as a single genetic characteristic, the C6 deficiency being associated with a silent allele at the structural locus. The propositus was found to have low quantities of an abnormal C6 which was both antigenically deficient and smaller in size than normal C6 (110,000 daltons compared with 140,000 daltons) and small quantities of apparently normal C7. It is concluded that the most likely explanation for this defect is that the subject has a structural mutation in his C6 gene which produces hyopsynthesis not only of C6 but also of the closely linked gene for C7. These findings suggest the possibility that C6 and C7 may function as a single genetic unit and that the primary transcript copied from the genome includes information for both proteins. (+info)In vitro and in vivo responses of murine granulocytes to human complement-derived, haemolytically inactive C5b67 (iC5b67). (2/83)
Haemolytically inactive C5b67 (iC5b67), which was made from purified human components and decayed to a haemolytically inactive form, was evaluated as an agonist for murine leucocytes both in vitro and in vivo. In an in vitro assay, iC5b67 stimulated chemotaxis for both neutrophils purified from mouse bone marrow and splenic eosinophils of IL-5 transgenic mice. The stimulation was dose-dependent, with high dose inhibition. As with human neutrophils, iC5b67 also failed to up-regulate CR3 (CD11b/CD18) expression and to stimulate superoxide generation in murine bone marrow neutrophils, in vitro. In vivo, iC5b67 elicited an inflammatory response in a mouse model of pleuritis. A marked infiltration of neutrophils, which peaked at 4 h, was followed by an infiltration of eosinophils and mononuclear leucocytes. This inflammatory response was dose- and time-dependent. However, the protein concentration in the pleural wash fluid did not increase, indicating that iC5b67 did not induce a capillary leak. Although the infiltration of neutrophils could not be reproduced by pure C7 or human serum albumin (HSA), C5b6 did induce an influx of neutrophils. We were able to document the existence of C7, both antigenically and functionally, in pleural washes of normal mice, making it likely that the activity of C5b6 resulted from the in situ formation of C5b67 and iC5b67. The mouse model of pleuritis promises to be a useful in vivo system in which to evaluate the pro- and anti-inflammatory effects of iC5b67 that have been noted in vitro. (+info)On the mechanism of cytolysis by complement: evidence on insertion of C5b and C7 subunits of the C5b,6,7 complex into phospholipid bilayers of erythrocyte membranes. (3/83)
The doughnut hypothesis of cytolysis by complement [Mayer, M. M. (1972) Proc. Nat. Acad. Sci. USA 69, 2954-2958] describes an annular structure made up of C5b-9 (complement factors C5b, C6, C7, C8, and C9) which becomes inserted in the lipid bilayer of the cell membrane, thus creating a hole. We now present initial explorations of this hypothesis. EAC1-6 and EAC1-7 (sheep erythrocytes carrying rabbit antibody and complement factors C1 through C6 or C1 through C7, respectively), prepared with either 125I-C3 or 125I-C5 were incubated with trypsin and the release of bound 125I was measured. In the case of 125I-C3, all of the radioactivity was released by trypsin from both intermediates. With 125I-C5, trypsin released all of the 125I from EAC1-6, but only 40-55% from EAC1-7. Possible reasons for resistance of the C5b subunit in EAC1-7 to tryptic digestion are discussed; in terms of the doughnut hypothesis it would be due to shielding by lipid molecules as a consequence of insertion into the lipid bilayer. In accord with this interpretation we have also found that C5b in EAC1-7, but not in EAC1-6, resists elution by 0.3 M NaC1. Similarly, we have found that 125I-C7 in EAC1-7 resists stripping by trypsin. Hence, we now propose the hypothesis that hydrophobic polypeptide chains from the C5b and the C7 subunits of C5b,6,7 complex become inserted in the phospholipid bilayer and that subsequent reactions with C8 and C9 open a channel across the membrane. (+info)Increased ion permeability of planar lipid bilayer membranes after treatment with the C5b-9 cytolytic attack mechanism of complement. (4/83)
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)Isolation, characterization, and cloning of porcine complement component C7. (5/83)
Activation of the complement system through the classical, alternative, or lectin pathway results in the formation of the terminal complement complex. C7 plays an integral role in the assembly of this complex with target cell membranes. To date, only human C7 has been cloned and characterized; thus, in this study, we characterized the porcine complement component C7. Porcine C7 was isolated by affinity chromatography as a single glycoprotein with an approximate molecular mass of 90 kDa and 100 kDa under reducing and nonreducing conditions, respectively. The full-length porcine C7 cDNA was isolated, and the predicted amino acid sequence exhibited 80% identity with human C7 with conservation of the cysteine backbone and two putative N-linked glycosylation sites. Porcine C7 mRNA expression was detected in all tissues investigated, except polymorphonuclear and mononuclear leukocytes. Addition of purified porcine C7 restored the hemolytic activity of C7-depleted human sera in a dose-dependent manner. A functionally inhibitory mAb against porcine C7 attenuated the hemolytic activity of human, rabbit, or rat sera, suggesting an important conserved C7 epitope among species. These data demonstrate that porcine and human C7 are highly conserved, sharing structural and functional characteristics. (+info)The endothelium is an extrahepatic site of synthesis of the seventh component of the complement system. (6/83)
The level of the terminal complement components secreted by human umbilical vein endothelial cells (HUVEC) was measured by a sensitive ELISA which allows the detection of 30-50 pg/ml of these components. C7 was the only terminal component detected in measurable amounts in the cell supernatant. The mean value was 11 ng/106 cells at 96 h and was slightly higher than that of C3 (9 ng/106 cells). HUVEC and serum C7 analysed by SDS-PAGE and immunoblot exhibited the same electrophoretic mobility. A proportion of C7 secreted by HUVEC was incorporated into the terminal complement complex (TCC) assembled spontaneously in the supernatant of cells cultured in C7-deficient human serum, and was not detected by the standard ELISA for C7 measurement. By adding the amount of C7 present in the TCC to that of free C7, the total amount of the component released by HUVEC was calculated to be approximately 35 ng/106 cells. Further TCC was produced following complement activation of the cell supernatant through the alternative pathway. Synthesis of C7 by HUVEC was confirmed by inhibition experiments in the presence of cycloheximide and by reverse transcriptase-polymerase chain reaction (RT-PCR) analysis of C7 mRNA expression. Addition of IL-1alpha and tumour necrosis factor-alpha to the cell culture stimulated the secretion of C3, but had no effect on the synthesis of C7. By contrast, interferon-gamma had only a marginal effect on the production of C3, but markedly down-regulated the synthesis of C7 as assessed both by ELISA and RT-PCR. (+info)Hereditary deficiency of the seventh component of complement. (7/83)
Deficiency of the seventh component of complement has been found in the serum of a 42-yr-old Caucasian woman who has Raynaud's phenomenon, sclerodactyly, and telangiectasia. Partial deficiency was found in the serum of the patient's parents and children, indicating a pattern of inheritance of autosomal codominance. Transfusion experiments indicated that exogenous C7 had a 91-h halk-life in the patient. There was no evidence for C7 synthesis after transfusion. No C7 inhibitors were detected in the patient's serum. The patient's serum was found to support the activation of complement by both the classical and properdin pathways to the C7 stage. The addition of C7 to the patient's serum permitted it to support hemolytic reactions initiated by either pathway. No defects could be detected in plasma or whole blood coagulation. The patient's serum was deficient in opsonizing unsensitized yeast particles in serum and in the generation of chemotactic factor by antigen-antibody complexes and endotoxin. Both deficiencies were corrected by the addition of C7. These observations suggest a key role for C7 for in vitro yeast phagocytosis and chemotaxis generation. However, the patient's lack of infections indicates a relatively minor role for C7 in human resistance to infection. (+info)Hypochlorite-induced alterations to canine serum complement. (8/83)
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)
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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 ...
Mosaic protein
... and complement components C6 and C7". Immunogenetics. 56 (2): 89-106. doi:10.1007/s00251-004-0665-2. ISSN 0093-7711. PMID ...
Graph homomorphism
... then H would be the complement graph of C7. A graph homomorphism from G to H is then a schedule assigning courses to time slots ... An L(2,1)-coloring is a homomorphism into the complement of the path graph that is locally injective, meaning it is required to ...
MACPF
Complement proteins C6-C9 all contain a MACPF domain and assemble into the membrane attack complex. C6, C7 and C8β appear to be ... proteins of the complement system (C6, C7, C8α, C8β and C9) and perforin (PF). Members of this protein family are pore-forming ... 3OJY Complement regulatory proteins such as CD59 function as MAC inhibitors and prevent inappropriate activity of complement ... "Nonsense mutation in exon 4 of human complement C9 gene is the major cause of Japanese complement C9 deficiency". Hum. Genet. ...
Eosinophilia
... complement complex (C5-C6-C7), interleukin 5, and histamine (though this has a narrow range of concentration). Harm resulting ...
Outline of immunology
C6 C7 C8 C9 Complement pathway inhibitors C1-inhibitor - Classical, Lectin, Alternate Decay-accelerating factor (CD59) - ... system Complement system Classical complement pathway Mannan-binding lectin pathway Alternate complement pathway Complement ... see complement proteins section) Collectins Mannan-binding lectin (MBL) Surfactant protein A (SP-A) Surfactant protein D (SP-D ... Classical complement pathway C1Q complex - C1R / C1S C4 - C4a C2 Mannan-binding lectin pathway MASP1 / MASP2 Mannan-binding ...
Alternative complement pathway
C5b binds sequentially to C6, C7, C8 and then to multiple molecules of C9 to form membrane attack complex. ... there are several different kinds of regulatory proteins that disrupt the complement activation process: *Complement Receptor 1 ... Complement Factor H can inhibit the formation of the C3 convertase by competing with factor B for binding to C3b;[1] accelerate ... "Inhibition of the alternative complement pathway by antisense oligonucleotides targeting complement factor B improves lupus ...
Complement membrane attack complex
MAC is composed of a complex of four complement proteins (C5b, C6, C7, and C8) that bind to the outer surface of the plasma ... This junction alters the configuration of the protein molecules exposing a hydrophobic site on C7 that allows the C7 to insert ... Media related to Complement membrane attack complex at Wikimedia Commons. *Complement+Membrane+Attack+Complex at the US ... The membrane attack complex (MAC) or terminal complement complex (TCC) is a structure typically formed on the surface of ...
Complement component 7
... is a protein involved in the complement system of the innate immune system. C7 is part of the membrane ... Terminal complement pathway deficiency Complement+C7 at the US National Library of Medicine Medical Subject Headings (MeSH) ... This junction alters the configuration of the protein molecules, exposing a hydrophobic site on C7 that allows the C7 to insert ... Human Complement component C7) at the PDBe-KB. GRCh38: Ensembl release 89: ENSG00000112936 - Ensembl, May 2017 "Human PubMed ...
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 ...
Terminal complement pathway deficiency
C7, and C8. (While C9 is part of the MAC, and deficiencies have been identified, it is not required for cell lysis.) People ... Terminal complement pathway deficiency is a genetic condition affecting the complement membrane attack complex (MAC). It ... Initial complement tests often include C3 and C4, but not C5 through C9. Instead, the CH50 result may play a role in diagnosis ... Suspect terminal complement pathway deficiency with patients who have more than one episode of Neisseria infection. ...
Complement system
C7, C8, and polymeric C9.[7] 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 ...
Complement system
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 ...
C5-convertase
The binding of C5 is influenced by C6 and C7, components which are thought to act subsequent to it in the complement sequence. ... The complement component C5 can be also activated by fluid phase C5 convertase. C5 is activated by CVFBb in the presence of ... The target of C5 convertase is complement protein C5. C5 is a two-chain (α, β) plasma glycoprotein (Mr = 196,000). C5 and C3 ... In these respects, the mode of action of C5 is completely analogous to that of the other components of complement. The C5 step ...
C7
... engineered transcription factors Complement component 7, protein encoded by the C7 gene in humans Coccinella septempunctata ("C ... C7, C07 or C-7 may refer to: C-7 Caribou, a military transport aircraft AEG C.VII, a World War I German armed reconnaissance ... "C7" (song), a song by Japanese band GO!GO!7188 C7 Sport, a defunct pay-TV service in Australia C7, the C (musical note) three ... Brazil Colt Canada C7 rifle or Diemaco C7, a Canadian derivative of the M16 C7, an international standard paper size (81×114 mm ...
Complement component 1s
... (EC 3.4.21.42, 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 ...
Autoimmune hemolytic anemia
Cases may also arise with complement alone or with IgA, IgM or a combination of these three antibody classes and complement. ... C7, 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 ...
Cylindric algebra
... set complement for complement, empty set as 0, X α {\displaystyle X^{\alpha }} as the unit, and ⊆ {\displaystyle \subseteq } ... C7) If κ ≠ λ {\displaystyle \kappa \neq \lambda } , then c κ ( d κ λ ⋅ x ) ⋅ c κ ( d κ λ ⋅ − x ) = 0 {\displaystyle c_{\kappa ... C7) If κ {\displaystyle \kappa } and λ {\displaystyle \lambda } are different variables, then ∃ κ . ( κ = λ ∧ x ) ∧ ∃ κ . ( κ ... It necessarily validates the axioms C1-C7 of a cylindric algebra, with ∪ {\displaystyle \cup } instead of + {\displaystyle ...
Primary immunodeficiency
Complement deficiencies are the result of a lack of any of these proteins. They may predispose to infections but also to ... C7 deficiency (idem, vasculitis) C8a deficiency C8b deficiency C9 deficiency (Neisserial infections) C1-inhibitor deficiency ( ... The complement system is part of the innate as well as the adaptive immune system; it is a group of circulating proteins that ... MASP2 deficiency Complement receptor 3 (CR3) deficiency Membrane cofactor protein (CD46) deficiency Membrane attack complex ...
List of primary immunodeficiencies
Complement deficiencies are the result of a lack of any of these proteins. They may predispose to infections but also to ... C7 deficiency (idem, vasculitis) C8a deficiency C8b deficiency C9 deficiency (Neisserial infections) C1-inhibitor deficiency ( ... Cherubism COPA defect Otulipenia/ORAS The complement system is part of the innate as well as the adaptive immune system; it is ... MASP2 deficiency Complement receptor 3 deficiency Membrane cofactor protein (CD46) deficiency Membrane attack complex inhibitor ...
C3b
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 ...
Glossary of chemical formulae
Contents A B C Ca-Cu D E F G H I K L M N O P R S T U V W Y Z External links C C2 C3 C4 C5 C6 C7 C8 C9 C10 C15 C20 Webelements ... This complements alternative listing at list of inorganic compounds. There is no complete list of chemical compounds since by ...
List of MeSH codes (D12.776.124)
... complement c5b MeSH D12.776.124.486.274.550 - complement c6 MeSH D12.776.124.486.274.650 - complement c7 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. ...
Sushi domain
Complement decay-accelerating factor (Antigen CD55) belongs to the Cromer blood group system and is associated with Cr(a), Dr(a ... C7, CD46, CD55, CFB, CFH, CFHR1, CFHR2, CFHR3, CFHR4, CFHR5, CR1, CR1L, CR2, CSMD1, CSMD2, CSMD3, CSPG3, DAF, F13B, FHR4, ... Complement receptor type 1 (C3b/C4b receptor) (Antigen CD35) belongs to the Knops blood group system and is associated with Kn( ... Complement components may activate B cells through CD21. CD21 is part of a large signal-transduction complex that also involves ...
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 ...
5.56×45mm NATO
Cartridge, Ball, C77: 5.56×45mm FN SS109 equivalent used in the C7, C8 and C9 type weapons. Made by General Dynamics Canada. ... Round, 5.56mm Tracer, L1A1/A2 [Red tip]: Tracer round complement to L2A1/A2, produced by Radway Green. Round, 5.56mm Tracer, ... Cartridge, Blank, C79 [Crimped tip]: 5.56×45mm blank cartridge used in the C7, C8 and C9 type weapons. Also made by General ... Patrone AA63, 5.56×45mm, DM21, Leuchtspur (Tracer) [Orange tip]: 5.56×45mm tracer complement to DM11, also produced by RUAG ...
Netherlands Marine Corps
The mess dress uniform is worn during formal occasions, such as a dinner or a ball and consists of a smoking, complemented with ... The basic infantry weapons of the Korps Mariniers are the Colt Canada C7 assault rifle, Colt Canada C8 carbine and Glock 17 ... Marine with Colt Canada C7 during the Capacity Building Mission Iraq (CBMI). DMV Anaconda off-road vehicle during tests before ...
Audi S6
The Audi S6 4.0 TFSI quattro and its mechanical twin S7 were launched as a 2013 models, a year after the C7 Audi A6 and Audi A7 ... A new Audi S6, now officially known as the Audi S6 quattro appeared in 1999, to complement its A6 platform-mate. It was ... The S6 5.2 FSI was discontinued after the 2011 model year, shortly before the release of the 2012 Audi A6 (C7). For the sprint ... Bi-Xenon high-intensity discharge (HID) adaptive headlights which swivel around corners complement the LED DRLs. The S6 Was ...
Komplemendisüsteem - Vikipeedia
Zipfel, P. F., Hallström, T., & Riesbeck, K. (2013). Human complement control and complement evasion by pathogenic microbes- ... C7, 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. ...
Vitamin C
InChI=1S/C6H8O6/c7-1-2(8)5-3(9)4(10)6(11)12-5/h2,5,7-10H,1H2/t2-,5+/m0/s1 Y ... "Toward a phylogenetic classification of Primates based on DNA evidence complemented by fossil evidence". Molecular ...
Jewel (supermarket)
p. C7.. Alternate Link via ProQuest.. *^ "Jewel Tea Acquires Eisner". Wall Street Journal. March 14, 1957. p. 4. (Subscription ... The acquisition of both Osco and Turn Style allowed Jewel to expand into non-food related retailing that would complement their ... p. C7.. Alternate Link via ProQuest.. *^ "Star Market Co. To Make Exchange Offer to Brigham's". Boston Globe. November 8, 1961 ... p. C7.. Alternate Link via ProQuest.. *^ "Venture to acquire 19 stores of Turn Style". WWD. 136 (46). March 8, 1978. p. 54. ...
Eosinophilia
... complement complex (C5-C6-C7), interleukin 5, and histamine (though this has a narrow range of concentration).[3] ...
The Real Adventures of Jonny Quest
Complementing the show's educational, real-world premise, the site hosted links to academic, archaeological, and exploratory ... C7.. ...
Dietary fiber
replace or complement in some plant taxa the starch as storage carbohydrate ... Diarylheptanoids (C6-C7-C6). *Anthraquinones. *Chalconoids (C6-C3-C6). *Kavalactones. *Naphthoquinones (C6-C4) ...
C5a receptor
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)[1] 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 ...
Basigin
... 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).[9][10] 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.[6] 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.[11][12][13] 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 ...
mTOR inhibitors
... methoxy group at C-7, and methyl groups at C-33, C-27 and C-25. All changes of the macrolide ring can have unpredictible ...
FernGully: The Last Rainforest
p. C7. ISSN 1197-2823. Rickitt, Richard (2000). Special Effects: The History and Technique. Billboard Books. p. 147. ISBN 0- ... Hand drawn scenes in the film were complemented by computer animation, which was used to create elements such as flocks of ...
Thumb
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 radial nerve (C7-C8). The extensor pollicis longus (EPL) originates on the dorsal ... It is innervated by the deep branch of the radial nerve (C7-C8). The extensor pollicis brevis (EPB) originates on the ulna ...
South Vietnam Air Force
The C-130 could carry more cargo than any of the types his nation would receive with 5 times the cargo capacity of a C-7 or ... By May 1969 the full complement of 54 A-37B jets was on hand and assigned to the 524th, 520th, and 516th Fighter Squadrons. The ... The USAF would accelerate the delivery of 14 RC-47s, 23 AC-119K gunships, 23 EC-47s, 28 C-7 transports and 14 C-119Gs modified ... The RVNAF 33rd and 53rd Tactical Wings were established flying C-123 Providers, C-47s and C-7 Caribous. As C-119 pilots began ...
List of OMIM disorder codes
PSAP Complement component 4, partial deficiency of; 120790; C1NH Complement factor H deficiency; 609814; HF1 Complement factor ... C6 C7 deficiency; 610102; C7 Caffey disease; 114000; COL1A1 Campomelic dysplasia with autosomal sex reversal; 114290; SOX9 ...
Royal Canadian Mounted Police
Remington 870 12-gauge shotgun Colt Canada C7 rifle (5.56mm NATO) Colt Canada C8 carbine (5.56mm NATO) - Adopted by ERT in ... to complement the Mounted Police capbadge and avoid using a Canadian coin that would deface the monarch. The RCMP last issued ...
Madrid Metro
... which ends at the Aravaca station providing a fast entry into Madrid though the C-7 or C-10 commuter lines and arriving in only ... the 8400 series are the newest train type to enter service on the Madrid Metro on line 6 since 2010 to complement the older ...
Laws of Form
By virtue of J1a, it is a complemented lattice whose upper bound is . By J0, is the corresponding lower bound and identity ... C7' in LoF, enables an algorithm, sketched in LoFs proof of T14, that transforms an arbitrary primary algebra formula to an ... His notation was isomorphic to that of LoF: concatenation read as conjunction, and "non-(X)" read as the complement of X. ... A primary algebra variable indicates a location where one can write the primitive value or its complement . Multiple instances ...
American Civil War fortifications in Louisville
It was complemented with ten additional artillery pieces. It also featured a caponier battery, built in the October 1864. Fort ... Civil War Engineering and Navigation www.usace.army.mil/usace-docs/misc/un22/c-7.pdf pg. 109-113 Civil War Engineering and ...
Human Complement C7 ELISA Kit (ab125964) | Abcam
Abcams Complement C7 ELISA Kit suitable for Cell culture supernatant, Saliva, Milk, Urine, Serum, Plasma, Cerebral Spinal ... Defects in C7 are a cause of complement component 7 deficiency (C7D) [MIM:610102]. A rare defect of the complement classical ... Belongs to the complement C6/C7/C8/C9 family.. Contains 1 EGF-like domain.. Contains 1 LDL-receptor class A domain.. Contains 1 ... A Complement C7 specific antibody has been precoated onto 96-well plates and blocked. Standards or test samples are added to ...
Complement C7
- Complement 7
Summary Report | CureHunter
In the formation of MEMBRANE ATTACK COMPLEX; C7 is the next component to bind the C5b-6 complex forming a trimolecular complex ... A 93-kDa serum glycoprotein encoded by C7 gene. It is a polypeptide chain with 28 disulfide bridges. ... C5b-7 which is lipophilic, resembles an integral membrane protein, and serves as an anchor for the late complement components, ... Complement 7; Complement Component 7; C7 Complement; C7, Complement; Complement, C7; Component 7, Complement ...
Complement C7 antibody | acris-antibodies.com
C7 gRNA vector 1 in pCas-Guide vector- KN209562G2, C7 gRNA vector 2 in pCas-Guide vector- KN209562D, donor vector… ... Background of Complement C7 antibody. Kit Component:. - KN209562G1, C7 gRNA vector 1 in pCas-Guide vector. - KN209562G2, C7 ... Western Blot analysis of C7 expression in transfected 293T cell line (H00000730-T01) by C7 MaxPab polyclonal antibody.Lane1:C7 ... Western Blot analysis of C7 expression in transfected 293T cell line (H00000730-T01) by C7 MaxPab polyclonal antibody.Lane1:C7 ...
The membrane attack complex of complement: relation of C7 to the metastable membrane binding site of the intermediate complex...
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 ... The membrane attack complex of complement: relation of C7 to the metastable membrane binding site of the intermediate complex ... The membrane attack complex of complement: relation of C7 to the metastable membrane binding site of the intermediate complex ... The membrane attack complex of complement: relation of C7 to the metastable membrane binding site of the intermediate complex ...
Anti-Complement C7 Antibody | Rabbit anti-Rat Polyclonal FITC | LSBio
Complement C7 antibody LS-C691310 is an FITC-conjugated rabbit polyclonal antibody to rat Complement C7. Validated for IHC and ... Complement C7 antibody LS-C691310 is an FITC-conjugated rabbit polyclonal antibody to rat Complement C7. Validated for IHC and ... The antibody is a rabbit polyclonal antibody raised against C7. It has been selected for its ability to recognize C7 in ...
Human Complement C7 ELISA Kit - Innovative Research
Human Complement C7 ELISA Kit is designed for detection of C7 in human plasma, serum, urine, milk, saliva, and cell culture ... Human Complement C7 ELISA Kit. Human Complement C7 ELISA Kit is designed for detection of C7 in human plasma, serum, urine, ... The amino-terminal of C7 has 23 - 30 homology with Complements C8 and C9 (1). C7 is one of five complement proteins (C5b, C6, ... Human Complement Component 7 (C7) is a single-chain glycoprotein consisting of 821 amino acid residues with a molecular weight ...
Complement component C7
... The C7 gene encodes a complement component involved in the membrane attack complex. Mutations cause ... al. (2006) Molecular defects of the C7 gene in two patients with complement C7 deficiency. ... None (1984) Genetic polymorphism and linkage of the sixth and seventh complement components (C6 and C7) in the common marmoset. ... Alvarez V et al. (1995) Genetic detection of the silent allele (*Q0) in hereditary deficiencies of the human complement C6, C7 ...
Self-association of the seventh component of human complement (C7): Dimerization and polymerization<...
C7): Dimerization and polymerization. Together they form a unique fingerprint. * Complement C7 Medicine & Life Sciences ... Preissner, K. T. ; Podack, E. R. ; Muller-Eberhard, H. J. / Self-association of the seventh component of human complement (C7 ... Self-association of the seventh component of human complement (C7): Dimerization and polymerization. Journal of Immunology. ... Preissner, KT, Podack, ER & Muller-Eberhard, HJ 1985, Self-association of the seventh component of human complement (C7): ...
Structural similarities between C6 and C7 of human complement<...
"Structural similarities between C6 and C7 of human complement",. abstract = "A new method for the isolation of C6 and C7 by ... Structural similarities between C6 and C7 of human complement. / Podack, E. R.; Kolb, W. P.; Esser, A. F.; Muller-Eberhard, H. ... Podack, ER, Kolb, WP, Esser, AF & Muller-Eberhard, HJ 1979, Structural similarities between C6 and C7 of human complement, ... Structural similarities between C6 and C7 of human complement. Journal of Immunology. 1979 Jan 1;123(3):1071-1077. ...
Two novel mutations in the C7 gene in a Korean patient with complement C7 deficiency.
In the Korean population, no case of C7 deficiency has been reported to date. Recently we experienced an 11-yr-... ... Complement C7 deficiency is an autosomal recessive disorder well known to be associated with increased susceptibility to ... Two novel mutations in the C7 gene in a Korean patient with complement C7 deficiency. Ki, Chang-Seok 관련메뉴. Kim, Jong-Won 관련메뉴. ... Complement C7 deficiency is an autosomal recessive disorder well known to be associated with increased susceptibility to ...
Complement membrane attack complex - Wikipedia
MAC is composed of a complex of four complement proteins (C5b, C6, C7, and C8) that bind to the outer surface of the plasma ... This junction alters the configuration of the protein molecules exposing a hydrophobic site on C7 that allows the C7 to insert ... Media related to Complement membrane attack complex at Wikimedia Commons. *Complement+Membrane+Attack+Complex at the US ... The membrane attack complex (MAC) or terminal complement complex (TCC) is a structure typically formed on the surface of ...
The complement C7 variant rs3792646 is associated with amyotrophic lateral sclerosis in a Han Chinese population - PubMed
We used whole exome sequencing to evaluate the role of C7 (rs3792646) in ALS in a Chi … ... Recently a C7 variant in rs3792646 was linked to neurodegenerative diseases in a Chinese population. ... The complement system has been shown to have a critical pathogenetic role in amyotrophic lateral sclerosis (ALS). ... The complement C7 variant rs3792646 is associated with amyotrophic lateral sclerosis in a Han Chinese population Ji He 1 , ...
Anti-C7 antibody (ab231047) | Abcam
Rabbit polyclonal C7 antibody. Validated in WB, IHC and tested in Rat. Immunogen corresponding to recombinant full length ... Defects in C7 are a cause of complement component 7 deficiency (C7D) [MIM:610102]. A rare defect of the complement classical ... Belongs to the complement C6/C7/C8/C9 family.. Contains 1 EGF-like domain.. Contains 1 LDL-receptor class A domain.. Contains 1 ... Anti-C7 antibody (ab231047) at 2 µg/ml + Recombinant rat C7 protein. Developed using the ECL technique. ...
Low-density lipoprotein (LDL) receptor class A, conserved site (IPR023415) | InterPro | EMBL-EBI
Complement components C6, C7, C8 and C9. They contain each one LDLRA domain. ... Complement factor I, which is responsible for cleaving the alpha-chains of C4b and C3b. It consists of a FIMAC domain (Factor I ... proteins C6/C7), a scavenger receptor-like domain, 2 copies of LDLRA and a C-terminal serine protease domain. ...
How to Dig Deeper? Improved Enrichment Methods for Mucin Core-1 Type Glycopeptides | Molecular & Cellular Proteomics
PROSITE: PDOC00021 (documentation file)
Innate Immune Responses Associated with Resistance against Haemonchus contortus in Morada Nova Sheep
... and complement system components (C7 and CFI). All target genes were quantified in the tissue samples, whereas, only the ... two complement factor genes were evaluated: CFI, related to the control of alternative route activation and C7, a membrane ... Superior activation of genes directly associated with complement activation (C7 and CFI) has been observed in sheep resistant ... Recent studies have also demonstrated superior expression of C7 and CFI in sheep with increased resistance to H. contortus ...
Aldo-keto Reductase 1C4/AKR1C4 Antibody (2C11) [DyLight 350] (H00001109-M01UV): Novus Biologicals
FAM48A Antibody (NBP1-90794): Novus Biologicals
C7 (Human) Recombinant Protein (P01) - (H00000730-P01) - Products - Abnova
Human C7 full-length ORF (BAG35608.1, 1 a.a. - 843 a.a.) recombinant protein with GST-tag at N-terminal. (H00000730-P01) - ... C7 is a component of the complement system. It participates in the formation of Membrane Attack Complex (MAC). People with C7 ... Human C7 full-length ORF (BAG35608.1, 1 a.a. - 843 a.a.) recombinant protein with GST-tag at N-terminal. ...
PRF1 - Perforin-1 precursor - Homo sapiens (Human) - PRF1 gene & protein
Table of Contents - July 15, 2000, 165 (2) | The Journal of Immunology
Isolation, Characterization, and Cloning of Porcine Complement Component C7 Azin Agah, Michael C. Montalto, Cheri L. Kiesecker ... Complement-Dependent Acute-Phase Expression of C-Reactive Protein and Serum Amyloid P-Component Alexander J. Szalai, Frederik W ... Distal Recognition Site for Classical Pathway Convertase Located in the C345C/Netrin Module of Complement Component C5 Ana ...
C6 - Complement component C6 precursor - Homo sapiens (Human) - C6 gene & protein
Belongs to the complement C6/C7/C8/C9 family.Curated. Keywords - Domaini. EGF-like domain, Repeat, Signal, Sushi. Phylogenomic ... "Structural homology of complement protein C6 with other channel-forming proteins of complement.". Chakravarti D.N., Chakravarti ... R-HSA-166665 Terminal pathway of complement. R-HSA-977606 Regulation of Complement cascade. ... R-HSA-166665 Terminal pathway of complement. R-HSA-977606 Regulation of Complement cascade. ...
Plus it
Two proteins marked by an asterisk (*) - biotinidase and complement component C7 - were later removed from the assay because of ... and AASGTQNNVLR from complement component C7 (P10643) was removed because it was not quantified in all of the replicates of 3 ... complement component C7 (P10643) using AASGTQNNVLR in S3, and vitamin K-dependent protein S (P07225) using SFQTGLFTAAR in S3. ... complement component C7 (P10643) using AASGTQNNVLR in S1, S3, S4; galectin-3-binding protein (Q08380) using ELSEALGQIFDSQR in ...
Frontiers | Clinical and Genetic Spectrum of a Large Cohort With Total and Sub-total Complement Deficiencies | Immunology
C7 (n = 17), C8 β (n = 7), C9 (n = 3), CFH (n = 7), CFI (n = 18), CFP (n = 10), CFD (n = 2)]. Molecular analysis identified 17 ... We identified 107 different hemizygous, homozygous, or compound heterozygous pathogenic variants in 14 complement genes [C1Qβ ( ... Thus, clinical indications for possible complete complement deficiencies include, among others, recurrent mild or serious ... When biochemical analysis reveals the causal abnormality of the complement deficiency (CD), molecular mechanisms remains ...
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 ...
Difficult-to-recruit respondents and their effect on prevalence estimates in an epidemiologic survey.
Complement-Related Disorders: Background, Pathophysiology, Activation
C5b activates the terminal complement pathway by associating with C6, C7, and C8 to form macromolecular complexes denoted as ... encoded search term (Complement-Related Disorders) and Complement-Related Disorders What to Read Next on Medscape. Related ... Table 3. Proteins of the Human Complement (C) System, Lectin Pathway *Table 4. Proteins of the Human Complement (C) System, C3 ... Table 1. Proteins of the Human Complement (C) System, Classical Pathway* *Table 2. Proteins of the Human Complement (C) System ...
Neisseria gonorrhoeae Meningitis in Pregnant Adolescent - Volume 14, Number 10-October 2008 - Emerging Infectious Diseases...
人末端补体复合物C5b-9(TCC C5b-9)ELISA试剂盒 中国教
ProteinsAntibodyProteinPathwayDeficienciesBindsCascadeMutationsPathways of the complement systemConvertaseCell lysisTerminal complement complexRegulatorNeisseriaGene encodesFragmentsRecurrent meningococcalAntibodiesELISAGlycoproteinActivationCleavageChromosomePatients with complementCoagulation cascadesImmuneComponentsHereditarySystemHuman Complement C5InfectionINHIBITORReceptorsCD59ComplexDefectsServes as a membrane anchorReceptor typeHydrophobicGenetic1998
Proteins49
- 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)
- C6 and C7 prepared by this method are hemolytically fully active, homogeneous proteins obtained in 25% yield. (elsevier.com)
- A comparison of the properties of isolated C6 and C7 gave the following results: The amino acid composition of the two proteins is very similar. (elsevier.com)
- 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)
- It consists of a FIMAC domain (Factor I/MAC proteins C6/C7), a scavenger receptor-like domain, 2 copies of LDLRA and a C-terminal serine protease domain. (ebi.ac.uk)
- The 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)
- with other complement proteins forms large holes in the microbe, allowing fluid to flow in and the microbe then bursts. (lifeworkpotential.com)
- The complement system as understood today is a multimolecular system composed of more than 32 proteins and consisting of serum proteins, serosal proteins, and cell membrane receptors that bind to complement fragments. (medscape.com)
- The complement system consists of 7 serum and 9 membrane regulatory proteins, 1 serosal regulatory protein, and 8 cell membrane receptors that bind complement fragments. (medscape.com)
- The combination and activation of this range of complement proteins forms the membrane attack complex , which is able to insert into bacterial cell walls and causes cell lysis with ensuing death. (wikipedia.org)
- The membrane attack complex (MAC) or terminal complement complex (TCC) is a complex of proteins typically formed on the surface of pathogen cell membranes as a result of the activation of the host's complement system, and as such is an effector of the immune system. (wikipedia.org)
- 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)
- Kirjavainen V, Jarva H, Biedzka Sarek M, Blom A, Skurnik M, Meri S. Yersinia enterocolitica serum resistance proteins YadA and ail bind the complement regulator C4b-binding protein. (labome.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 Classical pathway of activation of the complement system is a group of blood proteins that mediate the specific antibody response. (wikipathways.org)
- Most complement proteins are zymogens which are proteases that become activated when cleaved. (davidson.edu)
- C3b is a membrane associated protein which binds covalently to other complement proteins already on the pathogen surface. (davidson.edu)
- The complex of complement proteins on the cell surface after C3b binds forms the C5 convertase. (davidson.edu)
- After cleavage C5b interacts nonenzymatically with other complement proteins to form the MAC. (davidson.edu)
- The binding domain of C5b for other complement proteins such as C6 and C7 has been recently characterized and is homologous to a complement binding domain on C3 and C4. (davidson.edu)
- Other complement proteins such as C6 and C7 interact with C5 at the active site C345C in forming the MAC. (davidson.edu)
- Genetic abnormalities in the complement proteins or regulators of the cascade in the circulation or on cell surfaces appear to predipose individual patients to these disorders. (renalandurologynews.com)
- Genetic predisposition to MPGN is linked to mutations in factor H or other complement proteins such as C3 that lead to abnormal activation of the alternative pathway of complement. (renalandurologynews.com)
- Fragments resulting from proteolytic cleavage of complement proteins are designated with lower-case-letter suffixes, e.g. (thefreedictionary.com)
- Complement is the term used to describe a group of serum proteins that are critically important in our defense against infection. (primaryimmune.org)
- The complement system consists of more than 30 proteins, present in blood and tissues, as well as other proteins anchored on the surfaces of cells. (primaryimmune.org)
- Complement proteins in the circulation are not activated until triggered by an encounter with a bacterial cell, a virus, an immune complex, damaged tissue or other substance not usually present in the body. (primaryimmune.org)
- 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)
- To date, most, if not all, of the serum proteins that constitute the complement system have been identified and their activities characterized. (springer.com)
- 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)
- Vertebrate complement proteins C6 to C9. (embl.de)
- 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)
- Host cells are protected from complement activation by regulatory proteins that inactivate C3b deposited on their surface ( 12 , 13 ). (pnas.org)
- Both FH and FHL-1 (which is a truncated version of FH that results from an alternative splice site in the FH transcript [ 71 ]) are serum proteins that negatively regulate the alternative pathway of complement. (asm.org)
- 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)
- Meningococcal infections can be associated with abnormalities of the complement system, which contains 5 terminal complement proteins. (bvsalud.org)
- The complement system is composed of more than 25 different proteins and is usually divided into classical and alternative pathways. (bvsalud.org)
- Complement component 7(C7) is one of the five terminal complement proteins that, upon activation of either the classical or the alternative pathway, interacts sequentially to form a large protein-protein complex, called membrane attack complex(MAC). (bvsalud.org)
- Complement, a part of the innate immune system, is composed of more than 30 plasma- and cell membrane-bound proteins that function cooperatively in antimicrobial and inflammatory reactions. (psychiatryadvisor.com)
- Although deficiencies in complement components are rare, defects in the proteins that regulate complement are far more common. (psychiatryadvisor.com)
- Clusterin forms complexes with C5b:C6:C7, or C5b:C6:C7:C8 or C5b:C6:C7:C8:C9, as the proteins assemble into the amphiphilic MAC. (reactome.org)
Antibody22
- A Complement C7 specific antibody has been precoated onto 96-well plates and blocked. (abcam.com)
- Standards or test samples are added to the wells and subsequently a Complement C7 specific biotinylated detection antibody is added and then followed by washing with wash buffer. (abcam.com)
- C7 MaxPab polyclonal antibody. (acris-antibodies.com)
- Western Blot analysis of C7 expression in transfected 293T cell line ( H00000730-T01 ) by C7 MaxPab polyclonal antibody.Lane1:C7 transfected lysate(92.73 KDa).Lane2:Non-transfected lysate. (acris-antibodies.com)
- Formalin-fixed and paraffin-embedded human lymph tissue reacted with C7 Antibody (Center), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. (acris-antibodies.com)
- Western blot analysis of C7 Antibody (Center) (Cat. (acris-antibodies.com)
- C7 Antibody (Center) (Cat. (acris-antibodies.com)
- Complement C7 antibody LS-C691310 is an FITC-conjugated rabbit polyclonal antibody to rat Complement C7. (lsbio.com)
- The antibody is a rabbit polyclonal antibody raised against C7. (lsbio.com)
- A polyclonal antibody specific for C7 has been pre-coated onto a microplate. (innov-research.com)
- C7 in standards and samples is sandwiched by the immobilized antibody and a biotinylated polyclonal antibody specific for C7, which is recognized by a streptavidin peroxidase conjugate. (innov-research.com)
- 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 the serum contains antibody, i.e. is positive, an antibody-antigen complex is formed which also binds (fixes) complement. (thefreedictionary.com)
- 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)
- Ehrlich's term for the thermolabile substance, normally present in serum, that is destructive to certain bacteria and other cells sensitized by a specific complement-fixing antibody. (thefreedictionary.com)
- Complement 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)
- Constitutional symptoms ing that adequate levels of chronic stable angina and non-st are recommended for synergism, combination ther- mucosal epithelium of the anti-complement c7 antibody eculizumab clinical findings unless correction of the. (goodbelly.com)
- Therapy with tumor vaccines or complement-fixing monoclonal antibodies (mAb) inevitably results in sublytic levels of complement activation due to low antigen expression or periods of low antibody titers. (aacrjournals.org)
- We show here that while detectable levels of serum mAbs result in complement-mediated tumor cell lysis in vitro and tumor elimination in vivo , lower levels of antibody result in sublytic complement activation and more rapid tumor cell growth in vitro and in vivo . (aacrjournals.org)
- Immunofluorescence analysis of methanol-fixed HepG2, using Complement C9 antibody at 1/200 dilution. (acris-antibodies.com)
Protein31
- 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. (curehunter.com)
- Trypsin treatment released approximately 50% of the protein material from the C7 vesicle complex. (elsevier.com)
- C7 forms soluble, linear protein polymers that have sedimentation coefficients ranging from 20 to 30S. (elsevier.com)
- Recently we experienced an 11-yr-old girl with meningococcal meningitis who was diagnosed as having C7 deficiency based upon the undetectable serum C7 protein on radial immunodiffusion and the undetectable serum total and C7 hemolytic activities. (ndsl.kr)
- The membrane attack complex is initiated when the complement protein C5 convertase cleaves C5 into C5a and C5b. (wikipedia.org)
- Another complement protein, C6 , binds to C5b. (wikipedia.org)
- This junction alters the configuration of the protein molecules exposing a hydrophobic site on C7 that allows the C7 to insert into the phospholipid bilayer of the pathogen. (wikipedia.org)
- Recombinant full length protein (His-T7-tag) corresponding to Rat C7 aa 1-144. (abcam.com)
- WB: Recombinant rat C7 protein. (abcam.com)
- Human C7 full-length ORF (BAG35608.1, 1 a.a. - 843 a.a.) recombinant protein with GST-tag at N-terminal. (abnova.com)
- C6 (Complement C6) is a Protein Coding gene. (genecards.org)
- Able to break down fibrin clots, cleave complement protein C3, and activate Factor XII. (wikipedia.org)
- Using FACS to simultaneously evaluate viability, acrosomal status, and complement deposition, we found that complement activation was initiated by C-reactive protein (CRP) and was C1q, C2, and factor B dependent. (jci.org)
- Complement component 9 (C9) is a MACPF protein involved in the complement system, which is part of the innate immune system. (wikipedia.org)
- Backbone dynamics of complement control protein (CCP) modules reveals mobility in binding surfaces. (pdbj.org)
- Meri T, Blom A, Hartmann A, Lenk D, Meri S, Zipfel P. The hyphal and yeast forms of Candida albicans bind the complement regulator C4b-binding protein. (labome.org)
- There is wide variation of infections associated with complement deficiency depending on which complement protein and activation pathway is affected(Skattum et al 2011). (pediatriconcall.com)
- 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)
- The complement system consists of a series of plasma protein interactions which mediate direct attack on pathogens (opsonization) and induce inflammation. (davidson.edu)
- Patients with complement deficiencies encounter clinical problems that depend on the role of the specific complement protein in normal function. (primaryimmune.org)
- CD59 is a membrane-bound protein that is present in many types of cells and binds to homologous complement factors C8 and C9 of the terminal complement system. (davidson.edu)
- The CD59 protein is a complement system regulator that can inhibit the formation of the membrane attack complex (MAC). (davidson.edu)
- The protein encoded by this gene is the fifth component of complement, which plays an important role in inflammatory and cell killing processes. (creativebiomart.net)
- S206P protein was expressed on the cell surface but had a reduced ability to prevent complement activation, consistent with its reduced C3b binding and cofactor activity. (pnas.org)
- Mutations have been reported in the complement regulatory protein factor H in both sporadic ( n = 9) and familial ( n = 8 families) HUS ( 3 - 7 ) with mutations identified in 10-20% of cases studied. (pnas.org)
- 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)
- 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)
- Recently, we have experienced a 20-year-old military trainee with meningococcal sepsis and meningitis who was diagnosed as having C7 deficiency based upon the undetectable serum C7 protein on radioimmunoassay. (bvsalud.org)
- Infections are prominent in many complete complement deficiencies, and the kinds of infections are determined by the function of the deficient protein in host defense. (psychiatryadvisor.com)
- A wide range of diseases are therefore associated with abnormalities or deficiencies in complement and depend on the specific protein that is abnormal. (psychiatryadvisor.com)
- C3 is the central protein of all three complement pathways and plays a critical role in the opsonization of pathogens. (psychiatryadvisor.com)
Pathway23
- A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis. (abcam.com)
- Among its related pathways are Complement and coagulation cascades and Immune response Lectin induced complement pathway . (genecards.org)
- Depending on the nature of complement activators, the classic pathway, the alternative pathway, or the more recently discovered lectin pathway is activated predominantly to produce C3 convertase. (medscape.com)
- Binding of factor H to C3b increases its inactivation by factor I. Properdin stabilizes it, preventing its inactivation by factors H and I. The alternate pathway does not result in a truly nonspecific activation of complement because it requires specific types of compounds for activation. (medscape.com)
- Three biochemical pathways activate the complement system: the classical complement pathway , the alternative complement pathway , and the lectin pathway . (wikipedia.org)
- the sequence of reactions, each being the catalyst for the next, that leads to the terminal complement pathway and cell lysis. (thefreedictionary.com)
- The alternative pathway of the complement system is an innate component of the immune system 's natural defense against infections. (wikipedia.org)
- The alternative pathway is one of three complement pathways that opsonize and kill pathogens. (wikipedia.org)
- After the creation of C5 convertase (either as (C3b) 2 BbP or C4b2a3b from the classical pathway), the complement system follows the same path regardless of the means of activation (alternative, classical, or lectin). (wikipedia.org)
- The primary cases are generally the consequence of perturbations in the regulation of the alternative pathway of complement with constitutive activation of this cascade. (renalandurologynews.com)
- MPGN is one of a group of rare disorders that includes atypical hemolylic uremic syndrome (HUS) and macular degeneration that are characterized by abnormal activation of the alternative pathway of complement. (renalandurologynews.com)
- The Alternative Pathway (AP) is initiated by fragments of the complement component C3. (primaryimmune.org)
- This time-limitation is another control mechanism for the complement pathway. (primaryimmune.org)
- The Terminal Pathway (TP) is the final set of steps in the complement activation process that forms a membrane lesion or hole (membrane attack complex or MAC) that kills susceptible bacteria or other cells that activate complement on their surfaces. (primaryimmune.org)
- In the alternate pathway complement C3 undergoes spontaneous cleavage resulting in complement B binding to C3b. (sigmaaldrich.com)
- ACH-4471 4 targets complement factor D in the alternative pathway ( Online Supplementary Figure S1 ), and is being developed to treat paroxysmal nocturnal hemoglobinuria and C3 glomerulopathy. (haematologica.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)
- Pathway analysis implicated the complement and coagulation cascade. (deepdyve.com)
- Complement-mediated accelerated growth of cultured tumor cell lines initiated by low mAb levels was associated with activation of the phosphoinositide 3-kinase (PI3K)/AKT survival pathway and significantly elevated levels of both p-AKT and p-PRAS40. (aacrjournals.org)
- This sublytic complement activation was associated with activation of the phosphoinositide 3-kinase (PI3K)/AKT survival pathway. (aacrjournals.org)
- The complement system is composed of three pathways: the classical pathway, the alternative pathway, and the lectin pathway (Figure 1). (psychiatryadvisor.com)
- This gene encodes a serum glycoprotein that forms a membrane attack complex together with complement components C5b, C6, C8, and C9 as part of the terminal complement pathway of the innate immune system. (antibodypedia.com)
- Properdin is a serum glycoprotein that up-regulates the alternative pathway of complement by stabilizing the C3b-Bb complex. (embl.de)
Deficiencies18
- 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)
- 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)
- 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)
- Deficiency of C5, C6, C7 and C8 has less than 1000 reported cases of Neisserial meningitis and sepsis, while C9 deficiencies is more common in Japanese populations with prevalence around 1:1000 ( Fukumori et al 1989)(Nagata et al 1989). (pediatriconcall.com)
- Complement 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)
- Furthermore, deficiencies in 1 of these 5, complement component 7 (C7), leads to the loss of complement lytic function, and affected patients show increased susceptibility to recurrent meningococcal meningitis and systemic Neisseria gonorrhoeae infection. (bvsalud.org)
- Most of the genetically determined deficiencies of the complement system are inherited as autosomal recessive traits, with the exception of C1 esterase inhibitor, which is inherited as an autosomal dominant trait, and properdin deficiency, which is inherited as an X-linked recessive trait. (psychiatryadvisor.com)
- A complete deficiency of a complement component is rare, and partial deficiencies are rarely of any clinical significance. (psychiatryadvisor.com)
- Middle Complement Deficiencies - CRASH! (4doctors.net)
Binds3
- Freshly activated C5b binds to C6 to form a C5b-6 complex, then to C7 forming the C5b-6-7 complex. (wikipedia.org)
- C5b binds sequentially to C6, C7, C8 and then to multiple molecules of C9 to form membrane attack complex . (wikipedia.org)
- [3] 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)
Cascade9
- 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)
- Upon exposure of human spermatozoa to autologous serum or follicular fluid, we unexpectedly observed that acrosome-reacted spermatozoa activated the complement cascade efficiently through C3 but not beyond. (jci.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)
- The complement system is a biochemical cascade that helps, or complements, the ability of antibodies to clear pathogens from an organism. (wikipathways.org)
- Terminal complement components comprise the final 5 components of the complement cascade (C5 to C9). (scielo.org.za)
- The complement system is a complex cascade involving proteolytic cleavage of serum glycoproteins often activated by cell receptors. (sigmaaldrich.com)
- Ischaemic infarction may also cause initiation of the complement cascade. (sigmaaldrich.com)
- 1991). It is able to modulate the terminal complement cascade in vitro and prevent cellular lysis by the membrane attack complex (MAC), C5b-9. (reactome.org)
Mutations11
- Mutations cause complement C7 deficiency which may result in immunological abnormalities, predisposition to meningococcal infections in aprticular. (moldiag.com)
- Two novel mutations in the C7 gene in a Korean patient with complement C7 deficiency. (ndsl.kr)
- Mutations in this gene are associated with complement component-6 deficiency. (genecards.org)
- Degn SE, Jensenius JC, Thiel S. Disease-causing mutations in genes of the complement system. (medscape.com)
- 2010) Complement factor 7 gene mutations in relation to meningococcal infection and clinical recurrence of meningococcal disease. (sinobiological.com)
- Mutations in this gene cause complement component 5 deficiency, a disease where patients show a propensity for severe recurrent infections. (creativebiomart.net)
- The majority of mutations are missense changes in the exon encoding complement control module 20 of factor H, an area important for both binding to anionic molecules and C3b ( 8 ). (pnas.org)
- Such mutations result in impaired protection of host surfaces against complement activation ( 9 - 11 ). (pnas.org)
- We first described factor H mutations ( 4 ) after a linkage study in three families that mapped HUS to a 26-centiMorgan region of chromosome 1 (1q32) containing a cluster of complement related genes, including factor H. However, we identified a factor H mutation in only one of the three families used in this linkage study. (pnas.org)
- Her elder brother also had a similar bacterial meningoencephalitis history, which encouraged us to perform analyses for complement component and gene mutations. (bvsalud.org)
- Mutations in this gene are associated with a rare disorder called C7 deficiency. (antibodypedia.com)
Pathways of the complement system2
- All three pathways of the complement system ( classical , lectin and alternative pathways) initiate the formation of MAC. (wikipedia.org)
- Each of the three pathways of the complement system results in the formation of C3 convertase on the pathogen surface. (davidson.edu)
Convertase4
- In the first phase, a series of specific interactions leads to formation of intrinsic complement proteinase, termed C3 convertase. (medscape.com)
- The formation of a C3 convertase can also be prevented when a plasma protease called complement factor I cleaves C3b into its inactive form, iC3b. (wikipedia.org)
- 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)
- The three distinct activation pathways of complement converge with the formation of a C5 convertase. (acris-antibodies.com)
Cell lysis1
- C5b with C6, C7, C8, and C9 form the membrane attack complex that initiates cell lysis. (thefreedictionary.com)
Terminal complement complex3
- A fluid phase form of the MAC, called the Terminal Complement Complex (TCC) can be found in the circulation after complement activation occurs and makes a useful laboratory marker for complement activation. (primaryimmune.org)
- 2009) C7 is expressed on endothelial cells as a trap for the assembling terminal complement complex and may exert anti-inflammatory function. (sinobiological.com)
- After binding of C8, a variable number of C9 molecules associate with the C5b678 complex, which is also termed the terminal complement complex (TCC). (acris-antibodies.com)
Regulator3
- Factor H, a regulator of complement activity, is a major determinant of meningococcal disease susceptibility in UK Caucasian patients. (nih.gov)
- A unique feature of the AP is the presence of the only positive regulator in the complement system, Properdin. (primaryimmune.org)
- CD46) is a widely expressed transmembrane complement regulator. (pnas.org)
Neisseria4
- C7 deficiency exhibits an increased susceptibility to Neisseria meningitidis infections and recurrent meningococcal disease (2 - 3). (innov-research.com)
- Complement deficiency predisposes for meningitis due to nongroupable meningococci and Neisseria-related bacteria. (nih.gov)
- We have been interested in developing a complement dependent opsonophagocytic assay for Neisseria meningitidis serogroups A and C. Our problem is that when we add a complement source (baby rabbit) we get (as expected) bactericidal killing. (bio.net)
- C7 deficiency is associated frequently with recurrently bacterial infections, especially meningitis caused by Neisseria meningitidis. (bvsalud.org)
Gene encodes1
- The C7 gene encodes a complement component involved in the membrane attack complex. (moldiag.com)
Fragments2
- We propose that this localized deposition of complement fragments aids in the fusion process between the spermatozoa and egg, in a role akin to that of complement in immune adherence. (jci.org)
- Complement activation also results in the formation of many biologically active complement fragments that act as anaphylatoxins, opsonins, or chemotactic factors. (thefreedictionary.com)
Recurrent meningococcal1
- 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)
Antibodies7
- 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 complement system can be recruited and brought into action by antibodies generated by the adaptive immune system . (wikipedia.org)
- In the early 20th century, this controversy was resolved when it became understood that complement can act in combination with specific antibodies, or on its own in a non-specific way. (wikipedia.org)
- Monitoring patients with the C1q assay, which detects antibodies that fix complement, offers a minimally invasive means of identifying patients at risk for transplant glomerulopathy and graft loss. (labome.org)
- 05). The results of our study suggest that anti-beta2GPI antibodies may play a role in GMT formation, and this process might involve complement activation. (labome.org)
- 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)
- These findings were mirrored by in vitro studies with antibodies against these antigens as well as GD2 and CD20 (with Rituxan), and shown to be complement-dependent in all cases. (aacrjournals.org)
ELISA2
- Abcam's Complement C7 Human in vitro ELISA (Enzyme-Linked Immunosorbent Assay) kit is designed for the quantitative measurement of C7 in Human plasma, serum, saliva, milk, urine and cell culture supernatants. (abcam.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)
Glycoprotein2
- A 93-kDa serum glycoprotein encoded by C7 gene. (curehunter.com)
- 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)
Activation16
- On the other hand, resistant lambs had an enhanced response mediated by TLR recognition and complement activation. (hindawi.com)
- Activation of the complement pathways. (medscape.com)
- In addition, we speculate that this "targeted and restricted" form of complement activation on host cells is a common strategy to handle modified self. (jci.org)
- It inhibits complement activation at the yeast surface and, in addition, mediates adhesion of C. albicans to host endothelial cells. (labome.org)
- Opportunities for new therapies based on the natural regulators of complement activation. (wikipathways.org)
- Complement activation is involved in the detection and elimination of pathogens as well as cell turnover and tissue growth and regeneration. (separationsnow.com)
- Complement activation. (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)
- 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)
- Functional classification analysis of DE genes revealed dynamic changes in ion binding (such as DSG1 ) between AH and CS, proliferation and apoptotic processes (such as BAG3 , HLA-DQA1 , and UGT2B17 ) and complement activation (such as C7 ) between AH or RS and CS. (biomedcentral.com)
- Like factor H it inhibits complement activation by regulating C3b deposition on targets. (pnas.org)
- This complement-mediated PI3K activation and accelerated tumor growth in vitro and in vivo are eliminated by PI3K inhibitors NVP-BEZ235 and Wortmannin. (aacrjournals.org)
- Complement has many functions, including promoting phagocytosis of pathogens by acting as an opsonin, inducing lysis of bacteria or susceptible cells and generatinginflammation by products formed during complement activation. (psychiatryadvisor.com)
- Complement is often associated with unregulated inflammation, and many mechanisms exist for downregulation of complement activation. (psychiatryadvisor.com)
- TCC is present in normal human plasma and increased in patients with complement activation. (acris-antibodies.com)
Cleavage2
- The cleavage product C5B joins C6, C7, C8 and multiple copies of C9 to form the Membrane Attack Complex, which forms a channel for water to flood into the target cell, leading to osmotic lysis. (wikipathways.org)
- The C5b macromolecular cleavage product can form a complex with the C6 complement component, and this complex is the basis for formation of the membrane attack complex, which includes additional complement components. (creativebiomart.net)
Chromosome2
- The gene encoding MCP is in the cluster of complement-related genes on chromosome 1q32. (pnas.org)
- The gene for C7 has been assigned to chromosome 5p13. (bvsalud.org)
Patients with complement1
- Cephalosporins are often used for treatment of N meningitidis infection in patients with complement deficiency. (medscape.com)
Coagulation cascades1
- We selected most pathways C5 participated on our site, such as Complement and coagulation cascades, Prion diseases, Pertussis, which may be useful for your reference. (creativebiomart.net)
Immune13
- 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)
- 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)
- Functional complement C1q abnormality leads to impaired immune complexes and apoptotic cell clearance. (medscape.com)
- The primary functions of the complement system are to protect from infection, to remove particulate substances, (like damaged or dying cells, microbes or immune complexes) and to help modulate adaptive immune responses. (primaryimmune.org)
- As part of the innate immune system, complement acts immediately to start the process of removal and resolution of the problem. (primaryimmune.org)
- Complement works with the inflammatory cells of the innate immune system and those of adaptive or acquired immunity. (primaryimmune.org)
- 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)
- There was an increase in the relative hepatic mRNA levels of c7 (complement component 7), a gene involved in the innate immune system, at 22 mu g/L and at all higher concentrations, again following a clear dose-response. (gu.se)
- Complement inhibitors are being studied as potential therapeutics for immune diseases and Alzheimer's. (sigmaaldrich.com)
- Borrelia burgdorferi has developed efficient mechanisms for evading the innate immune response during mammalian infection and has been shown to be resistant to the complement-mediated bactericidal activity of human serum. (asm.org)
- This case emphasizes that although C7 deficiency is rare immune disorder, it is important to be aware of possibility about late complement deficiency among patients who present with meningococcal disease. (bvsalud.org)
Components15
- Complement components C6, C7, C8 and C9. (ebi.ac.uk)
- Complement C1r/C1s components (1 copy). (yale.edu)
- The MAC is composed of the complement components C5b, C6, C7, C8 and several C9 molecules. (wikipedia.org)
- C9 is one member of the complement membrane attack complex (MAC), which also includes complement components C5b, C6, C7 and C8. (wikipedia.org)
- In fish, the liver is the site where the majority of complement components are produced and expressed, but C9 can also be found in other tissues. (wikipedia.org)
- MAC formation starts with the assembly of a tetrameric complex with the complement components C6, C7, C8, and C5b. (wikipedia.org)
- We have tried to remove one of the terminal complement components but without much success (can remove component(s) but the removal process reduces the lytic complement activity when we reconstitute with the purified complement component). (bio.net)
- 2. is there a method to remove terminal components in human sera that does not reduce complement activity when the component is added back? (bio.net)
- various complement components may be deficient without serious effects on the host. (thefreedictionary.com)
- C5b begins the assembly of complement components by binding C6 and C7 to its C5-C345C domain, creating a C5b-7 complex which remains loosely associated with C3b (Dalmasso, 1998). (davidson.edu)
- These complement components form the final membrane attack complex (MAC). (thefreedictionary.com)
- It was originally identified and named as being common to five complement components (C6, C7, C8-alpha, C8-beta, and C9) and perforin. (embl.de)
- 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)
- 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)
- 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)
Hereditary4
- 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)
- Here, we report a Korean family with a hereditary C7 deficiency with susceptibility to meningococcal infections due to C7 gene mutation. (bvsalud.org)
- We report a case of a hereditary C7 deficiency associated with meningococcal meningitis. (bvsalud.org)
- Hereditary deficiency of the sixth component of complement in man. (moldiag.com)
System14
- The complement system has been shown to have a critical pathogenetic role in amyotrophic lateral sclerosis (ALS). (cdc.gov)
- Our study further elucidated the relationship between the complement system and ALS from a genetic perspective. (cdc.gov)
- Reid K. B. M., The complement system , in: B. D. Hames and D. M. Glover (eds. (wikipedia.org)
- 2001). "The complement system and innate immunity" . (wikipedia.org)
- C7 is a component of the complement system. (abnova.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)
- The complement system is known to be activated by the immunoglobulins IgM and IgG. (thefreedictionary.com)
- In the first, also referred to as the test system, antigen is mixed usually with serial dilutions of a test serum in the presence of complement. (thefreedictionary.com)
- Evaluation of his complement system showed an absence of functional and antigenic C7, compatible with a complete deficiency of the seventh component of complement. (annals.org)
- Dysregulation of the complement system has been implicated in several diseases and pathologies. (wikipedia.org)
- It activates C3 of the complement system and probably supports inflammation in other ways as well. (biology-pages.info)
- In several models of autoimmune diseases, the overactivation of the complement system (CS) is the cause of disease exacerbation. (scielo.br)
- Our findings indicate that the complement and coagulation system is dysregulated in the blood during childhood before the development of the PD. (deepdyve.com)
- C1q associates with the proenzymes C1r and C1s to yield C1, the first component of the serum complement system. (creativebiomart.net)
Human Complement C51
- Inherited human complement C5 deficiency. (moldiag.com)
Infection3
- People with C7 deficiency are prone to bacterial infection. (abnova.com)
- Complement C4 deficiency--a plausible risk factor for non-tuberculous mycobacteria (NTM) infection in apparently immunocompetent patients. (medscape.com)
- Chronic neisserial infection can be associated with C7 deficiency and must be distinguished from other causes of cutaneous vasculitis. (annals.org)
INHIBITOR1
- Role of a disulfide-bonded peptide loop within human complement C9 in the species-selectivity of complement inhibitor CD59. (embl.de)
Receptors1
- In this chapter, the structure and function of the complement receptors on the macrophage and related cells are described. (springer.com)
CD591
- CD59 on hematologic cells is required to prevent unregulated complement lysis. (psychiatryadvisor.com)
Complex10
- The membrane attack complex of complement: relation of C7 to the metastable membrane binding site of the intermediate complex C5b-7. (jimmunol.org)
- Direct evidence showing that the metastable membrane binding site of the C5b-7 complex resides in the C7 subunit was obtained by using the conjugates of C5b,6 and colloidal gold. (jimmunol.org)
- The C5bC6 complex is bound by C7 . (wikipedia.org)
- This binding leads to a conformational change of the C7 molecule which exposes a hydrophobic binding site and allows the complex to insert itself into the lipid bilayer of the pathogen. (davidson.edu)
- Such substances include eosinophil chemotactic factor of anaphylaxis, leukotriene B4, complement complex (C5-C6-C7), and histamine (over a narrow range of concentration). (merckmanuals.com)
- A complex of complements C5b,C6, C7, and C8 mediates the polymerization of up to eighteen C9 molecules into a tube-like membrane attack complex that is inserted into the plasma membrane of an unwanted organism such as of gram-negative bacteria and viral infected cells. (sigmaaldrich.com)
- IgE mediated eosinophil production is induced by compounds released by basophils and mast cells , including eosinophil chemotactic factor of anaphylaxis , leukotriene B4 and serotonin mediated release of eosinophil granules occur, complement complex (C5-C6-C7), interleukin 5 , and histamine (though this has a narrow range of concentration). (wikipedia.org)
- Our results suggest that both nutrients (VFAs) and particle sizes can alter expression of genes involved in cell proliferation/apoptosis process and complement complex. (biomedcentral.com)
- In addition, all three complement pathways result in the formation of the membrane attack complex, which is vital to bactericidal activity. (psychiatryadvisor.com)
- Binding of C6 facilitates binding of C7 which alters the conformation of the complex. (acris-antibodies.com)
Defects4
- Defects in C7 are a cause of complement component 7 deficiency (C7D) [MIM:610102]. (abcam.com)
- Defects in C7 are a cause of complement component 7 deficiency (C7D). (sinobiological.com)
- This study presents further evidence that complement dysregulation predisposes to development of thrombotic microangiopathy and that screening patients for such defects could provide informed treatment strategies. (pnas.org)
- To date, 15 different molecular defects leading to total or subtotal C7 deficient defects have been reported. (bvsalud.org)
Serves as a membrane anchor2
- C7 serves as a membrane anchor. (abcam.com)
- Complement component 7 serves as a membrane anchor. (sinobiological.com)
Receptor type1
- Human complement receptor type 1/CD35 is an Epstein-Barr Virus receptor. (nih.gov)
Hydrophobic2
- The incubation of isolated C7 in 1% deoxycholate results in hemolytically inactive dimeric C7 that has a sedimentation coefficient of 7.3S. Dimeric C7 expressed hydrophobic domains that bound 41 ± 4 mol deoxycholate per mol C7 and that aggregated upon removal of the detergent. (elsevier.com)
- The hydrophobic anchor of C7 in the membrane has a high affinity for phospholipids which ensures strong binding while other complement constituents of the MAC are brought together. (davidson.edu)
Genetic1
- 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)
19981
- The C5b fragment first exposes a binding site having high affinity for C6 and C7 with an associated half life of 2.3 minutes (Dalmasso, 1998). (davidson.edu)