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.
A glycoprotein that is important in the activation of CLASSICAL COMPLEMENT PATHWAY. C4 is cleaved by the activated COMPLEMENT C1S into COMPLEMENT C4A and COMPLEMENT C4B.
The smaller fragment formed when complement C4 is cleaved by COMPLEMENT C1S. It is an anaphylatoxin that causes symptoms of immediate hypersensitivity (HYPERSENSITIVITY, IMMEDIATE) but its activity is weaker than that of COMPLEMENT C3A or COMPLEMENT C5A.
The smaller fragment generated from the cleavage of complement C3 by C3 CONVERTASE. C3a, a 77-amino acid peptide, is a mediator of local inflammatory process. It induces smooth MUSCLE CONTRACTION, and HISTAMINE RELEASE from MAST CELLS and LEUKOCYTES. C3a is considered an anaphylatoxin along with COMPLEMENT C4A; COMPLEMENT C5A; and COMPLEMENT C5A, DES-ARGININE.
A subcomponent of complement C1, composed of six copies of three polypeptide chains (A, B, and C), each encoded by a separate gene (C1QA; C1QB; C1QC). This complex is arranged in nine subunits (six disulfide-linked dimers of A and B, and three disulfide-linked homodimers of C). C1q has binding sites for antibodies (the heavy chain of IMMUNOGLOBULIN G or IMMUNOGLOBULIN M). The interaction of C1q and immunoglobulin activates the two proenzymes COMPLEMENT C1R and COMPLEMENT C1S, thus initiating the cascade of COMPLEMENT ACTIVATION via the CLASSICAL COMPLEMENT PATHWAY.
The minor fragment formed when C5 convertase cleaves C5 into C5a and COMPLEMENT C5B. C5a is a 74-amino-acid glycopeptide with a carboxy-terminal ARGININE that is crucial for its spasmogenic activity. Of all the complement-derived anaphylatoxins, C5a is the most potent in mediating immediate hypersensitivity (HYPERSENSITIVITY, IMMEDIATE), smooth MUSCLE CONTRACTION; HISTAMINE RELEASE; and migration of LEUKOCYTES to site of INFLAMMATION.
The sequential activation of serum COMPLEMENT PROTEINS to create the COMPLEMENT MEMBRANE ATTACK COMPLEX. Factors initiating complement activation include ANTIGEN-ANTIBODY COMPLEXES, microbial ANTIGENS, or cell surface POLYSACCHARIDES.
The large fragment formed when COMPLEMENT C4 is cleaved by COMPLEMENT C1S. The membrane-bound C4b binds COMPLEMENT C2A, a SERINE PROTEASE, to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).
C5 plays a central role in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C5 is cleaved by C5 CONVERTASE into COMPLEMENT C5A and COMPLEMENT C5B. The smaller fragment C5a is an ANAPHYLATOXIN and mediator of inflammatory process. The major fragment C5b binds to the membrane initiating the spontaneous assembly of the late complement components, C5-C9, into the MEMBRANE ATTACK COMPLEX.
The larger fragment generated from the cleavage of COMPLEMENT C3 by C3 CONVERTASE. It is a constituent of the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb), and COMPLEMENT C5 CONVERTASES in both the classical (C4b2a3b) and the alternative (C3bBb3b) pathway. C3b participates in IMMUNE ADHERENCE REACTION and enhances PHAGOCYTOSIS. It can be inactivated (iC3b) or cleaved by various proteases to yield fragments such as COMPLEMENT C3C; COMPLEMENT C3D; C3e; C3f; and C3g.
Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY).
A 105-kDa serum glycoprotein with significant homology to the other late complement components, C7-C9. It is a polypeptide chain cross-linked by 32 disulfide bonds. C6 is the next complement component to bind to the membrane-bound COMPLEMENT C5B in the assembly of MEMBRANE ATTACK COMPLEX. It is encoded by gene C6.
A 206-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 (749-954), and C3dg (955-1303) in the presence COMPLEMENT FACTOR H.
A 302-amino-acid fragment in the alpha chain (672-1663) of C3b. It is generated when C3b is inactivated (iC3b) and its alpha chain is cleaved by COMPLEMENT FACTOR I into C3c, and C3dg (955-1303) in the presence COMPLEMENT FACTOR H. Serum proteases further degrade C3dg into C3d (1002-1303) and C3g (955-1001).
A component of the CLASSICAL COMPLEMENT PATHWAY. C2 is cleaved by activated COMPLEMENT C1S into COMPLEMENT C2B and COMPLEMENT C2A. C2a, the COOH-terminal fragment containing a SERINE PROTEASE, combines with COMPLEMENT C4B to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).
A 63-kDa serum glycoprotein encoded by gene C9. Monomeric C9 (mC9) binds the C5b-8 complex to form C5b-9 which catalyzes the polymerization of C9 forming C5b-p9 (MEMBRANE ATTACK COMPLEX) and transmembrane channels leading to lysis of the target cell. Patients with C9 deficiency suffer from recurrent bacterial infections.
Molecules on the surface of some B-lymphocytes and macrophages, that recognize and combine with the C3b, C3d, C1q, and C4b components of complement.
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).
A product of COMPLEMENT ACTIVATION cascade, regardless of the pathways, that forms transmembrane channels causing disruption of the target CELL MEMBRANE and cell lysis. It is formed by the sequential assembly of terminal complement components (COMPLEMENT C5B; COMPLEMENT C6; COMPLEMENT C7; COMPLEMENT C8; and COMPLEMENT C9) into the target membrane. The resultant C5b-8-poly-C9 is the "membrane attack complex" or MAC.
A 80-kDa subcomponent of complement C1, existing as a SERINE PROTEASE proenzyme in the intact complement C1 complex. When COMPLEMENT C1Q is bound to antibodies, the changed tertiary structure causes autolytic activation of complement C1r which is cleaved into two chains, A (heavy) and B (light, the serine protease), connected by disulfide bonds. The activated C1r serine protease, in turn, activates COMPLEMENT C1S proenzyme by cleaving the Arg426-Ile427 bond. No fragment is released when either C1r or C1s is cleaved.
Serum proteins that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host. The complement system is tightly regulated by inactivators that accelerate the decay of intermediates and certain cell surface receptors.
A 93-kDa serum glycoprotein encoded by C7 gene. It is a polypeptide chain with 28 disulfide bridges. In the formation of MEMBRANE ATTACK COMPLEX; C7 is the next component to bind the C5b-6 complex forming a trimolecular complex C5b-7 which is lipophilic, resembles an integral membrane protein, and serves as an anchor for the late complement components, C8 and C9.
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.
A glycine-rich, heat-labile serum glycoprotein that contains a component of the C3 CONVERTASE ALTERNATE PATHWAY (C3bBb). Bb, a serine protease, is generated when factor B is cleaved by COMPLEMENT FACTOR D into Ba and Bb.
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 activation initiated by the binding of COMPLEMENT C1 to ANTIGEN-ANTIBODY COMPLEXES at the COMPLEMENT C1Q subunit. This leads to the sequential activation of COMPLEMENT C1R and COMPLEMENT C1S subunits. Activated C1s cleaves COMPLEMENT C4 and COMPLEMENT C2 forming the membrane-bound classical C3 CONVERTASE (C4B2A) and the subsequent C5 CONVERTASE (C4B2A3B) leading to cleavage of COMPLEMENT C5 and the assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.
A 150-kDa serum glycoprotein composed of three subunits with each encoded by a different gene (C8A; C8B; and C8G). This heterotrimer contains a disulfide-linked C8alpha-C8gamma heterodimer and a noncovalently associated C8beta chain. C8 is the next component to bind the C5-7 complex forming C5b-8 that binds COMPLEMENT C9 and acts as a catalyst in the polymerization of C9.
The first complement component to act in the activation of CLASSICAL COMPLEMENT PATHWAY. It is a calcium-dependent trimolecular complex made up of three subcomponents: COMPLEMENT C1Q; COMPLEMENT C1R; and COMPLEMENT C1S at 1:2:2 ratios. When the intact C1 binds to at least two antibodies (involving C1q), C1r and C1s are sequentially activated, leading to subsequent steps in the cascade of COMPLEMENT ACTIVATION.
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.
An important soluble regulator of the alternative pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It is a 139-kDa glycoprotein expressed by the liver and secreted into the blood. It binds to COMPLEMENT C3B and makes iC3b (inactivated complement 3b) susceptible to cleavage by COMPLEMENT FACTOR I. Complement factor H also inhibits the association of C3b with COMPLEMENT FACTOR B to form the C3bB proenzyme, and promotes the dissociation of Bb from the C3bBb complex (COMPLEMENT C3 CONVERTASE, ALTERNATIVE PATHWAY).
The larger fragment generated from the cleavage of C5 by C5 CONVERTASE that yields COMPLEMENT C5A and C5b (beta chain + alpha' chain, the residual alpha chain, bound by disulfide bond). C5b remains bound to the membrane and initiates the spontaneous assembly of the late complement components to form C5b-8-poly-C9, the MEMBRANE ATTACK COMPLEX.
The COOH-terminal fragment of COMPLEMENT 2, released by the action of activated COMPLEMENT C1S. It is a SERINE PROTEASE. C2a combines with COMPLEMENT C4B to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).
A G-protein-coupled receptor that signals an increase in intracellular calcium in response to the potent ANAPHYLATOXIN peptide COMPLEMENT C5A.
Enzymes that activate one or more COMPLEMENT PROTEINS in the complement system leading to the formation of the COMPLEMENT MEMBRANE ATTACK COMPLEX, an important response in host defense. They are enzymes in the various COMPLEMENT ACTIVATION pathways.
Compounds that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host.
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.
Serum proteins that inhibit, antagonize, or inactivate COMPLEMENT C1 or its subunits.
Molecular sites on or in B-lymphocytes, follicular dendritic cells, lymphoid cells, and epithelial cells that recognize and combine with COMPLEMENT C3D. Human complement receptor 2 (CR2) serves as a receptor for both C3dg and the gp350/220 glycoprotein of HERPESVIRUS 4, HUMAN, and binds the monoclonal antibody OKB7, which blocks binding of both ligands to the receptor.
Serum peptides derived from certain cleaved COMPLEMENT PROTEINS during COMPLEMENT ACTIVATION. They induce smooth MUSCLE CONTRACTION; mast cell HISTAMINE RELEASE; PLATELET AGGREGATION; and act as mediators of the local inflammatory process. The order of anaphylatoxin activity from the strongest to the weakest is C5a, C3a, C4a, and C5a des-arginine.
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.
A serum protein which is important in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY. This enzyme cleaves the COMPLEMENT C3B-bound COMPLEMENT FACTOR B to form C3bBb which is ALTERNATIVE PATHWAY C3 CONVERTASE.
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.
A serum protein that regulates the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. It binds as a cofactor to COMPLEMENT FACTOR I which then hydrolyzes the COMPLEMENT C4B in the CLASSICAL PATHWAY C3 CONVERTASE (C4bC2a).
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.
GPI-linked membrane proteins broadly distributed among hematopoietic and non-hematopoietic cells. CD55 prevents the assembly of C3 CONVERTASE or accelerates the disassembly of preformed convertase, thus blocking the formation of the membrane attack complex.
Important enzymes in the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. They cleave COMPLEMENT C3 and COMPLEMENT C5.
The N-terminal fragment of COMPLEMENT 2, released by the action of activated COMPLEMENT C1S.
Small glycoproteins found on both hematopoietic and non-hematopoietic cells. CD59 restricts the cytolytic activity of homologous complement by binding to C8 and C9 and blocking the assembly of the membrane attack complex. (From Barclay et al., The Leukocyte Antigen FactsBook, 1993, p234)
Venoms from snakes of the genus Naja (family Elapidae). They contain many specific proteins that have cytotoxic, hemolytic, neurotoxic, and other properties. Like other elapid venoms, they are rich in enzymes. They include cobramines and cobralysins.
The complex formed by the binding of antigen and antibody molecules. The deposition of large antigen-antibody complexes leading to tissue damage causes IMMUNE COMPLEX DISEASES.
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).
Important enzymes in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY. They cleave COMPLEMENT C3 and COMPLEMENT C5.
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.
The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B.
The destruction of ERYTHROCYTES by many different causal agents such as antibodies, bacteria, chemicals, temperature, and changes in tonicity.
A serine protease that is the complex of COMPLEMENT C3B and COMPLEMENT FACTOR BB. It cleaves multiple COMPLEMENT C3 into COMPLEMENT C3A (anaphylatoxin) and COMPLEMENT C3B in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY.
A serine protease that cleaves multiple COMPLEMENT 5 into COMPLEMENT 5A (anaphylatoxin) and COMPLEMENT 5B in the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. It is a complex of CLASSICAL PATHWAY C3 CONVERTASE (C4b2a) with an additional COMPLEMENT C3B, or C4b2a3b.
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.
A serine protease that cleaves multiple COMPLEMENT 3 into COMPLEMENT 3A (anaphylatoxin) and COMPLEMENT 3B in the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. It is a complex of COMPLEMENT 4B and COMPLEMENT 2A (C4b2a).
A ubiquitously expressed complement receptor that binds COMPLEMENT C3B and COMPLEMENT C4B and serves as a cofactor for their inactivation. CD46 also interacts with a wide variety of pathogens and mediates immune response.
Proteins that bind to particles and cells to increase susceptibility to PHAGOCYTOSIS, especially ANTIBODIES bound to EPITOPES that attach to FC RECEPTORS. COMPLEMENT C3B may also participate.
Proteins that are present in blood serum, including SERUM ALBUMIN; BLOOD COAGULATION FACTORS; and many other types of proteins.
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.
A serine protease that cleaves multiple COMPLEMENT C5 into COMPLEMENT C5A (anaphylatoxin) and COMPLEMENT C5B in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY. It is the complex of ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb) with an additional COMPLEMENT C3B, or C3bBb3b.
The engulfing and degradation of microorganisms; other cells that are dead, dying, or pathogenic; and foreign particles by phagocytic cells (PHAGOCYTES).
The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.
Complement activation triggered by the interaction of microbial POLYSACCHARIDES with serum MANNOSE-BINDING LECTIN resulting in the activation of MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES. As in the classical pathway, MASPs cleave COMPLEMENT C4 and COMPLEMENT C2 to form C3 CONVERTASE (C4B2A) and the subsequent C5 CONVERTASE (C4B2A3B) leading to cleavage of COMPLEMENT C5 and assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.
A 53-kDa protein that is a positive regulator of the alternate pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It stabilizes the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb) and protects it from rapid inactivation, thus facilitating the cascade of COMPLEMENT ACTIVATION and the formation of MEMBRANE ATTACK COMPLEX. Individuals with mutation in the PFC gene exhibit properdin deficiency and have a high susceptibility to infections.
A derivative of complement C5a, generated when the carboxy-terminal ARGININE is removed by CARBOXYPEPTIDASE B present in normal human serum. C5a des-Arg shows complete loss of spasmogenic activity though it retains some chemotactic ability (CHEMOATTRACTANTS).
An adhesion-promoting leukocyte surface membrane heterodimer. The alpha subunit consists of the CD11b ANTIGEN and the beta subunit the CD18 ANTIGEN. The antigen, which is an integrin, functions both as a receptor for complement 3 and in cell-cell and cell-substrate adhesive interactions.
The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments.
Granular leukocytes having a nucleus with three to five lobes connected by slender threads of chromatin, and cytoplasm containing fine inconspicuous granules and stainable by neutral dyes.
The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.
A cluster of convoluted capillaries beginning at each nephric tubule in the kidney and held together by connective tissue.
The clear portion of BLOOD that is left after BLOOD COAGULATION to remove BLOOD CELLS and clotting proteins.
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.
A class of immunoglobulin bearing mu chains (IMMUNOGLOBULIN MU-CHAINS). IgM can fix COMPLEMENT. The name comes from its high molecular weight and originally being called a macroglobulin.
A genus of trematode flukes belonging to the family Schistosomatidae. There are over a dozen species. These parasites are found in man and other mammals. Snails are the intermediate hosts.
A test used to determine whether or not complementation (compensation in the form of dominance) will occur in a cell with a given mutant phenotype when another mutant genome, encoding the same mutant phenotype, is introduced into that cell.
An immunoassay utilizing an antibody labeled with an enzyme marker such as horseradish peroxidase. While either the enzyme or the antibody is bound to an immunosorbent substrate, they both retain their biologic activity; the change in enzyme activity as a result of the enzyme-antibody-antigen reaction is proportional to the concentration of the antigen and can be measured spectrophotometrically or with the naked eye. Many variations of the method have been developed.
Strains of mice in which certain GENES of their GENOMES have been disrupted, or "knocked-out". To produce knockouts, using RECOMBINANT DNA technology, the normal DNA sequence of the gene being studied is altered to prevent synthesis of a normal gene product. Cloned cells in which this DNA alteration is successful are then injected into mouse EMBRYOS to produce chimeric mice. The chimeric mice are then bred to yield a strain in which all the cells of the mouse contain the disrupted gene. Knockout mice are used as EXPERIMENTAL ANIMAL MODELS for diseases (DISEASE MODELS, ANIMAL) and to clarify the functions of the genes.
Inflammation of the renal glomeruli (KIDNEY GLOMERULUS) that can be classified by the type of glomerular injuries including antibody deposition, complement activation, cellular proliferation, and glomerulosclerosis. These structural and functional abnormalities usually lead to HEMATURIA; PROTEINURIA; HYPERTENSION; and RENAL INSUFFICIENCY.
Thickening of the walls of small ARTERIES or ARTERIOLES due to cell proliferation or HYALINE deposition.
Antibodies produced by a single clone of cells.
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.
Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN.
Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.
Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.
RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.
The relatively long-lived phagocytic cell of mammalian tissues that are derived from blood MONOCYTES. Main types are PERITONEAL MACROPHAGES; ALVEOLAR MACROPHAGES; HISTIOCYTES; KUPFFER CELLS of the liver; and OSTEOCLASTS. They may further differentiate within chronic inflammatory lesions to EPITHELIOID CELLS or may fuse to form FOREIGN BODY GIANT CELLS or LANGHANS GIANT CELLS. (from The Dictionary of Cell Biology, Lackie and Dow, 3rd ed.)
Established cell cultures that have the potential to propagate indefinitely.
The capacity of a normal organism to remain unaffected by microorganisms and their toxins. It results from the presence of naturally occurring ANTI-INFECTIVE AGENTS, constitutional factors such as BODY TEMPERATURE and immediate acting immune cells such as NATURAL KILLER CELLS.
Partial proteins formed by partial hydrolysis of complete proteins or generated through PROTEIN ENGINEERING techniques.
Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.
The species Oryctolagus cuniculus, in the family Leporidae, order LAGOMORPHA. Rabbits are born in burrows, furless, and with eyes and ears closed. In contrast with HARES, rabbits have 22 chromosome pairs.
Naturally occurring or experimentally induced animal diseases with pathological processes sufficiently similar to those of human diseases. They are used as study models for human diseases.
The insertion of recombinant DNA molecules from prokaryotic and/or eukaryotic sources into a replicating vehicle, such as a plasmid or virus vector, and the introduction of the resultant hybrid molecules into recipient cells without altering the viability of those cells.
The parts of a macromolecule that directly participate in its specific combination with another molecule.
The natural bactericidal property of BLOOD due to normally occurring antibacterial substances such as beta lysin, leukin, etc. This activity needs to be distinguished from the bactericidal activity contained in a patient's serum as a result of antimicrobial therapy, which is measured by a SERUM BACTERICIDAL TEST.
Differentiation antigens residing on mammalian leukocytes. CD stands for cluster of differentiation, which refers to groups of monoclonal antibodies that show similar reactivity with certain subpopulations of antigens of a particular lineage or differentiation stage. The subpopulations of antigens are also known by the same CD designation.
Electrophoresis in which a polyacrylamide gel is used as the diffusion medium.
A specific mannose-binding member of the collectin family of lectins. It binds to carbohydrate groups on invading pathogens and plays a key role in the MANNOSE-BINDING LECTIN COMPLEMENT PATHWAY.
Variant forms of the same gene, occupying the same locus on homologous CHROMOSOMES, and governing the variants in production of the same gene product.
Immunoglobulin molecules having a specific amino acid sequence by virtue of which they interact only with the ANTIGEN (or a very similar shape) that induced their synthesis in cells of the lymphoid series (especially PLASMA CELLS).
Proteins prepared by recombinant DNA technology.
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.
Conjugated protein-carbohydrate compounds including mucins, mucoid, and amyloid glycoproteins.
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.
Plasma glycoproteins that form a stable complex with hemoglobin to aid the recycling of heme iron. They are encoded in man by a gene on the short arm of chromosome 16.
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).
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 whose amino and carboxy ends are linked together with a peptide bond forming a circular chain. Some of them are ANTI-INFECTIVE AGENTS. Some of them are biosynthesized non-ribosomally (PEPTIDE BIOSYNTHESIS, NON-RIBOSOMAL).
Glomerulonephritis associated with autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Lupus nephritis is histologically classified into 6 classes: class I - normal glomeruli, class II - pure mesangial alterations, class III - focal segmental glomerulonephritis, class IV - diffuse glomerulonephritis, class V - diffuse membranous glomerulonephritis, and class VI - advanced sclerosing glomerulonephritis (The World Health Organization classification 1982).
Autoantibodies directed against various nuclear antigens including DNA, RNA, histones, acidic nuclear proteins, or complexes of these molecular elements. Antinuclear antibodies are found in systemic autoimmune diseases including systemic lupus erythematosus, Sjogren's syndrome, scleroderma, polymyositis, and mixed connective tissue disease.
The degree of similarity between sequences of amino acids. This information is useful for the analyzing genetic relatedness of proteins and species.
Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.
Plasmids containing at least one cos (cohesive-end site) of PHAGE LAMBDA. They are used as cloning vehicles.
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.
Proteins found in any species of bacterium.
Any of the processes by which nuclear, cytoplasmic, or intercellular factors influence the differential control (induction or repression) of gene action at the level of transcription or translation.
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.
A pathological process characterized by injury or destruction of tissues caused by a variety of cytologic and chemical reactions. It is usually manifested by typical signs of pain, heat, redness, swelling, and loss of function.
Transport proteins that carry specific substances in the blood or across cell membranes.
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.
A group of inherited disorders of the ADRENAL GLANDS, caused by enzyme defects in the synthesis of cortisol (HYDROCORTISONE) and/or ALDOSTERONE leading to accumulation of precursors for ANDROGENS. Depending on the hormone imbalance, congenital adrenal hyperplasia can be classified as salt-wasting, hypertensive, virilizing, or feminizing. Defects in STEROID 21-HYDROXYLASE; STEROID 11-BETA-HYDROXYLASE; STEROID 17-ALPHA-HYDROXYLASE; 3-beta-hydroxysteroid dehydrogenase (3-HYDROXYSTEROID DEHYDROGENASES); TESTOSTERONE 5-ALPHA-REDUCTASE; or steroidogenic acute regulatory protein; among others, underlie these disorders.
The restriction of a characteristic behavior, anatomical structure or physical system, such as immune response; metabolic response, or gene or gene variant to the members of one species. It refers to that property which differentiates one species from another but it is also used for phylogenetic levels higher or lower than the species.
An individual in which both alleles at a given locus are identical.
Body organ that filters blood for the secretion of URINE and that regulates ion concentrations.
The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment.
Biologically active substances whose activities affect or play a role in the functioning of the immune system.
Elements of limited time intervals, contributing to particular results or situations.
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.
Histochemical localization of immunoreactive substances using labeled antibodies as reagents.
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.
The genetic constitution of individuals with respect to one member of a pair of allelic genes, or sets of genes that are closely linked and tend to be inherited together such as those of the MAJOR HISTOCOMPATIBILITY COMPLEX.
Proteins which are found in membranes including cellular and intracellular membranes. They consist of two types, peripheral and integral proteins. They include most membrane-associated enzymes, antigenic proteins, transport proteins, and drug, hormone, and lectin receptors.
Antigens determined by leukocyte loci found on chromosome 6, the major histocompatibility loci in humans. They are polypeptides or glycoproteins found on most nucleated cells and platelets, determine tissue types for transplantation, and are associated with certain diseases.
Glycoproteins found on the membrane or surface of cells.
The sequential correspondence of nucleotides in one nucleic acid molecule with those of another nucleic acid molecule. Sequence homology is an indication of the genetic relatedness of different organisms and gene function.
The phenotypic manifestation of a gene or genes by the processes of GENETIC TRANSCRIPTION and GENETIC TRANSLATION.
Large, phagocytic mononuclear leukocytes produced in the vertebrate BONE MARROW and released into the BLOOD; contain a large, oval or somewhat indented nucleus surrounded by voluminous cytoplasm and numerous organelles.
The sum of the weight of all the atoms in a molecule.
The rate dynamics in chemical or physical systems.
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.
The parts of a transcript of a split GENE remaining after the INTRONS are removed. They are spliced together to become a MESSENGER RNA or other functional RNA.
Lymphoid cells concerned with humoral immunity. They are short-lived cells resembling bursa-derived lymphocytes of birds in their production of immunoglobulin upon appropriate stimulation.
The presence of proteins in the urine, an indicator of KIDNEY DISEASES.
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.
The production of ANTIBODIES by proliferating and differentiated B-LYMPHOCYTES under stimulation by ANTIGENS.
Any member of the group of ENDOPEPTIDASES containing at the active site a serine residue involved in catalysis.
A gram-positive organism found in the upper respiratory tract, inflammatory exudates, and various body fluids of normal and/or diseased humans and, rarely, domestic animals.
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.
Use of restriction endonucleases to analyze and generate a physical map of genomes, genes, or other segments of DNA.
A category of nucleic acid sequences that function as units of heredity and which code for the basic instructions for the development, reproduction, and maintenance of organisms.
Short sequences (generally about 10 base pairs) of DNA that are complementary to sequences of messenger RNA and allow reverse transcriptases to start copying the adjacent sequences of mRNA. Primers are used extensively in genetic and molecular biology techniques.
A plasma protein that circulates in increased amounts during inflammation and after tissue damage.
The genetic constitution of the individual, comprising the ALLELES present at each GENETIC LOCUS.
A positive regulatory effect on physiological processes at the molecular, cellular, or systemic level. At the molecular level, the major regulatory sites include membrane receptors, genes (GENE EXPRESSION REGULATION), mRNAs (RNA, MESSENGER), and proteins.
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)
Cytochrome P-450 monooxygenases (MIXED FUNCTION OXYGENASES) that are important in steroid biosynthesis and metabolism.
Detection of RNA that has been electrophoretically separated and immobilized by blotting on nitrocellulose or other type of paper or nylon membrane followed by hybridization with labeled NUCLEIC ACID PROBES.
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.
Single-stranded complementary DNA synthesized from an RNA template by the action of RNA-dependent DNA polymerase. cDNA (i.e., complementary DNA, not circular DNA, not C-DNA) is used in a variety of molecular cloning experiments as well as serving as a specific hybridization probe.
A method (first developed by E.M. Southern) for detection of DNA that has been electrophoretically separated and immobilized by blotting on nitrocellulose or other type of paper or nylon membrane followed by hybridization with labeled NUCLEIC ACID PROBES.
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.
Degenerative changes in the RETINA usually of older adults which results in a loss of vision in the center of the visual field (the MACULA LUTEA) because of damage to the retina. It occurs in dry and wet forms.
A constitution or condition of the body which makes the tissues react in special ways to certain extrinsic stimuli and thus tends to make the individual more than usually susceptible to certain diseases.
Models used experimentally or theoretically to study molecular shape, electronic properties, or interactions; includes analogous molecules, computer-generated graphics, and mechanical structures.
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.
A variation of the PCR technique in which cDNA is made from RNA via reverse transcription. The resultant cDNA is then amplified using standard PCR protocols.
The lipid- and protein-containing, selectively permeable membrane that surrounds the cytoplasm in prokaryotic and eukaryotic cells.
The record of descent or ancestry, particularly of a particular condition or trait, indicating individual family members, their relationships, and their status with respect to the trait or condition.
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.
Variation occurring within a species in the presence or length of DNA fragment generated by a specific endonuclease at a specific site in the genome. Such variations are generated by mutations that create or abolish recognition sites for these enzymes or change the length of the fragment.
The proportion of one particular in the total of all ALLELES for one genetic locus in a breeding POPULATION.
A common name used for the genus Cavia. The most common species is Cavia porcellus which is the domesticated guinea pig used for pets and biomedical research.
A method for the detection of very small quantities of antibody in which the antigen-antibody-complement complex adheres to indicator cells, usually primate erythrocytes or nonprimate blood platelets. The reaction is dependent on the number of bound C3 molecules on the C3b receptor sites of the indicator cell.
A species of gram-negative, facultatively anaerobic, rod-shaped bacteria (GRAM-NEGATIVE FACULTATIVELY ANAEROBIC RODS) commonly found in the lower part of the intestine of warm-blooded animals. It is usually nonpathogenic, but some strains are known to produce DIARRHEA and pyogenic infections. Pathogenic strains (virotypes) are classified by their specific pathogenic mechanisms such as toxins (ENTEROTOXIGENIC ESCHERICHIA COLI), etc.
A technique that combines protein electrophoresis and double immunodiffusion. In this procedure proteins are first separated by gel electrophoresis (usually agarose), then made visible by immunodiffusion of specific antibodies. A distinct elliptical precipitin arc results for each protein detectable by the antisera.
Potentially pathogenic bacteria found in nasal membranes, skin, hair follicles, and perineum of warm-blooded animals. They may cause a wide range of infections and intoxications.
The uptake of naked or purified DNA by CELLS, usually meaning the process as it occurs in eukaryotic cells. It is analogous to bacterial transformation (TRANSFORMATION, BACTERIAL) and both are routinely employed in GENE TRANSFER TECHNIQUES.
A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.
Either of the pair of organs occupying the cavity of the thorax that effect the aeration of the blood.
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.
Immunoglobulins produced in a response to BACTERIAL ANTIGENS.
The systematic study of the complete complement of proteins (PROTEOME) of organisms.
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.
A cytokine that stimulates the growth and differentiation of B-LYMPHOCYTES and is also a growth factor for HYBRIDOMAS and plasmacytomas. It is produced by many different cells including T-LYMPHOCYTES; MONOCYTES; and FIBROBLASTS.
The characteristic 3-dimensional shape of a protein, including the secondary, supersecondary (motifs), tertiary (domains) and quaternary structure of the peptide chain. PROTEIN STRUCTURE, QUATERNARY describes the conformation assumed by multimeric proteins (aggregates of more than one polypeptide chain).
Cells that line the inner and outer surfaces of the body by forming cellular layers (EPITHELIUM) or masses. Epithelial cells lining the SKIN; the MOUTH; the NOSE; and the ANAL CANAL derive from ectoderm; those lining the RESPIRATORY SYSTEM and the DIGESTIVE SYSTEM derive from endoderm; others (CARDIOVASCULAR SYSTEM and LYMPHATIC SYSTEM) derive from mesoderm. Epithelial cells can be classified mainly by cell shape and function into squamous, glandular and transitional epithelial cells.
The relationship between the chemical structure of a compound and its biological or pharmacological activity. Compounds are often classed together because they have structural characteristics in common including shape, size, stereochemical arrangement, and distribution of functional groups.
A large collection of DNA fragments cloned (CLONING, MOLECULAR) from a given organism, tissue, organ, or cell type. It may contain complete genomic sequences (GENOMIC LIBRARY) or complementary DNA sequences, the latter being formed from messenger RNA and lacking intron sequences.
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.
A latent susceptibility to disease at the genetic level, which may be activated under certain conditions.
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.
A single nucleotide variation in a genetic sequence that occurs at appreciable frequency in the population.
Group of diseases mediated by the deposition of large soluble complexes of antigen and antibody with resultant damage to tissue. Besides SERUM SICKNESS and the ARTHUS REACTION, evidence supports a pathogenic role for immune complexes in many other IMMUNE SYSTEM DISEASES including GLOMERULONEPHRITIS, systemic lupus erythematosus (LUPUS ERYTHEMATOSUS, SYSTEMIC) and POLYARTERITIS NODOSA.
Complement factor I can further cleave iC3b into a protein fragment known as C3d. Robbins Basic Pathology 8th ed 2007. R Cotran ... iC3b is produced when complement factor I cleaves C3b. Complement receptors on white blood cells are able to bind iC3b, so iC3b ... Unlike intact C3b, iC3b cannot associate with factor B, thus preventing amplification of the complement cascade through the ... iC3b is a protein fragment that is part of the complement system, a component of the vertebrate immune system. ...
2000). "Functional properties of complement factor H-related proteins FHR-3 and FHR-4: binding to the C3d region of C3b and ... Diaz-Guillen MA, Rodriguez de Cordoba S, Heine-Suner D (Jul 1999). "A radiation hybrid map of complement factor H and factor H- ... CFHR4 complement factor H-related 4". Hageman GS, Hancox LS, Taiber AJ, et al. (2007). "Extended Haplotypes in the Complement ... Complement factor H-related protein 4 is a protein that in humans is encoded by the CFHR4 gene. GRCh38: Ensembl release 89: ...
2000). "Functional properties of complement factor H-related proteins FHR-3 and FHR-4: binding to the C3d region of C3b and ... "Entrez Gene: CFHR3 complement factor H-related 3". Zipfel PF, Skerka C (1994). "Complement factor H and related proteins: an ... Diaz-Guillen MA, Rodriguez de Cordoba S, Heine-Suner D (Jul 1999). "A radiation hybrid map of complement factor H and factor H- ... Complement factor H-related protein 3 is a protein that in humans is encoded by the CFHR3 gene. GRCh38: Ensembl release 89: ...
February 2011). "Dual interaction of factor H with C3d and glycosaminoglycans in host-nonhost discrimination by complement". ... such as complement factor H-related genes, as well as in other complement proteins (e.g. factor I, C2/factor B, and C3) have ... Factor H has been shown to interact with complement component 3, amongst other complement proteins and factors, leading to ... in the complement factor H gene (CFH) often result in pathologies. Moreover, the complement inhibitory activities of factor H, ...
Factor I can cleave C3b into C3c and C3d, the latter of which plays a role in enhancing B cell responses. In the alternative ... Activated C3 can then interact with factor B. Factor B is then activated by factor D, to form Bb. The resultant complex, C3bBb ... "Interactions between human complement components factor H, factor I and C3b". The Biochemical Journal. 326 (2): 553-61. doi: ... Next, C3b is broken down progressively to first iC3b, then C3c + C3dg, and then finally C3d. Factor I is the protease cleaves ...
... complement receptor 2 is the C3d receptor. Factor H, another immunoregulatory protein, also maps to this location. The ... Fearon DT (July 1985). "Human complement receptors for C3b (CR1) and C3d (CR2)". The Journal of Investigative Dermatology. 85 ( ... 1989). "Immunoregulatory functions of complement: structural and functional studies of complement receptor type 1 (CR1; CD35) ... decay-accelerating factor (DAF: CD55: Cromer antigen) protects host cells from complement-mediated damage by regulating the ...
Decay Accelerating Factor, DAF (CD55) Protectin (CD59) Complement C3b/C4b Receptor 1, CR1 (CD35) Complement Regulator of the ... CR2 CCP modules complexed with C3d (pdb:1ghq)). Complement has been implicated in many diseases associated with inflammation ... Complement control protein are proteins that interact with components of the complement system. The complement system is ... Complement proteins protect against malignant cells- both by direct complement attack and through initiation of Complement- ...
Complement decay-accelerating factor (Antigen CD55) belongs to the Cromer blood group system and is associated with Cr(a), Dr(a ... CD21 (also called C3d receptor, CR2, Epstein Barr virus receptor or EBV-R) is the receptor for EBV and for C3d, C3dg and iC3b. ... 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 ...
CD21, complement receptor 2, can bind fragments of C3 that have covalently attached to glycoconjugates by complement activation ... Paired box transcription factor 5 (PAX5) plays a major role in B cell differentiation from pro B cell to mature B cell, the ... The CD19/CD21 complex arises from C3d binding to CD21; however, CD19 does not require CD21 for signal transduction. CD81, ... While colligated with the BCR, the CD19/CD21 complex bound to the antigen-complement complex can decrease the threshold for B ...
... complement factor h MeSH D12.776.124.486.274.920.325.210 - complement factor i MeSH D12.776.124.486.274.920.662 - complement ... 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. ... complement factor d MeSH D12.776.124.486.274.900 - complement factor b MeSH D12.776.124.486.274.920 - complement inactivator ... complement c1 inhibitor protein MeSH D12.776.124.486.274.920.287 - complement c3 nephritic factor MeSH D12.776.124.486.274.920. ...
These ligands include B cell receptor (for antigen), IgG Fc receptors, CD21, which binds complement C3d, Toll-like receptors 9 ... Cigarette smoking is now established as a major risk factor for both incidence and severity of rheumatoid arthritis. This may ... It remains entirely possible that the disease is due to e.g. an unusual interaction between immune complexes, complement ... Taking the experience of a beneficial factor in autoimmunity further, one might hypothesize with intent to prove that ...
Cases may also arise with complement alone or with IgA, IgM or a combination of these three antibody classes and complement. ... In cold agglutinin disease, the monospecific DAT is by definition positive for the compliment molecule C3d but IgM may be ... In 1904, Donath and Landsteiner suggested a serum factor was responsible for hemolysis in paroxysmal cold hemoglobinuria. ... Antibodies are produced against the RBCs, which leads to complement activation. Complement fragments, such as C3a, C4a and C5a ...
The complement system plays an important part in defense against pyogenic organisms. ... The complement system is part of the innate immune system. ... C3d of complement as a molecular adjuvant: bridging innate and ... Complement factor I deficiency in a family with recurrent infections. Immunopharmacology. 1997 Dec. 38(1-2):207-13. [QxMD ... Molecular basis of complement factor I deficiency in Tunisian atypical hemolytic and uremic syndrome Patients. Nephrology ( ...
The complement system plays an important part in defense against pyogenic organisms. ... The complement system is part of the innate immune system. ... C3d of complement as a molecular adjuvant: bridging innate and ... Complement factor I deficiency in a family with recurrent infections. Immunopharmacology. 1997 Dec. 38(1-2):207-13. [QxMD ... Molecular basis of complement factor I deficiency in Tunisian atypical hemolytic and uremic syndrome Patients. Nephrology ( ...
Of the 8 plasma membrane receptors for complement, only deficiencies of CR3 and CR4 due to CD18 deficiency have been described ... The complement system exerts many of its effects through complement receptors (CRs). ... CD11a/CD18 is also known as leukocyte factor antigen-1 (LFA-1), CD11b/CD18 is known as CR3, and CD11c/CD18 is known as CR4. ... CR2 binds C3dg, and C3d is present on B cells and dendritic cells (see the Table 1). CR2 associates with CD19 forming a CR2- ...
TACI is one of 3 tumor necrosis factor (TNF)-receptor family members and mediates isotype switching in B cells. TACI mutations ... Reduced class-switched memory B cells; hypogammaglobulinemia; reduced binding of C3d-containing immune complexes and EBV-gp350 ... Common variable immunodeficiency and the complement system; low mannose-binding lectin levels are associated with ... Successful treatment of granulomas with tumor necrosis factor-alpha antagonists has been reported in a few patients with common ...
The complement system plays an important part in defense against pyogenic organisms. ... The complement system is part of the innate immune system. ... C3d of complement as a molecular adjuvant: bridging innate and ... Complement factor I deficiency in a family with recurrent infections. Immunopharmacology. 1997 Dec. 38(1-2):207-13. [QxMD ... Molecular basis of complement factor I deficiency in Tunisian atypical hemolytic and uremic syndrome Patients. Nephrology ( ...
Reduced class-switched memory B cells; hypogammaglobulinemia; reduced binding of C3d-containing immune complexes and EBV-gp350 ... Common variable immunodeficiency and the complement system; low mannose-binding lectin levels are associated with ... the normal T cells in the culture may secrete factors that induce blastogenesis in the patient's lymphocytes. Therefore, using ... and tumor necrosis factor (TNF), in the culture supernatant. ...
Complement component C3d (substance) {71368001 , SNOMED-CT } Complement component C3d,g (substance) {103199005 , SNOMED-CT } ... Complement factor Ba (substance) {70434003 , SNOMED-CT } Complement factor Bb (substance) {82592003 , SNOMED-CT } ... Complement component fragment (substance) {78036008 , SNOMED-CT } Parent/Child (Relationship Type) Complement component C1q ( ... Complement component C1s (substance) {27273002 , SNOMED-CT } Complement component C2a (substance) {7161007 , SNOMED-CT } ...
The complement system plays an important part in defense against pyogenic organisms. ... The complement system is part of the innate immune system. ... C3d of complement as a molecular adjuvant: bridging innate and ... complement receptor 2 (CR2), and decay accelerating factor (DAF). A North African study of molecular basis of complement factor ... Cases of complement deficiency have helped defined the role of complement in host defense. [4] A registry of complement ...
... rejection is characterized by a combination of donor-specific antibodies in the blood and evidence of complement fixation (C3d ... tumor necrosis factor-α, and γ interferon via binding to glucocorticoid response elements and blocking cytokine activation.[63] ... which is needed to activate nuclear factor of activated T cells. This leads to inhibition of T-cell proliferation, T-cell ... although it binds to FK506-binding protein 12 to inhibit nuclear factor of activated T cells at a greater potency than ...
Risk factors for this adverse reaction include preexisting renal insufficiency, diabetes mellitus, dehydration, age older than ... Reduced class-switched memory B cells; hypogammaglobulinemia; reduced binding of C3d-containing immune complexes and EBV-gp350 ... Common variable immunodeficiency and the complement system; low mannose-binding lectin levels are associated with ... These events are related to hyperosmolality or activated coagulation factor, or high sodium content, or presence of anti-D ...
B-cell activating factor belonging to the tumor necrosis factor family) that functions to regulate B-cell proliferation and ... The role of complement in experimental bullous pemphigoid. J Clin Invest. 1995 Apr. 95(4):1539-44. [QxMD MEDLINE Link]. [Full ... Pfaltz K, Mertz K, Rose C, Scheidegger P, Pfaltz M, Kempf W. C3d immunohistochemistry on formalin-fixed tissue is a valuable ... Activation of the complement system is thought to play a critical role in attracting inflammatory cells to the basement ...
The complement system plays an important part in defense against pyogenic organisms. ... The complement system is part of the innate immune system. ... C3d of complement as a molecular adjuvant: bridging innate and ... Complement factor I deficiency in a family with recurrent infections. Immunopharmacology. 1997 Dec. 38(1-2):207-13. [QxMD ... Molecular basis of complement factor I deficiency in Tunisian atypical hemolytic and uremic syndrome Patients. Nephrology ( ...
B-cell activating factor belonging to the tumor necrosis factor family) that functions to regulate B-cell proliferation and ... The role of complement in experimental bullous pemphigoid. J Clin Invest. 1995 Apr. 95(4):1539-44. [QxMD MEDLINE Link]. [Full ... Pfaltz K, Mertz K, Rose C, Scheidegger P, Pfaltz M, Kempf W. C3d immunohistochemistry on formalin-fixed tissue is a valuable ... Activation of the complement system is thought to play a critical role in attracting inflammatory cells to the basement ...
The complement system plays an important part in defense against pyogenic organisms. ... The complement system is part of the innate immune system. ... C3d of complement as a molecular adjuvant: bridging innate and ... complement receptor 2 (CR2), and decay accelerating factor (DAF). A North African study of molecular basis of complement factor ... Cases of complement deficiency have helped defined the role of complement in host defense. [4] A registry of complement ...
The role of complement in experimental bullous pemphigoid. J Clin Invest. 1995 Apr. 95(4):1539-44. [QxMD MEDLINE Link]. [Full ... Liu YD, Wang YH, Ye YC, Zhao WL, Li L. Prognostic factors for mortality in patients with bullous pemphigoid: a meta-analysis. ... Pfaltz K, Mertz K, Rose C, Scheidegger P, Pfaltz M, Kempf W. C3d immunohistochemistry on formalin-fixed tissue is a valuable ... Risk factors for relapse in patients with bullous pemphigoid in clinical remission: a multicenter, prospective, cohort study. ...
The complement system plays an important part in defense against pyogenic organisms. ... The complement system is part of the innate immune system. ... C3d of complement as a molecular adjuvant: bridging innate and ... complement receptor 2 (CR2), and decay accelerating factor (DAF). A North African study of molecular basis of complement factor ... Cases of complement deficiency have helped defined the role of complement in host defense. [4] A registry of complement ...
It is a humanized monoclonal antibody directed against complement factor C1s and inhibits the classical complement pathway, ... antiglobulin reagents used to avoid misleading results due to the cold agglutinin in the serum and the RBC-bound C3d. ... Small trials report benefit from treatment with the complement inhibitor eculizumab. The complement inhibitor sutimlimab, which ... because they lack proteolytically cleaved complement on their surface. This may inhibit complement-mediated lysis. [63] ...
It is a humanized monoclonal antibody directed against complement factor C1s and inhibits the classical complement pathway, ... antiglobulin reagents used to avoid misleading results due to the cold agglutinin in the serum and the RBC-bound C3d. ... Small trials report benefit from treatment with the complement inhibitor eculizumab. The complement inhibitor sutimlimab, which ... because they lack proteolytically cleaved complement on their surface. This may inhibit complement-mediated lysis. [63] ...
... complement receptor 2 is the C3d receptor. Factor H, another immunoregulatory protein, also maps to this location. The ... Fearon DT (July 1985). "Human complement receptors for C3b (CR1) and C3d (CR2)". The Journal of Investigative Dermatology. 85 ( ... 1989). "Immunoregulatory functions of complement: structural and functional studies of complement receptor type 1 (CR1; CD35) ... decay-accelerating factor (DAF: CD55: Cromer antigen) protects host cells from complement-mediated damage by regulating the ...
Mouse Transforming growth factor-beta-induced protein ig-h3 (TGFBI) ELISA kit from Cusabio. Cat#: CSB-EL023450MO. US, UK & ... Human Complement Fragment 3d, C3d ELISA Kit , CSB-E14213h , CusabioHuman Complement Fragment 3d, C3d ELISA Kit is Available at ... Human Complement C1q tumor necrosis factor-related protein 1 (C1QTNF1/CTRP1) ELISA Kit , CSB-E16713h , CusabioHuman Complement ... Human Complement C1q tumor necrosis factor-related protein 6 (C1QTNF6) ELISA kit , CSB-EL003651HU , CusabioHuman Complement C1q ...
Description: C3d, Complement factor H. Source: Homo sapiens. Experimental method: X-RAY DIFFRACTION ... Complement Factor H and Simian Virus 40 bind the GM1 ganglioside in distinct conformations. ... PDB ID 4zh1: Complement factor H in complex with the GM1 glycan ... Transforming growth factor-β challenge alters the N-, O-, and ...
The complement system plays an important part in defense against pyogenic organisms. ... The complement system is part of the innate immune system. ... C3d of complement as a molecular adjuvant: bridging innate and ... Complement factor I deficiency in a family with recurrent infections. Immunopharmacology. 1997 Dec. 38(1-2):207-13. [QxMD ... Molecular basis of complement factor I deficiency in Tunisian atypical hemolytic and uremic syndrome Patients. Nephrology ( ...
The complement system plays an important part in defense against pyogenic organisms. ... The complement system is part of the innate immune system. ... C3d of complement as a molecular adjuvant: bridging innate and ... Complement factor I deficiency in a family with recurrent infections. Immunopharmacology. 1997 Dec. 38(1-2):207-13. [QxMD ... Molecular basis of complement factor I deficiency in Tunisian atypical hemolytic and uremic syndrome Patients. Nephrology ( ...
C3d complement. Clone: E28-P. Cat#: 10095. C3d is involved in the regulation of many aspects of the immune response including ... It is closely related in structure to the epidermal growth factor receptor. C-erbB- 2 oncoprotein is detectable in a proportion ... Activation of the classical complement pathway starts with binding of an activating substance to the Cl molecule, which ...
... a heterozygote variant in complement factor B was localized, potentially implying the diagnosis of a complement mediated TMA ... Marked activation of both T-cells, endothelium and complement were documented. She was successfully treated with antimicrobial ... We discuss the possible interactions between complement activation and other findings in severe infection and argue that ... The use of complement inhibition is well established for complement mediated thrombotic microangiopathy, but its role in ...
... known to bind complement C3b- and C3d-decorated microbial products and, following activation, produce IL-8 (CXCL8), a major ... which is a cause of T cell lymphomas and a candidate environmental factor in autoimmune disease. ... Higher complement receptor 2 (CR2) expression by human naive CD4+ T cells during de novo reconstitution. ... Neonatal and adult recent thymic emigrants produce IL-8 and express complement receptors CR1 and CR2. ...
complement C3d receptor 2 [Source:HGNC S.... CSF3. 1440. CSF3. colony stimulating factor 3 [Source:HGNC.... ... TFT: Transcription Factor Targets TFT:GTRD: GTRD TFT:TFT_Legacy: TFT_Legacy C4: Computational CGN: Cancer Gene Neighborhoods CM ...
Associations of complement factor B and complement component 2 genotypes with subtypes of polypoidal choroidal vasculopathy. ... Impact of the common genetic associations of age-related macular degeneration upon systemic complement component C3d levels. ... The effect of complement factor B gene variation on age-related macular degeneration in Iranian patients. Journal of current ... Role of complement factor B rs4151667 (L9H) polymorphisms and its interactional role with CFH Y402H and C3 rs2230199 (R102G) ...
... von Willebrand factor, IgG, IgM, IgD, complement/C3c, C3d, myeloperoxidase, and carcinoembryonic antigen (P , 0.05). ... Autoreactivity to blood vessels was also detected, with multiple immunoglobulins and complement factors. Conclusions: We ...
complement C3d receptor 2 [Source:HGNC.... CSF1. 1435. CSF1. colony stimulating factor 1 [Source:HG.... ... TFT: Transcription Factor Targets TFT:GTRD: GTRD TFT:TFT_Legacy: TFT_Legacy C4: Computational CGN: Cancer Gene Neighborhoods CM ...
7) complement, such as a trimer of complement component C3d.. In another embodiment, the adjuvant is a mixture of 2, 3, or more ... granulocyte macrophage colony stimulating factor (GM-CSF), macrophage colony stimulating factor (M-CSF), tumor necrosis factor ... EP0471177), cytokines, lymphokines, growth factors or hormones (See International Patent Application Publication No. WO 91/ ... complement and HL-60 cells, 10 μl of the opsonophagocytic reaction was transferred to an individual well on a Millipore 96-well ...
One possible explanation is individual genetic polymorphism in different complement regulators, such as factor H,31,32 factor I ... Complement assessment.. CH50 activity, C3d deposition, and C3 and C4 circulating levels were measured concomitantly to clinical ... Factor H mediated cell surface protection from complement is critical for the survival of PNH erythrocytes. ... complement hemolytic activity assay, complement system proteins, eculizumab, paroxysmal nocturnal hemoglobinuria, hemolysis ...
Complement receptors have only been identified in the last 2 decades. ... The complement system, interacting with its regulatory molecules and cellular receptors, plays a central role in the induction ... CR1 also regulates complement activation by acting as a cofactor for factor I in the cleavage and degradation of bound C3b and ... C3d-or C3dg-bearing immune complexes can bind to CR2. C3dg and C3d also can bind to the surface of bacteria and fungi, allowing ...
Complement receptors have only been identified in the last 2 decades. ... The complement system, interacting with its regulatory molecules and cellular receptors, plays a central role in the induction ... CR1 also regulates complement activation by acting as a cofactor for factor I in the cleavage and degradation of bound C3b and ... C3d-or C3dg-bearing immune complexes can bind to CR2. C3dg and C3d also can bind to the surface of bacteria and fungi, allowing ...
Complement Activation in Kidneys of Patients With COVID-19.jpeg ... Both C3 cleavage products C3c and C3d were strongly detected in ... Using immunohistochemistry for complement factors C1q, MASP-2, C3c, C3d, C4d, and C5b-9 we investigated the involvement of the ... Therefore, activation of the complement system might be involved in the worsening of renal injury. Complement inhibition might ... The complement system is an important component of the innate immune system that is involved in the opsonization of viruses but ...
No antibody or complement coating of erythrocytes was found that could trigger bystander hemolysis of uninfected erythrocytes. ... Secondary objectives were to compare in patients with posttreatment hemolysis and those without it possible risk factors (age, ... None of the patients with hemolysis showed coating of erythrocytes with IgG, IgM, or C3d. ... posttreatment hemolysis after antimalarial treatment might be a clinically relevant factor. Recently, a large study in Nigeria ...
Normally warm antibody AIHA (WAIHA) is readily detectable in RBCs coated with IgG or IgG + complement; if only with C3d, the ... Diagnosis of secondary AIHA includes evaluation of patients status, onset of illness, possible predisposing factors such as ... IgM-coated RBCs are bound by complement at the level of C3 resulting in the formation of a complex with other complement ... Autoantibody and complement-coated red cells rupture or lyze and are eliminated by phagocytic while blood cells (phagocytes) in ...
Cobra venom factor: evidence for its being altered cobra C3 (the third component of complement). Science 191:1275. ... did not have elevated C3d levels. In TNF-α-galactosamine-induced lethal hepatitis, levels of total complement that produces 50 ... The role of complement activation in tumour necrosis factor-induced lethal hepatitis. Cytokine 11:617. ... CVF depletion of complement. To achieve virtually complete depletion of serum complement, CVF (50 μg/animal) was injected i.p. ...
Complement components factor H CCP19-20 (S1191L mutant) and C3D in complex. ... NMR structure of CCP modules 2-3 of complement factor H. 2uwn. Crystal structure of Human Complement Factor H, SCR domains 6-8 ... Crystal Structure of Complement C3b in Complex with Factor B. 2yby. Structure of domains 6 and 7 of the mouse complement ... Solution structure of CCP modules 10-11 of complement factor H. 4b2s. Solution structure of CCP modules 11-12 of complement ...
PMID 18806293] Analysis of rare variants in the complement component 2 (C2) and factor B (BF) genes refine association for age- ... Impact of the common genetic associations of age-related macular degeneration upon systemic complement component C3d levels ... Age-related macular degeneration 14 Atypical hemolytic uremic syndrome Macular degeneration Complement component 2 deficiency ... PMID 23313922] Complement gene single nucleotide polymorphisms and biomarker endophenotypes of Alzheimers disease ...
Complement components factor H CCP19-20 (S1191L mutant) and C3D in complex. ... NMR structure of CCP modules 2-3 of complement factor H. 2uwn. Crystal structure of Human Complement Factor H, SCR domains 6-8 ... Crystal Structure of Complement C3b in Complex with Factor B. 2yby. Structure of domains 6 and 7 of the mouse complement ... Solution structure of CCP modules 10-11 of complement factor H. 4b2s. Solution structure of CCP modules 11-12 of complement ...
GO:0006957 complement activation, alternative pathway GO:0006958 complement activation, classical pathway GO:0007186 G-protein ... Other proteases in vitro have been shown to cleave Cdg into C3d and C3g; however this has not been observed in vivo and thus is ... C3b is not stable in serum and is rapidly cleaved by factor I and a cofactor to form the more stable fragment iC3b (inactivated ... Complement C3g Fragment. UniProt. P01024 Entrez Gene. C3 GO Terms. GO:0005102 receptor binding GO:0004866 endopeptidase ...
Complement receptor type 2 (CR2) (Complement C3D receptor) (Epstein-Barr virus receptor) CD22 CD22_HUMAN P20273 107266 CD22 B- ... Complement decay-accelerating factor CD56 NCA1_HUMAN P13591 116930 NCAM1; NCAM Neural cell adhesion molecule 1 NCA2_HUMAN ... TNFR1 Tumor necrosis factor receptor 1 (Tumor necrosis factor binding protein 1) (TBPI) (p60) (TNF-R1) (p55) CD120b TR1B_HUMAN ... Complement component C1q receptor (Complement component 1, q subcomponent, receptor 1) (C1qRp) (C1qR(p)) (C1q/MBL/SPA receptor ...
Several complement-regulatory and complement-inhibitory proteins operate at different levels of the cascade particularly in the ... 1 2 Three essential tyrosines Tyr239 Tyr240 and Tyr317 in p52are phosphorylated pursuing growth factor arousal. These ... C3d Bb C5a) and increased soluble C5b-9 levels. Whereas C5 and properdin levels were significantly decreased in both DDD and ... classic lectin or choice complement pathways (CP LP or AP respectively).. * Post author By californiaehealth ...
Toapanta, Franklin Ramiro (2006) Immune Enhancement Mechanisms by the Complement Protein C3d. Doctoral Dissertation, University ... Lavasani, Mitra and Lu, Aiping and Peng, Hairong and Cummins, James and Huard, Johnny (2006) Nerve growth factor improves the ... Ghosh, P and Wasil, LR and Hatfull, GF (2006) Control of phage Bxb1 excision by a novel recombination directionality factor. ... Galletta, Dennis F (2006) Human Factors and E-Commerce. In: E-Commerce and the Digital Economy. M.E. Sharpe, Armonk, 91 - 111. ...
Targeted complement inhibition by C3d recognition ameliorates tissue injury without apparent increase in susceptibility to ... Exploring factors associated with nurses adoption of an evidence-based practice to reduce catheter-associated urinary tract ... Mice;Receptors, Complement 3d;Biological Availability;Half-Life;Complement C3b;Complement Activation;Peptide Fragments; ... Exploring factors associated with nurses adoption of an evidence-based practice to reduce catheter-associated urinary tract ...
C3d of complement as a molecular adjuvant: bridging innate and acquired immunity. Science ... the guanine-nucleotide exchange factor for the Rho family of small GTPases (13). Significantly, the coligation of the BCR and ... the receptor for the C3d component of complement; and Leu 13 (CD225), a small transmembrane IFN-α- and IFN-γ-inducible protein ... fused to three tandemly arranged copies of C3d (HEL-C3d) require 1/1000th the Ag dose of HEL alone to elicit the same Ab ...
intrinsic-factor protects B12 from pancreatic proteolysis + permits absorption in terminal ileum (bind to intrinsic factor ... EBV glycoprotein gp350 binds to C3d complement component receptor CD21 50 Q ...
  • Here, by differential gene expression analysis followed by protein expression and functional studies, we define that the naive T cells having divided the least since thymic emigration express complement receptors (CR1 and CR2) known to bind complement C3b- and C3d-decorated microbial products and, following activation, produce IL-8 (CXCL8), a major chemoattractant for neutrophils in bacterial defense. (jci.org)
  • The complement system, interacting with its regulatory molecules and cellular receptors, plays a central role in the induction and regulation of immunity. (medscape.com)
  • Complement receptors have only been identified in the last 2 decades. (medscape.com)
  • Effector proteins interacting with the Fc portion of immunoglobulin M (IgM) include complement and complement receptors. (medscape.com)
  • Partial or complete deficiencies of the components of the complement system, including its receptors and regulatory proteins, are now described in humans and may be of a genetic or familial origin or acquired. (medscape.com)
  • Four distinct complement receptors, CR1, CR2, CR3, and CR4, have been described for the surface-bound complement fraction C3 and its cleavage fragments. (medscape.com)
  • Complement and complement receptors play a critical role in immune defense by initiating the rapid destruction of invading microorganisms, amplifying the innate and adaptive immune responses, and mediating solubilization and clearance of immune complexes. (medscape.com)
  • Defects in the expression of complement or complement receptors may result in loss of tolerance to self-proteins and the development of immune complex-mediated autoimmune diseases such as systemic lupus erythematosus (SLE). (medscape.com)
  • One-day preoperative serum levels of immunoglobulins, complement, cytokines and chemokines, soluble adhesion molecules and receptors as well as clinical chemistry parameters such as differential counts, creatinine, blood coagulation status (altogether 56 parameters) were analyzed in peripheral blood samples of 75 children (aged 3-18 years) undergoing cardiopulmonary bypass surgery (29 with postoperative effusions and edema within the first postoperative week). (biomedcentral.com)
  • This effect may be partly mediated by complement receptors on the surface of B lymphocytes that bind immunogenic complexes bearing fragments of C3 and C4. (elsevier.com)
  • We have shown by immunoprecipitation analysis that the two complement receptors expressed by B lymphocytes, complement receptor 1 (CR1) and CR2, form a detergent-sensitive complex on the surface of tonsillar B lymphocytes and on K562 erythroleukemia cells that were co-transfected with cDNAs encoding CR1 and CR2. (elsevier.com)
  • Receptors, Complement" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (ouhsc.edu)
  • This graph shows the total number of publications written about "Receptors, Complement" by people in this website by year, and whether "Receptors, Complement" was a major or minor topic of these publications. (ouhsc.edu)
  • Below are the most recent publications written about "Receptors, Complement" by people in Profiles. (ouhsc.edu)
  • Of all the lectin proteins, only MBL has been shown to have the ability to activate the complement system. (medscape.com)
  • The complement control protein (CCP) modules (also known as short consensus repeats SCRs or SUSHI repeats) contain approximately 60 amino acid residues and have been identified in several proteins of the complement system. (embl.de)
  • These modules have been identified more than 140 times in over 20 proteins, including 12 proteins of the complement system. (embl.de)
  • Genes that encode the proteins of complement components or their isotypes are distributed throughout different chromosomes, with 19 genes comprising 3 significant complement gene clusters in the human genome. (medscape.com)
  • The important components of this system are various cell membrane-associated proteins such as complement receptor 1 (CR1), complement receptor 2 (CR2), and decay accelerating factor (DAF). (medscape.com)
  • Complement proteins circulate in the blood as inactive precursors, but are immediately activated upon contact with target cells. (biomedcentral.com)
  • For optimal comprehension, the student needs to be familiar with all lymphoid organs and tissues, the types of immune cells from both the erythroid and lymphoid lines, and the biochemistry of immune globulins (Ig) and complement proteins. (lecturio.com)
  • The use of complement inhibition is well established for complement mediated thrombotic microangiopathy, but its role in secondary forms of thrombotic microangiopathy is debated. (biomedcentral.com)
  • We discuss the possible interactions between complement activation and other findings in severe infection and argue that complement inhibition proved beneficial to this patient's rapid recovery. (biomedcentral.com)
  • Complement inhibition might thus be a promising treatment option to prevent deregulated activation and subsequent collateral tissue injury. (figshare.com)
  • The effect of systemic levels of TNF-alpha and complement pathway activity on outcomes of VEGF inhibition in neovascular AMD. (cdc.gov)
  • 1. Berentsen S. Complement activation and inhibition in autoimmune hemolytic anemia: focus on cold agglutinin disease. (understandingcad.com)
  • The clinical history of patients with classic pathway deficiencies varies slightly from other complement-deficient patients. (medscape.com)
  • In the alternative pathway, a deficiency of factor B, factor D, or properdin can result in a decreased amount of C3b. (medscape.com)
  • Activation of the classical complement pathway starts with binding of an activating substance to the Cl molecule, which activates the Cl sub-units, resulting in cleavage of C4 to C4b releasing C4a in the process. (immbio.hu)
  • In conclusion, various complement pathways were activated in COVID-19 kidneys, the lectin pathway mainly in peritubular capillaries and in part the classical pathway in renal arteries whereas the alternative pathway seem to be crucial for tubular complement activation. (figshare.com)
  • Association between polymorphisms of complement pathway genes and age-related macular degeneration in a Chinese population. (snpedia.com)
  • The complement cascade consists of 3 separate pathways that converge in a final common pathway. (medscape.com)
  • Lectins activate the lectin pathway in a manner similar to the antibody interaction with complement in the classical pathway. (medscape.com)
  • Complement lectin pathway protein levels reflect disease activity in juvenile idiopathic arthritis: a longitudinal study of the Nordic JIA cohort. (omicsdi.org)
  • Human brain trauma severity is associated with lectin complement pathway activation. (omicsdi.org)
  • We explored the involvement of the lectin pathway of complement in post-traumatic brain injury (TBI) pathophysiology in humans. (omicsdi.org)
  • Imaging analysis indicated that lectin pathway initiator molecules (MBL, ficolin-1, ficolin-2 and ficolin-3), the key enzymes MASP-2 and MASP-3, and the downstream complement components (C3 fragments and TCC) were present inside and outside brain vessels in all contusions. (omicsdi.org)
  • Factors involved in initiation and regulation of complement lectin pathway influence postoperative outcome after pediatric cardiac surgery involving cardiopulmonary bypass. (omicsdi.org)
  • We have investigated some factors of the innate immune system involved in the initiation or regulation of complement lectin pathway activation (MASP-1, MASP-2 MASP-3, MAp19, MAp44, ficolin-3) and related them to complications and prognosis in 190 pediatric patients undergoing CHD repair with the use of cardiopulmonary bypass (CPB). (omicsdi.org)
  • In cold agglutinin disease, certain abnormal bone marrow cells produce antibodies called cold agglutinins, which activate a part of the immune system known as the complement pathway. (understandingcad.com)
  • Complement receptor type 1 (CR1) also known as C3b/C4b receptor or CD35 (cluster of differentiation 35) is a protein that in humans is encoded by the CR1 gene. (wikipedia.org)
  • The protein mediates cellular binding to particles and immune complexes that have activated complement. (wikipedia.org)
  • In 5'-3' order the genes in this region are: membrane cofactor protein - CR1 - complement receptor type 2 - decay-accelerating factor - C4-binding protein. (wikipedia.org)
  • Factor H, another immunoregulatory protein, also maps to this location. (wikipedia.org)
  • Mouse Transforming growth factor-beta-induced protein ig-h3 (TGFBI) ELISA kit is Available at Gentaur Genprice with the fastest delivery. (joplink.net)
  • CusabioMouse Insulin-like growth factor binding protein 3, IGFBP-3 ELISA KIT is Available at Gentaur Genprice. (joplink.net)
  • CusabioHuman Transforming growth factor-beta-induced protein ig-h3 (TGFBI/BIGH3) ELISA kit is. (joplink.net)
  • CusabioHuman latent transforming growth factor beta binding protein 2, LTBP2 ELISA Kit is Available at. (joplink.net)
  • The complement protein C3b, along with its cleavage product C3bi, is a potent agent of opsonization in the complement cascade. (medscape.com)
  • In order to generate an antibody response, an antigen must bind to the complement receptor (CR2) on B cells and the complement protein C3d. (medscape.com)
  • The MBL protein can activate the C4 and C2 components of complement by forming a complex with serine proteases known as MASP1 and MASP2. (medscape.com)
  • They are single-chain molecules present on host cellular membranes and belong to the complement control protein family. (medscape.com)
  • The sushi domain is also known as the complement controle protein (CCP) module or the short consensus repeat (SCR). (embl.de)
  • Three-dimensional structure of a complement control protein module in solution. (embl.de)
  • The complement control protein (CCP) modules (also known as short consensus repeats) are defined by a consensus sequence within a stretch of about 60 amino acid residues. (embl.de)
  • Note that, in the absence of antibody, many of the molecules that activate the complement system are carbohydrate or lipid in nature (e.g. lipopolysaccharides, mannose), suggesting that the system evolved mainly to recognize bacterial surfaces via their non-protein features. (pediagenosis.com)
  • Complement is a large protein network in plasma that is crucial for human immune defenses and a major cause of aberrant inflammatory reactions. (biomedcentral.com)
  • The complement system is a large protein network in plasma that forms the primary host defense barrier against microbial infections [ 1 , 2 ]. (biomedcentral.com)
  • These C3 convertases cleave the major complement protein C3 into the large, reactive C3b molecule that can covalently attach to target surfaces to label them for rapid opsonization and phagocytosis [ 7 ]. (biomedcentral.com)
  • Complement C3, C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1, C3, CPAMD1. (prospecbio.com)
  • C5a - A proteolytic product of the complement protein C5. (drg-diagnostics.de)
  • C3d is involved in the regulation of many aspects of the immune response including antigen processing and presentation. (immbio.hu)
  • The C3d/antigen complex mediates B cell activation. (immbio.hu)
  • When combined with our LIFECODES LSA Single Antigen Kits, the assay offers a simple method for the detection of complement binding to HLA antibodies. (immucor.com)
  • The complement system augments the humoral immune response to low concentrations of antigen. (elsevier.com)
  • This study aims to assess the association of the expression of Lewis antigen as an independent risk factor for CAD separately in males and females. (ajts.org)
  • The individuals were examined for established CAD risk factor and Lewis antigen expression on red cell. (ajts.org)
  • The 3 major sequelae of complement deficiencies, based on the pathophysiology of each defect, are (1) defects that result in inadequate opsonization, (2) defects in cell lysis, and (3) the association of complement deficiencies with immune complex diseases. (medscape.com)
  • The CR1/CR2 complex is distinct from the CR2/CD19 complex and may assist B cell activation by efficiently capturing C3b-containing immunogens and maintaining such immunogens on the B cell after CR1 and factor I-mediated cleavage to iC3b and C3dg. (elsevier.com)
  • Finally, this iC3b is cleaved with trypsin to yield C3d.If the C3b precursor was attached to a surface (as occurs during complement activation) the iC3b, C3dg and C3d fragments would remain covalently attached to that surface.C3d is able to attach to a surfaceonly during complement activation on a surface. (prospecbio.com)
  • In this study, we demonstrate that complement activation products (C3a, C3b/iC3b) are generated in the serum of experimental mice after CCl 4 injection and that complement activation is required for normal liver regeneration. (jimmunol.org)
  • However, C3b is not stable in serum and is rapidly cleaved by factor I and a cofactor to form the more stable fragment iC3b (inactivated C3b) and C3f. (bio-rad-antibodies.com)
  • CD21, also known as CR2 (complement receptor 2) and C3d receptor, binds C3d and iC3b. (biolegend.com)
  • CusabioMouse Transforming growth factor beta receptor type 3 (TGFBR3) ELISA kit is Available at Gentaur. (joplink.net)
  • CusabioHuman Transforming growth factor beta receptor type 3 (TGFBR3) ELISA kit is Available at Gentaur. (joplink.net)
  • The partly folded back solution structure arrangement of the 30 SCR domains in human complement receptor type 1 (CR1) permits access to its C3b and C4b ligands. (ouhsc.edu)
  • Complement receptor type 3 mediates phagocytosis and killing of Listeria monocytogenes by a TNF-alpha- and IFN-gamma-stimulated macrophage precursor hybrid. (ouhsc.edu)
  • Role of complement factor B rs4151667 (L9H) polymorphisms and its interactional role with CFH Y402H and C3 rs2230199 (R102G) risk variants in age-related macular degeneration: a case control study. (cdc.gov)
  • Genetic factors associated with response to as-needed aflibercept therapy for typical neovascular age-related macular degeneration and polypoidal choroidal vasculopathy. (cdc.gov)
  • The effect of complement factor B gene variation on age-related macular degeneration in Iranian patients. (cdc.gov)
  • Association Between Complement Factor C2/C3/CFB/CFH Polymorphisms and Age-Related Macular Degeneration: A Meta-Analysis. (cdc.gov)
  • PMID 18806293 ] Analysis of rare variants in the complement component 2 (C2) and factor B (BF) genes refine association for age-related macular degeneration (AMD). (snpedia.com)
  • Systemic activation of the complement system in patients with advanced age-related macular degeneration. (ucdenver.edu)
  • Therefore, activation of the complement system might be involved in the worsening of renal injury. (figshare.com)
  • Detect C3d-binding donor specific anti-HLA antibodies (DSAs) for better risk stratification of patients. (immucor.com)
  • Our laboratory has focused considerable effort on defining the role of complement in liver regeneration ( 5 , 6 ). (jimmunol.org)
  • Note the image below, as well as the article Pediatric Complement Receptor Deficiency may be helpful. (medscape.com)
  • A missense mutation in seventh CCP domain causes deficiency of the b subunit of factor XIII. (embl.de)
  • Molecular and cellular basis of deficiency of the b subunit for factor XIII secondary to a Cys430-Phe mutation in the seventh Sushi domain. (embl.de)
  • We studied the defect responsible for deficiency of the b subunit for factor XIII in the first known case of this condition. (embl.de)
  • Cases of complement deficiency have helped defined the role of complement in host defense. (medscape.com)
  • A North African study of molecular basis of complement factor I deficiency in atypical hemolytic and uremic syndrome patients suggested that the Ile357Met mutation may be a founding effect. (medscape.com)
  • There are a plenitude of causes, including ADAMTS-13 dependent thrombotic thrombocytopenic purpura (TTP), shigatoxin-associated hemolytic uremic syndrome (HUS), complement mediated TMA (CM-TMA, also called atypical HUS/aHUS) and "secondary TMAs" that can be infection associated (IA-HUS), pregnancy related, drug induced or autoimmune, amongst others. (biomedcentral.com)
  • These findings indicate a need for increased vigilance for hemolytic events in malaria patients, particularly those with predisposing factors for anemia. (cdc.gov)
  • Factor I Autoantibodies in Patients with Atypical Hemolytic Uremic Syndrome: Disease-Associated or an Epiphenomenon? (ncl.ac.uk)
  • It has been shown that CR1 can act as a negative regulator of the complement cascade, mediate immune adherence and phagocytosis and inhibit both the classic and alternative pathways. (wikipedia.org)
  • Activation of the complement cascade always leads to the enzymatic cleavage of complement fraction C3, which is important in host defense to bacterial infections and phagocytosis. (medscape.com)
  • C3, the third component of the complement system, has a central role in the complement cascade. (jimmunol.org)
  • Deficiencies in the complement cascade can lead to overwhelming infection and sepsis. (medscape.com)
  • New studies point to the complex interplay between the complement cascade and adaptive immune response, and complement is also being studied in association with ischemic injury as a target of therapy. (medscape.com)
  • In addition to playing an important role in host defense against infection, the complement system is a mediator in both the pathogenesis and prevention of immune complex diseases, such as systemic lupus erythematosus (SLE). (medscape.com)
  • Although complement is crucial for local clearance of bacteria at the site of infection, complement activation products (especially C5a) can cause an overwhelming inflammatory response during systemic infections [ 4 ]. (biomedcentral.com)
  • The complement system is an important component of the innate immune system that is involved in the opsonization of viruses but also in triggering further immune cell responses. (figshare.com)
  • Complement is traditionally recognized as part of the innate immune system, defending the host against the invasion of foreign pathogens. (jimmunol.org)
  • Activation of complement can be started either via adaptive or innate immune recognition. (pediagenosis.com)
  • The complement system is part of the innate immune system. (medscape.com)
  • Although the complement system is part of the body's innate, relatively nonspecific defense against pathogens, its role is hardly primitive or easily understood. (medscape.com)
  • GCL phosphorylates a microsatellite of a 24:5(n-3 innate cytosol( GCLC) and a human I1-5 complement( GCLM). (familie-vos.de)
  • CR1 also regulates complement activation by acting as a cofactor for factor I in the cleavage and degradation of bound C3b and C4b to its inactive forms. (medscape.com)
  • An intricate system regulates complement activity. (medscape.com)
  • To our knowledge, this is the first description of C. canimorsus associated TMA with verified complement activation, extensive inflammatory workup, and treatment with eculizumab. (biomedcentral.com)
  • Complement can rapidly label bacterial cells for phagocytosis and generate chemoattractants to recruit immune cells to the site of infection [ 2 ]. (biomedcentral.com)
  • Both C3 cleavage products C3c and C3d were strongly detected in renal arteries but also occurs in glomerular capillaries of COVID-19 biopsies, while tubular C3d was stronger than C3c in biopsies from COVID-19 patients. (figshare.com)
  • C5a production is a consequence of the enzymatic cleavage of C5 by the C5 convertase, which can be formed only in the presence of active C3 cleavage products that are generated during complement activation. (jimmunol.org)
  • They are encoded by separate but linked genes termed regulation of complement activation (RCA) genes, located on the q32 region of chromosome 1. (medscape.com)
  • Evaluating the Occurrence of Rare Variants in the Complement Factor H Gene in Patients With Early-Onset Drusen Maculopathy. (cdc.gov)
  • Higher complement receptor 2 (CR2) expression by human naive CD4 + T cells during de novo reconstitution. (jci.org)
  • The solution structure of the 16th CCP module from human complement factor H has been determined by a combination of 2-dimensional nuclear magnetic resonance spectroscopy and restrained simulated annealing. (embl.de)
  • The developed model and molecular insights are essential to understand the molecular basis of deregulated complement activity in human disease and will facilitate future design of therapeutic interventions against these critical enzymes in inflammation. (biomedcentral.com)
  • The factors like increased human settlements and encroachments, expanded travel and trade networks, altered wildlife and livestock practices, modernised and mass-farming practices, compromised ecosystems and habitat destruction, and global climate change have impact on the interactions between virome and its hosts and other species and act as drivers of trans-species viral spill-over and human transmission. (heighpubs.org)
  • C3d is derived from human C3 after a series of proteolytic cleavages.C3d is prepared from purified human C3. (prospecbio.com)
  • Human Complement C3d produced in Human plasma having a molecular mass of 33.8kDa. (prospecbio.com)
  • C3d Human is stable at 4°C if entire vial will be used within 2-4 weeks. (prospecbio.com)
  • What offers it give about a layer when data and factors can reverse their salaries on optimistic while receipts, highly uncover them into their talks and please down Proceedings of human activities and lines? (plywoodskyscraper.com)
  • Autoreactivity to blood vessels was also detected, with multiple immunoglobulins and complement factors. (najms.org)
  • In primates, CR1 serves as the main system for processing and clearance of complement opsonized immune complexes. (wikipedia.org)
  • Using immunohistochemistry for complement factors C1q, MASP-2, C3c, C3d, C4d, and C5b-9 we investigated the involvement of the complement system in six kidney biopsies with acute kidney failure in different clinical settings and three kidneys from autopsy material of patients with COVID-19. (figshare.com)
  • Although the complement system has been implicated in liver regeneration after toxic injury and partial hepatectomy, the mechanism or mechanisms through which it participates in these processes remains ill-defined. (jimmunol.org)
  • The complement system plays an important part in defense against pyogenic organisms. (medscape.com)
  • These findings underscore the duality of the complement system. (medscape.com)
  • Knowledge about the complement system is expanding. (medscape.com)
  • Taking the experience of a beneficial factor in autoimmunity further, one might hypothesize with intent to prove that autoimmunity is always a self-defense mechanism of the mammal system to survive. (hatenablog.com)
  • Tumor necrosis-initiated complement activation stimulates proliferation of medulloblastoma cells. (ouhsc.edu)
  • For all histocompatibility laboratories, Complement-Dependent Cytotoxicity (CDC) testing proves to be time consuming and tedious. (immucor.com)
  • Cross matching techniques have also changed from complement-dependent cytotoxicity (which is still considered a gold standard) to microbead-based assay to flow cytometry. (gjtmonline.com)
  • In mice, CR1 is an alternatively spliced variant of the complement receptor 2 (CR2) gene. (wikipedia.org)
  • Complement deficiencies are said to comprise between 1 and 10% of all primary immunodeficiencies. (medscape.com)
  • [ 4 ] A registry of complement deficiencies has been established as a means to promote joint projects on treatment and prevention of diseases associated with defective complement function. (medscape.com)
  • This article outlines some of the disease states associated with complement deficiencies and their clinical implications. (medscape.com)
  • Deficiencies in complement predispose patients to infection via 2 mechanisms: (1) ineffective opsonization and (2) defects in lytic activity (defects in MAC). (medscape.com)
  • Specific complement deficiencies are also associated with an increased risk of developing autoimmune disease, such as SLE. (medscape.com)
  • C3 (MW 180 000), the central component of all complement reac- tions, split by its convertase into a small (C3a) and a large (C3b) fragment. (pediagenosis.com)
  • Furthermore, there is a large list of inflammatory disorders in which erroneous activation of complement on the body's own cells causes aberrant inflammatory reactions and pathology [ 5 ]. (biomedcentral.com)
  • The functions of CR1 and CR2 on T cells remain to be determined, but we note that CR2 is the receptor for Epstein-Barr virus, which is a cause of T cell lymphomas and a candidate environmental factor in autoimmune disease. (jci.org)
  • I forgot to mention that I also ran a precision allergy test by Precision Point Diagnostics because they test IgE, IgG, IgG4 and complement (C3d) from blood serum. (libsyn.com)
  • Some new clinical entities are linked with partial complement defects. (medscape.com)
  • There are over 90 known serotypes of pneumococci, and the capsule is the principle virulence determinant for pneumococci, as the capsule not only protects the inner surface of the bacteria from complement, but is itself poorly immunogenic. (justia.com)
  • Furthermore, complement directly kills bacteria via a pore-forming membrane attack complex [ 3 ]. (biomedcentral.com)
  • After several weeks, a heterozygote variant in complement factor B was localized, potentially implying the diagnosis of a complement mediated TMA over an isolated infection related TMA. (biomedcentral.com)
  • Purpose: To describe bleeding and thrombotic events and their risk factors in patients receiving extracorporeal membrane oxygenation (ECMO) for severe coronavirus disease 2019 (COVID-19) and to evaluate their impact on in-hospital mortality. (researchgate.net)
  • The defect in persons with Leiner disease is usually attributed to a defect of the fifth component of complement (C5). (medscape.com)
  • However, a child was described by Sonea and associates who had Leiner disease associated with diminished C3, and another was described by Goodyear and Harper with a low level of the fourth component of complement and reduced neutrophil mobility. (medscape.com)
  • at the same time, the inflammation promoted by complement activation can result in cellular damage when not kept in check. (medscape.com)
  • Complement activity can activate C5, leading to intravascular hemolysis and chronic inflammation. (understandingcad.com)
  • However, several recent reports have described novel "unconventional" functions of complement components ( 1 ). (jimmunol.org)
  • Molecules on the surface of some B-lymphocytes and macrophages, that recognize and combine with the C3b, C3d, C1q, and C4b components of complement. (ouhsc.edu)
  • Two 'C3b inactivator' enzymes rapidly inactivate C3b, releasing the fragment C3c and leaving membrane bound C3d. (pediagenosis.com)
  • The complement activating immunogen may then trigger signal transduction by the CR1/CR2 complex, the CR2/CD19 complex, or membrane immunoglobulin. (elsevier.com)
  • Find out if your dog could pass down genetic risk factors to their puppies. (wisdompanel.com)
  • Find out if they carry genetic factors that could affect the health of their future puppies. (wisdompanel.com)