Complement c1 inactivator proteins. Medical search

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.

Serum proteins that inhibit, antagonize, or inactivate COMPLEMENT C1 or its subunits.

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.

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.

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.

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.

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.

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.

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 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 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).

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 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 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 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).

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 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.

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.

A 93-kDa serum glycoprotein encoded by C7 gene. It is a polypeptide chain with 28 disulfide bridges. In the formation of MEMBRANE ATTACK COMPLEX; C7 is the next component to bind the C5b-6 complex forming a trimolecular complex C5b-7 which is lipophilic, resembles an integral membrane protein, and serves as an anchor for the late complement components, C8 and C9.

Complement 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.

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).

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.

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.

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.

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.

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.

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.

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.

The destruction of ERYTHROCYTES by many different causal agents such as antibodies, bacteria, chemicals, temperature, and changes in tonicity.

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.

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.

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).

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.

Proteins that are present in blood serum, including SERUM ALBUMIN; BLOOD COAGULATION FACTORS; and many other types of proteins.

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)

A metallocarboxypeptidase that removes C-terminal basic amino acid from peptides and proteins, with preference shown for lysine over arginine. It is a plasma zinc enzyme that inactivates bradykinin and anaphylatoxins.

Serum proteins with an electrophoretic mobility that falls between ALPHA-GLOBULINS and GAMMA-GLOBULINS.

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.

The rate dynamics in chemical or physical systems.

A technique that combines protein electrophoresis and double immunodiffusion. In this procedure proteins are first separated by gel electrophoresis (usually agarose), then made visible by immunodiffusion of specific antibodies. A distinct elliptical precipitin arc results for each protein detectable by the antisera.

A major cytochrome P-450 enzyme which is inducible by PHENOBARBITAL in both the LIVER and SMALL INTESTINE. It is active in the metabolism of compounds like pentoxyresorufin, TESTOSTERONE, and ANDROSTENEDIONE. This enzyme, encoded by CYP2B1 gene, also mediates the activation of CYCLOPHOSPHAMIDE and IFOSFAMIDE to MUTAGENS.

The engulfing and degradation of microorganisms; other cells that are dead, dying, or pathogenic; and foreign particles by phagocytic cells (PHAGOCYTES).

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.

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).

Mercuriphenols substituted with one or more chlorine atoms and one or more nitro groups. Some of these are sulfhydryl reagents which act as chromophoric probes in enzymes and other proteins.

Important enzymes in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY. They cleave COMPLEMENT C3 and COMPLEMENT C5.

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.

Swelling involving the deep DERMIS, subcutaneous, or submucosal tissues, representing localized EDEMA. Angioedema often occurs in the face, lips, tongue, and larynx.

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.

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.

The most common mineral of a group of hydrated aluminum silicates, approximately H2Al2Si2O8-H2O. It is prepared for pharmaceutical and medicinal purposes by levigating with water to remove sand, etc. (From Merck Index, 11th ed) The name is derived from Kao-ling (Chinese: "high ridge"), the original site. (From Grant & Hackh's Chemical Dictionary, 5th ed)

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.

All blood proteins except albumin ( = SERUM ALBUMIN, which is not a globulin) and FIBRINOGEN (which is not in the serum). The serum globulins are subdivided into ALPHA-GLOBULINS; BETA-GLOBULINS; and GAMMA-GLOBULINS on the basis of their electrophoretic mobilities. (From Dorland, 28th ed)

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.

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.

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.

Compounds or agents that combine with an enzyme in such a manner as to prevent the normal substrate-enzyme combination and the catalytic reaction.

Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN.

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.

The parts of a macromolecule that directly participate in its specific combination with another molecule.

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.

Benzoate derivatives that contain one or more alkyl or aryl groups linked to the benzene ring structure by OXYGEN.

An oxidoreductase involved in pyrimidine base degradation. It catalyzes the catabolism of THYMINE; URACIL and the chemotherapeutic drug, 5-FLUOROURACIL.

Hydrocarbons with at least one triple bond in the linear portion, of the general formula Cn-H2n-2.

Proteolytic enzymes from the serine endopeptidase family found in normal blood and urine. Specifically, Kallikreins are potent vasodilators and hypotensives and increase vascular permeability and affect smooth muscle. They act as infertility agents in men. Three forms are recognized, PLASMA KALLIKREIN (EC 3.4.21.34), TISSUE KALLIKREIN (EC 3.4.21.35), and PROSTATE-SPECIFIC ANTIGEN (EC 3.4.21.77).

Electrophoresis in which a polyacrylamide gel is used as the diffusion medium.

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.

Electrophoresis applied to BLOOD PROTEINS.

Carboxypeptidases that are primarily found the DIGESTIVE SYSTEM that catalyze the release of C-terminal amino acids. Carboxypeptidases A have little or no activity for hydrolysis of C-terminal ASPARTIC ACID; GLUTAMIC ACID; ARGININE; LYSINE; or PROLINE. This enzyme requires ZINC as a cofactor and was formerly listed as EC 3.4.2.1 and EC 3.4.12.2.

Chromatography on non-ionic gels without regard to the mechanism of solute discrimination.

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.

An enzyme that converts brain gamma-aminobutyric acid (GAMMA-AMINOBUTYRIC ACID) into succinate semialdehyde, which can be converted to succinic acid and enter the citric acid cycle. It also acts on beta-alanine. EC 2.6.1.19.

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).

A novel diuretic with uricosuric action. It has been proposed as an antihypertensive agent.

Partial proteins formed by partial hydrolysis of complete proteins or generated through PROTEIN ENGINEERING techniques.

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.

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.

The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.

Conjugated protein-carbohydrate compounds including mucins, mucoid, and amyloid glycoproteins.

Proteins prepared by recombinant DNA technology.

A sympathomimetic agent with properties similar to DEXTROAMPHETAMINE. It is used in the treatment of obesity. (From Martindale, The Extra Pharmacopoeia, 30th ed, p1222)

Serine proteinase inhibitors which inhibit trypsin. They may be endogenous or exogenous compounds.

Elements of limited time intervals, contributing to particular results or situations.

A cluster of convoluted capillaries beginning at each nephric tubule in the kidney and held together by connective tissue.

A large group of cytochrome P-450 (heme-thiolate) monooxygenases that complex with NAD(P)H-FLAVIN OXIDOREDUCTASE in numerous mixed-function oxidations of aromatic compounds. They catalyze hydroxylation of a broad spectrum of substrates and are important in the metabolism of steroids, drugs, and toxins such as PHENOBARBITAL, carcinogens, and insecticides.

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.

Serum proteins that have the most rapid migration during ELECTROPHORESIS. This subgroup of globulins is divided into faster and slower alpha(1)- and alpha(2)-globulins.

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 product of the lysis of plasminogen (profibrinolysin) by PLASMINOGEN activators. It is composed of two polypeptide chains, light (B) and heavy (A), with a molecular weight of 75,000. It is the major proteolytic enzyme involved in blood clot retraction or the lysis of fibrin and quickly inactivated by antiplasmins.

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.

The sum of the weight of all the atoms in a molecule.

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.

An acute hypersensitivity reaction due to exposure to a previously encountered ANTIGEN. The reaction may include rapidly progressing URTICARIA, respiratory distress, vascular collapse, systemic SHOCK, and death.

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.

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.

Thickening of the walls of small ARTERIES or ARTERIOLES due to cell proliferation or HYALINE deposition.

An enzyme that transfers methyl groups from O(6)-methylguanine, and other methylated moieties of DNA, to a cysteine residue in itself, thus repairing alkylated DNA in a single-step reaction. EC 2.1.1.63.

Antibodies produced by a single clone of cells.

Enzymes that act at a free C-terminus of a polypeptide to liberate a single amino acid residue.

Specific, characterizable, poisonous chemicals, often PROTEINS, with specific biological properties, including immunogenicity, produced by microbes, higher plants (PLANTS, TOXIC), or ANIMALS.

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.

A serine endopeptidase that is formed from TRYPSINOGEN in the pancreas. It is converted into its active form by ENTEROPEPTIDASE in the small intestine. It catalyzes hydrolysis of the carboxyl group of either arginine or lysine. EC 3.4.21.4.

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.

Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.

The processes triggered by interactions of ANTIBODIES with their ANTIGENS.

A membrane or barrier with micrometer sized pores used for separation purification processes.

Technique involving the diffusion of antigen or antibody through a semisolid medium, usually agar or agarose gel, with the result being a precipitin reaction.

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.)

A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.

A protease of broad specificity, obtained from dried pancreas. Molecular weight is approximately 25,000. The enzyme breaks down elastin, the specific protein of elastic fibers, and digests other proteins such as fibrin, hemoglobin, and albumin. EC 3.4.21.36.

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.

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.

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.

A single-chain polypeptide derived from bovine tissues consisting of 58 amino-acid residues. It is an inhibitor of proteolytic enzymes including CHYMOTRYPSIN; KALLIKREIN; PLASMIN; and TRYPSIN. It is used in the treatment of HEMORRHAGE associated with raised plasma concentrations of plasmin. It is also used to reduce blood loss and transfusion requirements in patients at high risk of major blood loss during and following open heart surgery with EXTRACORPOREAL CIRCULATION. (Reynolds JEF(Ed): Martindale: The Extra Pharmacopoeia (electronic version). Micromedex, Inc, Englewood, CO, 1995)

A cytochrome P-450 suptype that has specificity for a broad variety of lipophilic compounds, including STEROIDS; FATTY ACIDS; and XENOBIOTICS. This enzyme has clinical significance due to its ability to metabolize a diverse array of clinically important drugs such as CYCLOSPORINE; VERAPAMIL; and MIDAZOLAM. This enzyme also catalyzes the N-demethylation of ERYTHROMYCIN.

The relationship between the dose of an administered drug and the response of the organism to the drug.

A superfamily of hundreds of closely related HEMEPROTEINS found throughout the phylogenetic spectrum, from animals, plants, fungi, to bacteria. They include numerous complex monooxygenases (MIXED FUNCTION OXYGENASES). In animals, these P-450 enzymes serve two major functions: (1) biosynthesis of steroids, fatty acids, and bile acids; (2) metabolism of endogenous and a wide variety of exogenous substrates, such as toxins and drugs (BIOTRANSFORMATION). They are classified, according to their sequence similarities rather than functions, into CYP gene families (>40% homology) and subfamilies (>59% homology). For example, enzymes from the CYP1, CYP2, and CYP3 gene families are responsible for most drug metabolism.

The movement of leukocytes in response to a chemical concentration gradient or to products formed in an immunologic reaction.

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.

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 characteristic feature of enzyme activity in relation to the kind of substrate on which the enzyme or catalytic molecule reacts.

A basic science concerned with the composition, structure, and properties of matter; and the reactions that occur between substances and the associated energy exchange.

Techniques used to separate mixtures of substances based on differences in the relative affinities of the substances for mobile and stationary phases. A mobile phase (fluid or gas) passes through a column containing a stationary phase of porous solid or liquid coated on a solid support. Usage is both analytical for small amounts and preparative for bulk amounts.

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.

The composition, conformation, and properties of atoms and molecules, and their reaction and interaction processes.

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).

Closed vesicles of fragmented endoplasmic reticulum created when liver cells or tissue are disrupted by homogenization. They may be smooth or rough.

Models used experimentally or theoretically to study molecular shape, electronic properties, or interactions; includes analogous molecules, computer-generated graphics, and mechanical structures.

An IgG autoantibody against the ALTERNATIVE PATHWAY C3 CONVERTASE, found in serum of patients with MESANGIOCAPILLARY GLOMERULONEPHRITIS. The binding of this autoantibody to C3bBb stabilizes the enzyme thus reduces the actions of C3b inactivators (COMPLEMENT FACTOR H; COMPLEMENT FACTOR I). This abnormally stabilized enzyme induces a continuous COMPLEMENT ACTIVATION and generation of C3b thereby promoting the assembly of MEMBRANE ATTACK COMPLEX and cytolysis.

Multi-subunit proteins which function in IMMUNITY. They are produced by B LYMPHOCYTES from the IMMUNOGLOBULIN GENES. They are comprised of two heavy (IMMUNOGLOBULIN HEAVY CHAINS) and two light chains (IMMUNOGLOBULIN LIGHT CHAINS) with additional ancillary polypeptide chains depending on their isoforms. The variety of isoforms include monomeric or polymeric forms, and transmembrane forms (B-CELL ANTIGEN RECEPTORS) or secreted forms (ANTIBODIES). They are divided by the amino acid sequence of their heavy chains into five classes (IMMUNOGLOBULIN A; IMMUNOGLOBULIN D; IMMUNOGLOBULIN E; IMMUNOGLOBULIN G; IMMUNOGLOBULIN M) and various subclasses.

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).

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.

Chemical substances that attract or repel cells. The concept denotes especially those factors released as a result of tissue injury, microbial invasion, or immunologic activity, that attract LEUKOCYTES; MACROPHAGES; or other cells to the site of infection or insult.

The formation of a solid in a solution as a result of a chemical reaction or the aggregation of soluble substances into complexes large enough to fall out of solution.

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).

Nicotinamide adenine dinucleotide phosphate. A coenzyme composed of ribosylnicotinamide 5'-phosphate (NMN) coupled by pyrophosphate linkage to the 5'-phosphate adenosine 2',5'-bisphosphate. It serves as an electron carrier in a number of reactions, being alternately oxidized (NADP+) and reduced (NADPH). (Dorland, 27th ed)

Liquid chromatographic techniques which feature high inlet pressures, high sensitivity, and high speed.

Hydrolases that specifically cleave the peptide bonds found in PROTEINS and PEPTIDES. Examples of sub-subclasses for this group include EXOPEPTIDASES and ENDOPEPTIDASES.

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).

The process of cleaving a chemical compound by the addition of a molecule of water.

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.

An amino acid produced in the urea cycle by the splitting off of urea from arginine.

A subclass of EXOPEPTIDASES that act on the free N terminus end of a polypeptide liberating a single amino acid residue. EC 3.4.11.

The location of the atoms, groups or ions relative to one another in a molecule, as well as the number, type and location of covalent bonds.

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.

Conversion of an inactive form of an enzyme to one possessing metabolic activity. It includes 1, activation by ions (activators); 2, activation by cofactors (coenzymes); and 3, conversion of an enzyme precursor (proenzyme or zymogen) to an active enzyme.

Transport proteins that carry specific substances in the blood or across cell membranes.

Domesticated bovine animals of the genus Bos, usually kept on a farm or ranch and used for the production of meat or dairy products or for heavy labor.

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.

The class of all enzymes catalyzing oxidoreduction reactions. The substrate that is oxidized is regarded as a hydrogen donor. The systematic name is based on donor:acceptor oxidoreductase. The recommended name will be dehydrogenase, wherever this is possible; as an alternative, reductase can be used. Oxidase is only used in cases where O2 is the acceptor. (Enzyme Nomenclature, 1992, p9)

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.

Familial anglo-oedema--a particularly severe form. (1/297)

A case of hereditary angio-oedema is described together with the family history and manifestations in the father of the patient. The problems encountered in his management are discussed, including tracheostomy and genetic counselling. (+info)

Consumption of C4b-binding protein (C4BP) during in vivo activation of the classical complement pathway. (2/297)

C4BP has a central role in regulating the classical complement (C') pathway, but it is still uncertain whether or not it is consumed during in vivo complement activation. Attempts to demonstrate changes in C4BP plasma levels in systemic lupus erythematosus and essential mixed cryoglobulinaemia have failed, probably due to up-regulation of this protein during the inflammatory reaction. We have studied one patient with severe post-transfusion complement-mediated anaphylaxis (CMA), and 67 patients with hereditary C1 inhibitor deficiency (hereditary angioedema (HAE)). The first of these two conditions is characterized by the absence of systemic inflammatory reaction and the second by acute and chronic activation of the C' classical pathway. C4BP, C4BP-C4b complex, and soluble terminal C' complex (sC5b-9) were measured in the patients' plasmas by ELISA techniques and C3a and C4a by radioimmunoassays. In CMA, 15 min after the transfusion, there was a massive C' activation, with increases in C4a, C3a, sC5b-9, C4BP-C4b complexes and decreases in C4, C3 and C4BP. All parameters reverted to preinfusion values within 24 h. Depletion of C4 was correlated with that of C4BP. In patients with HAE, the median value of C4BP (83% range 54-165) was significantly lower (P < 0.0001) than in normal controls (99% range 70-159), with no difference between patients in remission or during acute attacks. C4BP-C4b complexes could not be detected in HAE patients. The results of this study indicate that C4BP is consumed in vivo during acute, and possibly during chronic activation of the C' classical pathway, and that this protein, after interaction with C4b, not longer circulates in plasma. (+info)

The promoter of the C1 inhibitor gene contains a polypurine.polypyrimidine segment that enhances transcriptional activity. (3/297)

The C1 inhibitor (C1INH) promoter is unusual in two respects: 1) It contains no TATA sequence, but instead contains a TdT-like initiator element (Inr) at nucleotides -3 to +5; 2) it contains a polypurine.polypyrimidine tract between nucleotides -17 and -45. Disruption of the Inr by the introduction of point mutations reduced promoter activity by 40%. A TATA element inserted at nucleotide -30 in the wild-type promoter and in promoter constructs containing the mutated Inr led to a 2-fold increase in basal promoter activity. Previous studies suggested that the potential hinged DNA-forming polypurine.polypyrimidine tract might be important in the regulation of C1INH promoter activity. The present studies indicate that this region is capable of such intramolecular triple helix formation. Disruption of the polypurine.polypyrimidine sequence by substitution of 5 of the 23 cytosine residues with adenine prevented triple helix formation. Site-directed mutagenesis experiments demonstrate that the regulation of promoter activity is independent of hinged DNA-forming capacity but requires an intact AC box (ACCCTNNNNNACCCT) or the overlapping PuF binding site (GGGTGGG). The C1INH gene also contains a number of potential regulatory elements, including an Sp-1 and an hepatocyte nuclear factor-1 binding site and a CAAT box. The role of these elements in regulation of the C1INH promoter was examined. Elimination of the hepatocyte nuclear factor-1 site at nucleotides -94 to -81 by truncation reduced the activity of the promoter by approximately 50%. Similarly, site-directed mutations that disrupt this site reduce promoter activity by 70%. (+info)

Adjuvant treatment of severe acute pancreatitis with C1 esterase inhibitor concentrate after haematopoietic stem cell transplantation. (4/297)

BACKGROUND: With an incidence of 4%, acute pancreatitis is a common complication of bone marrow or peripheral haematopoietic stem cell transplantation, which contributes significantly to morbidity and mortality in these patients. In most cases, the pathogenesis of acute pancreatitis cannot be attributed to a single pathogenetic factor, as treatment toxicity, acute graft versus host disease, infection, and cholestasis may all contribute. Acute pancreatitis is characterised by inflammation and activation of digestive proenzymes leading to autodigestive destruction of the pancreas and systemic activation of protease cascades including the complement system. AIM: To describe the effects of human C1 esterase inhibitor in two children, who developed severe acute pancreatitis with considerable complement activation after allogeneic haematopoietic stem cell transplantation. METHODS: Both children showed clinical features resembling those observed in capillary leakage syndrome. In both patients, treatment with C1 esterase inhibitor concentrate contributed to a rapid clinical stabilisation. CONCLUSIONS: These observations strongly support the proposed pathophysiological concept that early treatment with C1 esterase inhibitor interferes with the activation of the complement system in acute pancreatitis. Inhibition of complement activation prevents its adverse effects on vascular function and permeability, and thus stabilises intravascular fluid status and prevents multiorgan failure in acute pancreatitis. (+info)

C1q and C4b bind simultaneously to CR1 and additively support erythrocyte adhesion. (5/297)

Previously, we showed that soluble C1q bound specifically to CR1 on transfected cells. If the CR1-C1q interaction were to participate in immune complex clearance, then this interaction should support E adhesion. Using a tip plate adhesion assay, we found that immobilized C1q mediated adhesion of human E. E binding to C1q was specifically inhibited by polyclonal anti-CR1 Fab fragments. Intact C1 was not efficient as an adherence ligand until it was treated with EDTA or the C1 inhibitor to remove the C1r2C1s2 complex from C1, leaving C1q. Titration of C1q alone, C4b alone, and C1q + C4b indicated that the two complement ligands were additive in their ability to support CR1-mediated adhesion of E. Analysis of binding to immobilized CR1 using a BIAcore instrument documented that C1q, C4b, and C3b binding were independent events. Additionally, C1q-dependent binding of immune complexes and heat-aggregated IgG to E was documented. These experiments confirm that the immune adherence receptor in humans, CR1, is the single receptor for all of the opsonic ligands of complement, provide evidence for a single C1q binding site on LHR-D of CR1, and suggest that C1q may participate in immune clearance. (+info)

Complement activation in patients with systemic lupus erythematosus without nephritis. (6/297)

OBJECTIVE: To study the association between disease activity and complement activation prospectively in patients with systemic lupus erythematosus (SLE). PATIENTS AND METHODS: Twenty-one SLE patients were examined monthly for 1 yr. Disease activity, autoantibodies, conventional complement tests and the following complement activation products were investigated: C1rs-C1inh complexes, C4bc, Bb, C3a, C3bc, C5a and the terminal SC5b-9 complement complex (TCC). RESULTS: Modest variation in disease activity was noted. None of the patients had nephritis. Flare was observed at 27 visits. Four patients had anti-C1q antibodies in conjunction with modestly low C1q concentrations. The complement parameters were rather constant during the observation period. Slightly to moderately decreased C4 (0.05-0.10 g/l) was found in 10 patients and severely decreased C4 (<0.05 g/l) in seven patients. Decreased C4 was not associated with increased complement activation. Complement activation products were either normal or slightly elevated. TCC was the only activation product correlating significantly with score for disease activity at flare. None of the variables tested predicted flares. CONCLUSION: Complement tests are of limited importance in routine examination of SLE without nephritis, although TCC is suggested to be one of the most sensitive markers for disease activity. (+info)

Hepatitis C virus NS3 serine protease interacts with the serpin C1 inhibitor. (7/297)

Both NS3 protein (1007-1657) and its protease moiety (NS3p, 1027-1207) were able to interact in vitro with C1 Inhibitor (C1Inh) to give a 95-kDa Mr C1Inh cleavage product similar to that obtained upon proteolysis by complement protease C1s. High-Mr reaction products were also detected after incubation of C1Inh with NS3 but not with NS3p; they correspond to ester-bonded complexes from their hydroxylamine lability. Similar reactivity of NS3 was observed upon incubation with alpha2-antiplasmin. Serpin cleavage was prevented by treatment of NS3 with synthetic serine protease inhibitors. This interaction between viral NS3 and host serpins suggests that NS3 is likely to be controlled by infected cell protease inhibitors. (+info)

The Arthus reaction in rodents: species-specific requirement of complement. (8/297)

We induced reverse passive Arthus (RPA) reactions in the skin of rodents and found that the contribution of complement to immune complex-mediated inflammation is species specific. Complement was found to be necessary in rats and guinea pigs but not in C57BL/6J mice. In rats, within 4 h after initiation of an RPA reaction, serum alternative pathway hemolytic titers decreased significantly below basal levels, whereas classical pathway titers were unchanged. Thus the dermal reaction proceeds coincident with systemic activation of complement. The serine protease inhibitor BCX 1470, which blocks the esterolytic and hemolytic activities of the complement enzymes Cls and factor D in vitro, also blocked development of RPA-induced edema in the rat. These data support the proposal that complement-mediated processes are of major importance in the Arthus reaction in rats and guinea pigs, and suggest that BCX 1470 will be useful as an anti-inflammatory agent in diseases where complement activation is known to be detrimental. (+info)

... complement c1 inactivator proteins MeSH D12.776.124.486.274.920.250.500 - complement c1 inhibitor protein MeSH D12.776.124.486. ... complement c3b inactivator proteins MeSH D12.776.124.486.274.920.325.200 - complement factor h MeSH D12.776.124.486.274.920. ... complement factor b MeSH D12.776.124.486.274.920 - complement inactivator proteins MeSH D12.776.124.486.274.920.124 - antigens ... complement c1 MeSH D12.776.124.486.274.050.270 - complement c1q MeSH D12.776.124.486.274.050.280 - complement c1r MeSH D12.776. ...

https://en.wikipedia.org/wiki/List_of_MeSH_codes_(D12.776.124)

C4b-binding protein inhibits the haemolytic function of cell-bound C4b. C4b-binding protein and C3b inactivator control the C3 ... In the classical pathway, this is by sequential proteolytic activation of proteins within the C1 complex (C1q, C1r, C1s) in ... complement receptor 1 (CR1), C4b-binding protein and Factor H. Convertase assembly is suppressed by the proteolytic cleavage of ... "Modulation of the Classical Pathway C3 Convertase by Plasma Proteins C4 Binding Protein and C3b Inactivator". Proc Natl Acad ...

https://en.wikipedia.org/wiki/C3-convertase

... arising as a result of alternate complement activation or mutations in the complement-regulating proteins. C3NeF is commonly ... The binding of NFa to C3b,Bb stabilizes the complex, preventing degradation by its normal inactivators, resulting in complement ... The classic pathway is activated by the interaction of C1 with an antigen-antibody complex. This interaction results in the ... Patterns of complement activation in idiopathic membranoproliferative glomerulonephritis, types I, II, and III. Am J Kidney Dis ...

https://emedicine.staging.medscape.com/article/240056-overview

Other inhibitors, such as antithrombin, alpha1-antitrypsin, and C1 inactivator of complement, have in vitro antiplasmin ... In addition, other noncoagulation proteins, such as complement, growth hormone, corticotropin, and glucagon, are substrates for ... Several other proteins are also involved in the complex process of regulation of fibrinolysis in vivo. Physiologically, the end ... Alpha2-plasmin inhibitor (alpha2-PI), also known as alpha2-antiplasmin, is a protein that is primarily synthesized by the liver ...

https://www.staging.medscape.com/answers/198336-172282/what-is-alpha-2-plasmin-inhibitor-deficiency

https://www.staging.medscape.com/answers/198336-172284/what-is-the-prevalence-of-alpha-2-plasmin-inhibitor-deficiency-in-the-us

Complement component 3 inactivator deficiency, see Complement factor I deficiency. *Complement component 8 deficiency ... C1 inhibitor deficiency, see Hereditary angioedema. *C2 deficiency, see Complement component 2 deficiency ... Congenital lysinuria, see Lysinuric protein intolerance. *Congenital megacolon, see Hirschsprung disease. *Congenital ... C1 esterase inhibitor deficiency, see Hereditary angioedema. * ... C3 inactivator deficiency, see Complement factor I deficiency. ...

https://medlineplus.gov/genetics/condition-c/

https://www.medscape.com/answers/198336-172287/what-are-the-sexual-predilections-of-alpha-2-plasmin-inhibitor-deficiency

https://www.staging.medscape.com/answers/198336-172287/what-are-the-sexual-predilections-of-alpha-2-plasmin-inhibitor-deficiency

... a plasma protein that enhances inactivation of the complement cascade by binding to C4b and promoting its proteolytic ... that is a potent inactivator of bradykinin and of the activated complement components, C3a and C5a.94,95,96 ... 113-11) are composed of two polypeptide chains, one bearing the A1-A2 domains and the other bearing the A3-C1-C2 domains. ... Membrane-bound protein C, protein S, thrombomodulin (TM) and endothelial cell protein C receptor (EPCR). Each protein is a ...

https://hemonc.mhmedical.com/content.aspx?bookid=1581§ionid=108080365

Complement C3. Complement C4. Malaria Test/ MP - QBC Method. Cell Count - Synovial fluid. Progesterone Level. Protein ... C1 Esterase Inhibitor - Quantification (C1 Inactivator - Quantitative). X-RAY BOTH KNEE SKYLINE VIEW. 24hrs Urine - Osmolality ... APCR - Activated Protein C Resistance. Protein C. c protein. Protein S. s protein. FOOD ALLERGY PANEL FOR NON VEGETARIAN. SS - ... Glucose Protein- Cerebrospinal fluid. Glucose & Protein- Subgaleal Fluid. Subgalial fluid. Glucose & Protein- Synovial fluid. ...

https://bloodoxy.com/view/cart

https://bloodoxy.com/view/product/Lab-Tests-Health-Checkup/Lab-Test/CYSTATIN-C/BOBT00303?id=303

Complement C1 Inactivator Proteins [D12.644.861.140] + Complement C1 Inactivator Proteins + * HSP47 Heat Shock Proteins [ ... Antithrombin Proteins - Preferred Concept UI. M0001510. Scope note. An endogenous family of proteins belonging to the serpin ... An endogenous family of proteins belonging to the serpin superfamily that neutralizes the action of thrombin. Six naturally ... Complement C1 Inactivator Proteins [D12.776.872.140] + Complement C1 Inactivator Proteins + * HSP47 Heat Shock Proteins [ ...

https://decs.bvsalud.org/en/ths/resource/?id=54060

https://bloodoxy.com/view/product/Lab-Tests-Health-Checkup/Lab-Test/Cholesterol-Total/BOBT00103?id=103

https://bloodoxy.com/view/product/Lab-Tests-Health-Checkup/Radiology/DOPPLER-ILIAC-VESSELS-AND-IVC/BOBT00759?id=759

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https://bloodoxy.com/view/product/Lab-Tests-Health-Checkup/Lab-Test/ANA-PROFILE/BOBT00175?id=175

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C1 esterase inhibitor * C1 esterase inhibitor / activity * C1-Inactivator , Laboratory research * C1Q- complement , Laboratory ... Coronavirus (SARS-Cov-2) Spike (S) Protein , Antibodies * Coronavirus (SARS-Cov-2) Spike (S) Protein , Antibodies & Home ... Profile of prophylactic screening & antibodies to SARS-Cov-2 spike (S) protein ...

https://synevo.ge/en/content/

https://bloodoxy.com/view/product/Lab-Tests-Health-Checkup/Lab-Test/SARS-CoV-2-COVID-19-ANTIGEN-RAPID/BOBT00688?id=688

Activation of the complement system underlies one of the main effector pathways contributing to antibody-mediated immunity. ... Cell-Surface-Based Inactivators of Complement. CD59 and CD55 are cell-surface molecules anchored by ... C1 inhibitor is a serine-protease inhibitor that inactivates the serine esterases gener-ated by complement activation (C1r and ... These two proteins inactivate any C3 con-vertase molecules deposited on cell sur-faces. Somatic mutation of the enzyme (PIGA: ...

https://www.brainkart.com/article/Inherited-Deficiency-of-the-Complement-System_17694/

Complement C1 Inactivator Proteins [D12.776.124.486.274.920.250] * Complement C3 Nephritic Factor [D12.776.124.486.274.920.287] ... Complement 4b Binding Protein Complement C3b-C4b Inactivator Cofactor Complement Component 4b-Binding Protein Pharm Action. ... Complement C3b Inactivator Proteins [D12.776.124.486.274.920.325] * Complement C4b-Binding Protein [D12.776.124.486.274.920.662 ... Complement System Proteins [D12.776.124.486.274] * Complement Inactivator Proteins [D12.776.124.486.274.920] * Membrane ...

https://meshb-prev.nlm.nih.gov/record/ui?ui=D050716

Antithrombin Proteins [D12.644.861.060] * Complement C1 Inactivator Proteins [D12.644.861.140] * HSP47 Heat-Shock Proteins [ ... Amino Acids, Peptides, and Proteins [D12] * Proteins [D12.776] * Blood Proteins [D12.776.124] * Serum Globulins [D12.776. ... Amino Acids, Peptides, and Proteins [D12] * Proteins [D12.776] * Glycoproteins [D12.776.395] * Activins [D12.776.395.022] ... Amino Acids, Peptides, and Proteins [D12] * Proteins [D12.776] * Globulins [D12.776.377] * Serum Globulins [D12.776.377.715] * ...

https://meshb.nlm.nih.gov/record/ui?ui=D000979

Tricorn protease C1 domain 14 Domain IPR005183:Domain of unknown function DUF305 14 Active_site IPR008266:Tyrosine-protein ... Alpha-macroglobulin complement component 1 Family IPR010767:Campylobacter phage CGC-2007, Cje0229 1 Domain IPR006869:Domain of ... DnaA regulatory inactivator Hda 6 Domain IPR003696:Carbamoyltransferase 6 Domain IPR025691:GspL periplasmic domain 6 Domain ... SD-repeat containing protein, B domain 6 Domain IPR027398:SecD export protein N-terminal TM domain 6 Family IPR021953:Protein ...

https://www.cbrc.kaust.edu.sa/aamg/1502720332816_CamiLowProdigalV2_40.0_intikhab/WWWAAMG/visuals/CamiLowProdigalV2_AAMG_DOMAINS_data_summary.txt

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... protein a based, gravity flow ) 7948 igg purification kit ( protein a based, spin column ) 7949 igg purification kit ( protein ... inactivator broth ) 1179 cae ( citrate azide enterococcus ) agar base 1180 ttc solution 1% ( 10 ml per vial ) 1181 cae ( ... c1 ) 7570 prewash solution concentrate ( pw ) 7571 wash solution concentrate ( ws ) 7572 lysis solution ( al ) 7573 ... from yeast 10683 guar gum powder 10684 guar gum powder ip 10685 guinea pig complement 10686 gum acacia, powder 10687 gum ghatti ...

http://www.chhattisgarhtenders.net/SearchTender?Keyword=agarose gel

Complement C9 [D12.776.124.486.274.850] * Complement Factor B [D12.776.124.486.274.900] * Complement Inactivator Proteins [ ... Complement Activating Enzymes [D12.776.124.486.274.045] * Complement C1 [D12.776.124.486.274.050] * Complement C2 [D12.776. ... C5 Complement Complement 5 Complement C5, Precursor Complement Component 5 Precursor C5 Pro-C5 Pro-complement 5 Pharm Action. ... Proteins [D12.776] * Blood Proteins [D12.776.124] * Immunoproteins [D12.776.124.486] * Complement System Proteins [D12.776. ...

https://meshb-prev.nlm.nih.gov/record/ui?ui=D003182

Complement C1s. Complement C1 Inhibitor Protein. Complement C1 Inactivator Proteins. Immunologic Factors. Physiological Effects ... Biological: Low-volume C1-esterase inhibitor. Biological: Higher-volume placebo. Experimental: Low-volume C1-esterase inhibitor ... Biological: Higher-volume C1-esterase inhibitor. Biological: Low-volume placebo. Experimental: Higher-volume C1-esterase ... Biological: Low-volume C1-esterase inhibitor Biological: Higher-volume C1-esterase inhibitor Biological: Low-volume placebo ...

https://clinicaltrials.gov/ct2/show/NCT01912456

Complement C1 Inactivator Proteins Entry term(s). Complement C1 Inactivating Proteins Complement C1 Inhibiting Proteins ... Complement C1 Inactivating Proteins. Complement C1 Inhibiting Proteins. Complement C1 Inhibitor Proteins. Complement C1r ... Complement C1 Inhibitor Proteins Complement Component 1 Inactivator Proteins Complement C1s Esterase Inhibitor Proteins - ... Complement C1 Inactivator Proteins - Preferred Concept UI. M0004926. Scope note. Serum proteins that inhibit, antagonize, or ...

https://decs.bvsalud.org/en/ths/resource/?id=27972

... On-line free medical diagnosis assistant. Ranked list of possible diseases from either several symptoms or ... Complement C1 Inactivator Proteins. 1. + 31. Biopterin. 1. + 32. Transferrin. 1. + 33. Folic Acid. 1. + ...

https://lookfordiagnosis.com/results.php?symptoms=protein+deficiency&lang=1&parent=%2F&mode=F&therapy_ap=1

Learn about the three pathways lead to complement activation and some of their key inhibitors. ... Complement inhibitors include the plasma serine proteinase inhibitor serpin (C1 inactivator). The plasma proteins, Factor I and ... C1 is the first molecule in the classical complement cascade and comprises C1q and two molecules of C1r and C1s respectively. ... C1 is the first molecule in the classical complement cascade and comprises C1q and two molecules of C1r and C1s respectively. ...

https://www.abcam.com/pathways/complement-cascade-and-its-inhibitors

Complement C1 Inactivator Proteins D12.644.822.750.140 D12.776.645.750.140 Complement C1 Inhibitor Protein D12.644.822.750. ... Son of Sevenless Protein, Drosophila D12.776.402.300.700.700.600 Son of Sevenless Proteins D12.776.402.300.700.700 SOS1 Protein ... Proto-Oncogene Protein c-fli-1 D12.776.930.635.400 D12.776.930.720.400 Proto-Oncogene Proteins c-bcl-6 D12.776.930.633 D12.776. ... Proto-Oncogene Protein c-ets-1 D12.776.930.635.100 D12.776.930.720.100 Proto-Oncogene Protein c-ets-2 D12.776.930.635.200 ...

https://decs2018.bvsalud.org/I/mnchg2016.txt

https://decs2019.bvsalud.org/E/mnchg2016.txt

https://decs2016.bvsalud.org/E/mnchg2016.txt

https://decs2020.bvsalud.org/E/mnchg2016.txt

https://decs2017.bvsalud.org/E/mnchg2016.txt

https://meshb.nlm.nih.gov/record/ui?ui=D000979

Complement. Complement System Proteins. Complement 1. Complement C1. Complement 1 Inactivators. Complement C1 Inactivator ... Complement Inactivators. Complement Inactivator Proteins. DNA-Binding Protein, Cyclic AMP-Responsive. Cyclic AMP Response ... Proto-Oncogene Protein c-met. Proto-Oncogene Proteins c-met. Proto-Oncogene Protein p21(ras). Proto-Oncogene Proteins p21(ras) ... Complement 1s. Complement C1s. Glyceraldehyde 3 Phosphate-Dehydrogenase (NADP+)(Phosphorylating). Glyceraldehyde-3-Phosphate ...

https://decs.bvs.br/I/rep_i2006.htm

https://decs2012.bvsalud.org/I/rep_i2006.htm

https://decs2008.bvsalud.org/I/rep_i2006.htm

https://decs2010.bvsalud.org/I/rep_i2006.htm

https://decs2014.bvsalud.org/I/rep_i2006.htm

https://decs2013.bvsalud.org/I/rep_i2006.htm

https://decs2011.bvsalud.org/I/rep_i2006.htm

https://decs2015.bvsalud.org/I/rep_i2006.htm

https://meshb-prev.nlm.nih.gov/record/ui?ui=D050716

Complement C1 Inactivator Proteins. *HSP47 Heat-Shock Proteins. *Ovalbumin. *Plasminogen Inactivators. *Thyroxine-Binding ... A thyroid hormone transport protein found in serum. It binds about 75% of circulating THYROXINE and 70% of circulating ... Thyroid hormones, their carrier proteins, and thyroid antibodies in the pleural effusion of two patients with graves disease- ...

https://connects.catalyst.harvard.edu/Profiles/display/Concept/Thyroxine-Binding%20Globulin

https://www.staging.medscape.com/answers/198336-172287/what-are-the-sexual-predilections-of-alpha-2-plasmin-inhibitor-deficiency

C1 A Ca2+-dependent union of three components: Clq (MW 400 000), a curious protein with six valencies for Ig linked by collagen ... and in some cases there are special inactivators (represented here by scissors). Nevertheless, excessive complement activation ... Complement. Fifteen or more serum components constitute the complement system, the sequential activation and assembly into ... Complement inhibitors. In order to prevent over-activation of the complement cascade, there are numerous inhibitory mechanisms ...

https://www.pediagenosis.com/2018/07/complement.html

https://medlineplus.gov/genetics/condition-c/

Complement Inactivator Proteins [D12.776.124.486.274.920] Complement Inactivator Proteins * Complement Membrane Attack Complex ... Complement Complement Protein Complement Proteins Protein, Complement Proteins, Complement Proteins, Complement System ... Complement. Complement Protein. Complement Proteins. Complement, Hemolytic. Hemolytic Complement. Protein, Complement. Proteins ... Complement Activating Enzymes [D12.776.124.486.274.045] Complement Activating Enzymes * Complement C1 [D12.776.124.486.274.050 ...

https://decs.bvsalud.org/en/ths/resource/?id=22132&filter=ths_exact_term&q=HEMOLYTIC+COMPLEMENT

proteins involved. KEGG pathway ID. Description. 64.71. map04610. Complement and coagulation cascades. ... Plasma protease C1 inhibitor (P05155) (SMART). OMIM:106100: Angioedema, hereditary. Alpha-1-antitrypsin (P01009) (SMART). OMIM: ... Crystal Structure of a novel small molecule inactivator bound to plasminogen activator inhibitor-1. ... Click on the protein counts, or double click on taxonomic names to display all proteins containing SERPIN domain in the ...

https://smart.embl.de/smart/do_annotation.pl?DOMAIN=SERPIN&START=13&END=388&E_VALUE=4.33e-143&TYPE=SMART&BLAST=LDVFKELSSNNVGENIFFSPLTTFYALSMLLLGTRGKSAEQMEKVLHYDSFSGVLKAKTKNSSECSQVGVMHPDFRALISHINQQNSLSVANRIYGTRSISFHKQYVRCCEKLYQAKLQTVDFELSTEETRKSINAWVKNKTNGKITNLFAKGTIDPSSVMVLVSAIYFKGQWQNKFQKRETVKAPFHMGVGKSAVVNMMYQTGTFKLAIIKEPEMQVLELPYANNKLRMIILLPVGTASVSQIEKHLNVKMLREWTNPSNMVEREVDVHIPKFSLSVKYDLNTLLKSLGMRDIFNVANADLSGMSPDKGLYLSKVVHKSYVDVNEEGTEAAAATGESISVKRLPVTVQFTANCPFLFFIWDESGNILFAGKFASP

C1 INACTIVATOR 3 PLACAS. Cod.: TBSIDR RN020.3. COMPLEMENT C1q 3 PLACAS. Cod.: TBSIDR RN022.3. COMPLEMENT C2 3 PLACAS. Cod.: ... COMPLEMENT C3 3 PLACAS. Cod.: TBSIDR RN025.3. COMPLEMENT C4 3 PLACAS. Cod.: TBSIDR RN027.3. COMPLEMENT C5 3 PLACAS. Cod.: ... Introducing Optilite, the future of special protein analysis. + INFO + INFO #linea1, #linea4, #linea7, #linea10, #linea14, # ... C1 INACT KIT SPA X 50. Cod.: TBSSPARNK023.S. C3c KIT SPA X 100. Cod.: TBSSPARNK025.S. C4 KIT SPA X 100. Cod.: TBSSPARNK032.S. ...

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protein C, inactivator of coagulation factors Va and VIIIa Previous and unofficial names. anticoagulant protein C , ... complement C1r Previous and unofficial names. complement C1r-A subcomponent , complement component 1, r subcomponent A , ... C1sb , complement component C1SB , r-gsp , Gm5077 , predicted gene 5077 , complement component 1 , complement component 1, s ... This target is also described as a ligand entry: protein C; protein C; protein C. ...

https://www.guidetopharmacology.org/GRAC/FamilyDisplayForward?familyId=751

https://www.cbrc.kaust.edu.sa/aamg/1502720332816_CamiLowProdigalV2_40.0_intikhab/WWWAAMG/visuals/CamiLowProdigalV2_AAMG_DOMAINS_data_summary.txt

Learn about the three pathways lead to complement activation and some of their key inhibitors. (abcam.com)
Alpha2-PI belongs to the serpin family of inhibitors, is synthesized by the liver, and is present in plasma as a single-chain protein at approximately half the concentration of plasminogen. (medscape.com)
Because the deduced amino acid sequence qualified the protein as a novel member of the serpin family of serine protease inhibitors, we called it neuroserpin. (embl.de)

Serum proteins that inhibit, antagonize, or inactivate COMPLEMENT C1 or its subunits. (bvsalud.org)
A thyroid hormone transport protein found in serum. (harvard.edu)
Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX . (bvsalud.org)

Two 'C3b inactivator' enzymes rapidly inactivate C3b, releasing the fragment C3c and leaving membrane bound C3d. (pediagenosis.com)

It binds as a cofactor to COMPLEMENT FACTOR I which then hydrolyzes the COMPLEMENT C4B in the CLASSICAL PATHWAY C3 CONVERTASE (C4bC2a). (nih.gov)
The essential feature is the requirement for a specific antigen-antibody interaction, leading via components C1, C2 and C4 to the formation of a 'convertase' which splits C3. (pediagenosis.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)

the classical pathway initiated by antibodies bound to the surface of foreign bodies and the alternative and lectin pathways that provide an antibody-independent mechanism for complement activation, induced by the presence of bacteria and other micro-organisms. (abcam.com)
Thyroid hormones, their carrier proteins, and thyroid antibodies in the pleural effusion of two patients with graves' disease-induced thyrotoxicosis. (harvard.edu)

Mannan-binding lectin (MBL) and MBL-associated serine proteases (MASPs) are involved in the initial step of the lectin pathway of complement activation. (abcam.com)
Following these cleavage events, complement pathway activation continues as in the classical pathway. (abcam.com)
and LECTIN COMPLEMENT PATHWAY ). (bvsalud.org)

The aim of this study is to assess the efficacy of C1-esterase inhibitor in preventing hereditary angioedema attacks when it is administered under the skin of subjects with hereditary angioedema. (clinicaltrials.gov)
The safety of C1-esterase inhibitor will also be assessed. (clinicaltrials.gov)
In the treatment phase, subjects will be randomized to one of four arms consisting of treatment with low- or higher-volume C1-esterase inhibitor in one treatment period and treatment with low- or higher-volume placebo in the other treatment period. (clinicaltrials.gov)
A higher-volume dose of placebo will be administered subcutaneously twice a week for up to 16 weeks, then a low-volume dose of C1-esterase inhibitor will be administered subcutaneously twice a week for up to 16 weeks. (clinicaltrials.gov)

The complement system is a heat-labile component of blood that confers bactericidal properties. (abcam.com)

Alpha2-plasmin inhibitor (alpha2-PI), also known as alpha2-antiplasmin, is a protein that is primarily synthesized by the liver and is present in plasma and platelets. (medscape.com)

It is the principal plasmin inactivator in blood, rapidly forming a very stable complex with plasmin. (nih.gov)
Plasminogen activators convert the zymogen plasminogen to the active enzyme plasmin, which then hydrolyzes susceptible arginine and lysine bonds in a variety of proteins. (medscape.com)
Plasmin not only degrades fibrin, which is its principal substrate, but it also degrades fibrinogen, factors V and VIII, proteins involved in platelet adhesion (glycoprotein I and vWF), platelet aggregation (glycoprotein IIb/IIIa) and maintenance of platelet aggregates (thrombospondin, fibronectin, histidine-rich glycoprotein), and the attachment of platelets and fibrin to the endothelial surface. (medscape.com)

C1 is the first molecule in the classical complement cascade and comprises C1q and two molecules of C1r and C1s respectively. (abcam.com)

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)

Activation of complement can be started either via adaptive or innate immune recognition. (pediagenosis.com)
Activation is usually limited to the immediate vicinity by the very short life of the active products, and in some cases there are special inactivators (represented here by scissors). (pediagenosis.com)
Nevertheless, excessive complement activation can cause unpleasant side-effects (see Fig. 36). (pediagenosis.com)

Proteínas séricas que inhiben, antagonizan o inactivan el COMPLEMENTO C1 o sus subunidades. (bvsalud.org)
Glicoproteínas séricas que participan en el mecanismo de defensa del hospedador de la ACTIVACIÓN DEL COMPLEMENTO que crea el COMPLEJO DE ATAQUE A LA MEMBRANA DEL COMPLEMENTO. (bvsalud.org)

The functions of complement include the attraction of inflammatory cells, opsonization to promote phagocytosis, immune complex clearance and direct microbial killing through the formation of the membrane attack complex (MAC). (abcam.com)

Unless otherwise stated all data on this page refer to the human proteins. (guidetopharmacology.org)

One of the cysteine conjugates was found in a digestion mixture containing hepatic proteins from animals administered TOL. (bvsalud.org)

Click on the protein counts, or double click on taxonomic names to display all proteins containing SERPIN domain in the selected taxonomic class. (embl.de)

C5 plays a central role in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. (labome.org)
An important soluble regulator of the alternative pathway of complement activation ( COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). (lookformedical.com)
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 ). (lookformedical.com)
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. (lookformedical.com)
This enzyme cleaves the COMPLEMENT C3B-bound COMPLEMENT FACTOR B to form C3bBb which is ALTERNATIVE PATHWAY C3 CONVERTASE. (lookformedical.com)
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. (umassmed.edu)

The development of atypical hemolytic uremic syndrome depends on complement C5. (labome.org)

International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency. (nih.gov)

The major fragment C5b binds to the membrane initiating the spontaneous assembly of the late complement components, C5-C9, into the MEMBRANE ATTACK COMPLEX. (labome.org)

Pangburn M, Rawal N. Structure and function of complement C5 convertase enzymes. (labome.org)
The larger fragment generated from the cleavage of COMPLEMENT C3 by C3 CONVERTASE. (lookformedical.com)
C3 can be cleaved into COMPLEMENT C3A and COMPLEMENT C3B , spontaneously at low level or by C3 CONVERTASE at high level. (lookformedical.com)
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 . (lookformedical.com)
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). (lookformedical.com)

Factors initiating complement activation include ANTIGEN-ANTIBODY COMPLEXES, microbial ANTIGENS, or cell surface POLYSACCHARIDES. (lookformedical.com)
Complement activation initiated by the interaction of microbial ANTIGENS with COMPLEMENT C3B . (lookformedical.com)

C1 esterase inhibitor [human] is a serine proteinase inhibitor derived from human plasma that is used in treating hereditary angioedema. (nih.gov)
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. (lookformedical.com)

Various international consensus panels state that human plasma-derived C1 esterase inhibitor is considered to be the therapy of choice for both treatment and prophylaxis of maternal hereditary angioedema during lactation. (nih.gov)
Three patients with hereditary angioedema received C1 esterase inhibitor concentrate on 12 occasions to relieve abdominal edematous episodes during breastfeeding. (nih.gov)
In a case series spanning 12 years, 21 mothers with hereditary angioedema breastfed their infants for a median duration of 4.8 months (range 1 to 34 months) while receiving C1 esterase inhibitor concentrate as needed. (nih.gov)
A pregnant woman with severe hereditary angioedema required 500 IU of C1 inhibitor concentrate every 2 days to maintain her pregnancy. (nih.gov)
A woman with hereditary angioedema received C1 inhibitor concentrate 1000 units every week during pregnancy. (nih.gov)
A woman with hereditary angioedema used subcutaneous C1 esterase inhibitor concentrate 1500 units prophylactically twice a week during pregnancy and postpartum. (nih.gov)
A 29-year-old woman with a long history of hereditary angioedema received subcutaneous C1-esterase therapy during pregnancy and postpartum in a dose of "2 x 3000 IU/week" (presumably 3000 IU twice weekly). (nih.gov)
The natural history of hereditary angioedema and the impact of treatment with human C1-inhibitor concentrate during pregnancy: A long-term survey. (nih.gov)

A ubiquitously expressed complement receptor that binds COMPLEMENT C3B and COMPLEMENT C4B and serves as a cofactor for their inactivation. (childrensmercy.org)

Plasmin not only degrades fibrin, which is its principal substrate, but it also degrades fibrinogen, factors V and VIII, proteins involved in platelet adhesion (glycoprotein I and vWF), platelet aggregation (glycoprotein IIb/IIIa) and maintenance of platelet aggregates (thrombospondin, fibronectin, histidine-rich glycoprotein), and the attachment of platelets and fibrin to the endothelial surface. (medscape.com)

Thus, after processing sufficient C3 at the surface of a microorganism, the enzymes switch to processing C5, which initiates the formation of the cytolytic membrane attack complex of complement. (labome.org)

17. Immunoglobulin, complement, and immune complex levels during a migraine attack. (nih.gov)

Requisite role for complement C5 and the C5a receptor in granulomatous response to mycobacterial glycolipid trehalose 6,6'-dimycolate. (labome.org)

Gulati S, Agarwal S, Vasudhev S, Rice PA, Ram S. Properdin is critical for antibody-dependent bactericidal activity against Neisseria gonorrhoeae that recruit C4b-binding protein. (umassmed.edu)

Measurement of complement components in systemic lupus erythematosus by radial immunodiffusion. (umassmed.edu)

Plasminogen activators convert the zymogen plasminogen to the active enzyme plasmin, which then hydrolyzes susceptible arginine and lysine bonds in a variety of proteins. (medscape.com)

These data offer exciting prospects for targeted treatment of complement-mediated diseases without the detrimental inhibition of the opsonic roles of complement. (labome.org)

20. [Activity of total complement and concentration of its components C1q, C3, C4 and C1-inactivator in cancer]. (nih.gov)

Novel approach to control and regulate the classical complement pathway : highly specific inhibition of C1q globular head binding to human IgG using an engineered single chain antibody variable fragment : recruiting C1q to HER2neu positive cells. (musc.edu)

1. Stage-related correlations between immunoglobulins and complement components in preoperative sera from patients with gastric carcinoma. (nih.gov)
Profile of immunoglobulins, complement components and C-reactive protein in sera of leprosy patients and healthy controls. (nih.gov)

Unless otherwise stated all data on this page refer to the human proteins. (guidetopharmacology.org)

This graph shows the total number of publications written about "Complement C7" by people in this website by year, and whether "Complement C7" was a major or minor topic of these publications. (jefferson.edu)

Oparil S, Low J, Ehrlich EN, Lindheimer MD. The renin-angiotensin system in mother and fetus at cesarean section: a preliminary communication. (uchicago.edu)
13. [Immunoglobulins and the complement system in colorectal cancer]. (nih.gov)

Complement factor h. (lookformedical.com)
Bb, a serine protease, is generated when factor B is cleaved by COMPLEMENT FACTOR D into Ba and Bb. (lookformedical.com)
They include COMPLEMENT FACTOR H and COMPLEMENT FACTOR I (C3b/C4b inactivator). (lookformedical.com)

Breastmilk levels of C1 esterase inhibitor have not been measured after exogenous administration in humans. (nih.gov)

Alpha2-plasmin inhibitor (alpha2-PI), also known as alpha2-antiplasmin, is a protein that is primarily synthesized by the liver and is present in plasma and platelets. (medscape.com)

Taxonomic distribution of proteins containing SERPIN domain. (embl.de)
The complete taxonomic breakdown of all proteins with SERPIN domain is also avaliable . (embl.de)

There are 21666 SERPIN domains in 21322 proteins in SMART's nrdb database. (embl.de)

Anatomy13

Organisms10

Diseases12

Chemicals and Drugs141

Analytical, Diagnostic and Therapeutic Techniques and Equipment25

Phenomena and Processes52

Disciplines and Occupations2

Information Science3

Health Care2

Familial anglo-oedema--a particularly severe form. (1/297)

Consumption of C4b-binding protein (C4BP) during in vivo activation of the classical complement pathway. (2/297)

The promoter of the C1 inhibitor gene contains a polypurine.polypyrimidine segment that enhances transcriptional activity. (3/297)

Adjuvant treatment of severe acute pancreatitis with C1 esterase inhibitor concentrate after haematopoietic stem cell transplantation. (4/297)

C1q and C4b bind simultaneously to CR1 and additively support erythrocyte adhesion. (5/297)

Complement activation in patients with systemic lupus erythematosus without nephritis. (6/297)

Hepatitis C virus NS3 serine protease interacts with the serpin C1 inhibitor. (7/297)

The Arthus reaction in rodents: species-specific requirement of complement. (8/297)

Complement C3

Complement C1 Inactivator Proteins

Complement C3b Inactivator Proteins

Complement C4

Complement Inactivator Proteins

Complement C4a

Complement C3a

Complement Activation

Complement C1q

Complement C5

Complement C5a

Complement C4b

Complement C3b

Complement System Proteins

Complement C2

Complement C6

Complement C3c

Complement C3d

Complement C9

Receptors, Complement

Complement C1s

Complement C3-C5 Convertases

Complement Membrane Attack Complex

Complement C1r

Complement Factor B

Complement Pathway, Alternative

Complement C7

Complement Pathway, Classical

Complement C8

Complement C1

Complement Factor H

Anaphylatoxins

Complement Activating Enzymes

Properdin

Receptors, Complement 3b

Complement C5b

Complement C2a

Receptor, Anaphylatoxin C5a

Complement Inactivating Agents

Complement Hemolytic Activity Assay

Receptors, Complement 3d

Hemolysis

Complement Fixation Tests

Complement Factor D

Antigen-Antibody Complex

Complement Factor I

Complement C4b-Binding Protein

Antigens, CD55

Complement C3-C5 Convertases, Classical Pathway

Complement C2b

Blood Proteins

Antigens, CD59

Lysine Carboxypeptidase

Beta-Globulins

Molecular Sequence Data

Kinetics

Immunoelectrophoresis

Cytochrome P-450 CYP2B1

Phagocytosis

Cobra Venoms

Steroid 21-Hydroxylase

Chloromercurinitrophenols

Complement C3-C5 Convertases, Alternative Pathway

Amino Acid Sequence

Angioedema

Complement C1 Inhibitor Protein

Immunoglobulin G

Complement C3 Convertase, Alternative Pathway

Kaolin

Complement C5 Convertase, Classical Pathway

Serum Globulins

Neutrophils

Complement C3 Convertase, Classical Pathway

Antigens, CD46

Protein Binding

Enzyme Inhibitors

Erythrocytes

Opsonin Proteins

Binding Sites

Lupus Erythematosus, Systemic