A glycoprotein that is central in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C3 can be cleaved into COMPLEMENT C3A and COMPLEMENT C3B, spontaneously at low level or by C3 CONVERTASE at high level. The smaller fragment C3a is an ANAPHYLATOXIN and mediator of local inflammatory process. The larger fragment C3b binds with C3 convertase to form C5 convertase.
A glycoprotein that is important in the activation of CLASSICAL COMPLEMENT PATHWAY. C4 is cleaved by the activated COMPLEMENT C1S into COMPLEMENT C4A and COMPLEMENT C4B.
The smaller fragment formed when complement C4 is cleaved by COMPLEMENT C1S. It is an anaphylatoxin that causes symptoms of immediate hypersensitivity (HYPERSENSITIVITY, IMMEDIATE) but its activity is weaker than that of COMPLEMENT C3A or COMPLEMENT C5A.
The smaller fragment generated from the cleavage of complement C3 by C3 CONVERTASE. C3a, a 77-amino acid peptide, is a mediator of local inflammatory process. It induces smooth MUSCLE CONTRACTION, and HISTAMINE RELEASE from MAST CELLS and LEUKOCYTES. C3a is considered an anaphylatoxin along with COMPLEMENT C4A; COMPLEMENT C5A; and COMPLEMENT C5A, DES-ARGININE.
A subcomponent of complement C1, composed of six copies of three polypeptide chains (A, B, and C), each encoded by a separate gene (C1QA; C1QB; C1QC). This complex is arranged in nine subunits (six disulfide-linked dimers of A and B, and three disulfide-linked homodimers of C). C1q has binding sites for antibodies (the heavy chain of IMMUNOGLOBULIN G or IMMUNOGLOBULIN M). The interaction of C1q and immunoglobulin activates the two proenzymes COMPLEMENT C1R and COMPLEMENT C1S, thus initiating the cascade of COMPLEMENT ACTIVATION via the CLASSICAL COMPLEMENT PATHWAY.
The minor fragment formed when C5 convertase cleaves C5 into C5a and COMPLEMENT C5B. C5a is a 74-amino-acid glycopeptide with a carboxy-terminal ARGININE that is crucial for its spasmogenic activity. Of all the complement-derived anaphylatoxins, C5a is the most potent in mediating immediate hypersensitivity (HYPERSENSITIVITY, IMMEDIATE), smooth MUSCLE CONTRACTION; HISTAMINE RELEASE; and migration of LEUKOCYTES to site of INFLAMMATION.
The sequential activation of serum COMPLEMENT PROTEINS to create the COMPLEMENT MEMBRANE ATTACK COMPLEX. Factors initiating complement activation include ANTIGEN-ANTIBODY COMPLEXES, microbial ANTIGENS, or cell surface POLYSACCHARIDES.
The large fragment formed when COMPLEMENT C4 is cleaved by COMPLEMENT C1S. The membrane-bound C4b binds COMPLEMENT C2A, a SERINE PROTEASE, to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).
C5 plays a central role in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C5 is cleaved by C5 CONVERTASE into COMPLEMENT C5A and COMPLEMENT C5B. The smaller fragment C5a is an ANAPHYLATOXIN and mediator of inflammatory process. The major fragment C5b binds to the membrane initiating the spontaneous assembly of the late complement components, C5-C9, into the MEMBRANE ATTACK COMPLEX.
The larger fragment generated from the cleavage of COMPLEMENT C3 by C3 CONVERTASE. It is a constituent of the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb), and COMPLEMENT C5 CONVERTASES in both the classical (C4b2a3b) and the alternative (C3bBb3b) pathway. C3b participates in IMMUNE ADHERENCE REACTION and enhances PHAGOCYTOSIS. It can be inactivated (iC3b) or cleaved by various proteases to yield fragments such as COMPLEMENT C3C; COMPLEMENT C3D; C3e; C3f; and C3g.
Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY).
A 105-kDa serum glycoprotein with significant homology to the other late complement components, C7-C9. It is a polypeptide chain cross-linked by 32 disulfide bonds. C6 is the next complement component to bind to the membrane-bound COMPLEMENT C5B in the assembly of MEMBRANE ATTACK COMPLEX. It is encoded by gene C6.
A 206-amino-acid fragment in the alpha chain (672-1663) of C3b. It is generated when C3b is inactivated (iC3b) and its alpha chain is cleaved by COMPLEMENT FACTOR I into C3c (749-954), and C3dg (955-1303) in the presence COMPLEMENT FACTOR H.
A 302-amino-acid fragment in the alpha chain (672-1663) of C3b. It is generated when C3b is inactivated (iC3b) and its alpha chain is cleaved by COMPLEMENT FACTOR I into C3c, and C3dg (955-1303) in the presence COMPLEMENT FACTOR H. Serum proteases further degrade C3dg into C3d (1002-1303) and C3g (955-1001).
A component of the CLASSICAL COMPLEMENT PATHWAY. C2 is cleaved by activated COMPLEMENT C1S into COMPLEMENT C2B and COMPLEMENT C2A. C2a, the COOH-terminal fragment containing a SERINE PROTEASE, combines with COMPLEMENT C4B to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).
A 63-kDa serum glycoprotein encoded by gene C9. Monomeric C9 (mC9) binds the C5b-8 complex to form C5b-9 which catalyzes the polymerization of C9 forming C5b-p9 (MEMBRANE ATTACK COMPLEX) and transmembrane channels leading to lysis of the target cell. Patients with C9 deficiency suffer from recurrent bacterial infections.
Molecules on the surface of some B-lymphocytes and macrophages, that recognize and combine with the C3b, C3d, C1q, and C4b components of complement.
A 77-kDa subcomponent of complement C1, encoded by gene C1S, is a SERINE PROTEASE existing as a proenzyme (homodimer) in the intact complement C1 complex. Upon the binding of COMPLEMENT C1Q to antibodies, the activated COMPLEMENT C1R cleaves C1s into two chains, A (heavy) and B (light, the serine protease), linked by disulfide bonds yielding the active C1s. The activated C1s, in turn, cleaves COMPLEMENT C2 and COMPLEMENT C4 to form C4b2a (CLASSICAL C3 CONVERTASE).
A product of COMPLEMENT ACTIVATION cascade, regardless of the pathways, that forms transmembrane channels causing disruption of the target CELL MEMBRANE and cell lysis. It is formed by the sequential assembly of terminal complement components (COMPLEMENT C5B; COMPLEMENT C6; COMPLEMENT C7; COMPLEMENT C8; and COMPLEMENT C9) into the target membrane. The resultant C5b-8-poly-C9 is the "membrane attack complex" or MAC.
A 80-kDa subcomponent of complement C1, existing as a SERINE PROTEASE proenzyme in the intact complement C1 complex. When COMPLEMENT C1Q is bound to antibodies, the changed tertiary structure causes autolytic activation of complement C1r which is cleaved into two chains, A (heavy) and B (light, the serine protease), connected by disulfide bonds. The activated C1r serine protease, in turn, activates COMPLEMENT C1S proenzyme by cleaving the Arg426-Ile427 bond. No fragment is released when either C1r or C1s is cleaved.
Serum proteins that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host. The complement system is tightly regulated by inactivators that accelerate the decay of intermediates and certain cell surface receptors.
A 93-kDa serum glycoprotein encoded by C7 gene. It is a polypeptide chain with 28 disulfide bridges. In the formation of MEMBRANE ATTACK COMPLEX; C7 is the next component to bind the C5b-6 complex forming a trimolecular complex C5b-7 which is lipophilic, resembles an integral membrane protein, and serves as an anchor for the late complement components, C8 and C9.
Serine proteases that cleave COMPLEMENT C3 into COMPLEMENT C3A and COMPLEMENT C3B, or cleave COMPLEMENT C5 into COMPLEMENT C5A and COMPLEMENT C5B. These include the different forms of C3/C5 convertases in the classical and the alternative pathways of COMPLEMENT ACTIVATION. Both cleavages take place at the C-terminal of an ARGININE residue.
A glycine-rich, heat-labile serum glycoprotein that contains a component of the C3 CONVERTASE ALTERNATE PATHWAY (C3bBb). Bb, a serine protease, is generated when factor B is cleaved by COMPLEMENT FACTOR D into Ba and Bb.
Complement activation initiated by the interaction of microbial ANTIGENS with COMPLEMENT C3B. When COMPLEMENT FACTOR B binds to the membrane-bound C3b, COMPLEMENT FACTOR D cleaves it to form alternative C3 CONVERTASE (C3BBB) which, stabilized by COMPLEMENT FACTOR P, is able to cleave multiple COMPLEMENT C3 to form alternative C5 CONVERTASE (C3BBB3B) leading to cleavage of COMPLEMENT C5 and the assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.
Complement activation initiated by the binding of COMPLEMENT C1 to ANTIGEN-ANTIBODY COMPLEXES at the COMPLEMENT C1Q subunit. This leads to the sequential activation of COMPLEMENT C1R and COMPLEMENT C1S subunits. Activated C1s cleaves COMPLEMENT C4 and COMPLEMENT C2 forming the membrane-bound classical C3 CONVERTASE (C4B2A) and the subsequent C5 CONVERTASE (C4B2A3B) leading to cleavage of COMPLEMENT C5 and the assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.
A 150-kDa serum glycoprotein composed of three subunits with each encoded by a different gene (C8A; C8B; and C8G). This heterotrimer contains a disulfide-linked C8alpha-C8gamma heterodimer and a noncovalently associated C8beta chain. C8 is the next component to bind the C5-7 complex forming C5b-8 that binds COMPLEMENT C9 and acts as a catalyst in the polymerization of C9.
The first complement component to act in the activation of CLASSICAL COMPLEMENT PATHWAY. It is a calcium-dependent trimolecular complex made up of three subcomponents: COMPLEMENT C1Q; COMPLEMENT C1R; and COMPLEMENT C1S at 1:2:2 ratios. When the intact C1 binds to at least two antibodies (involving C1q), C1r and C1s are sequentially activated, leading to subsequent steps in the cascade of COMPLEMENT ACTIVATION.
Molecular sites on or in some B-lymphocytes and macrophages that recognize and combine with COMPLEMENT C3B. The primary structure of these receptors reveal that they contain transmembrane and cytoplasmic domains, with their extracellular portion composed entirely of thirty short consensus repeats each having 60 to 70 amino acids.
An important soluble regulator of the alternative pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It is a 139-kDa glycoprotein expressed by the liver and secreted into the blood. It binds to COMPLEMENT C3B and makes iC3b (inactivated complement 3b) susceptible to cleavage by COMPLEMENT FACTOR I. Complement factor H also inhibits the association of C3b with COMPLEMENT FACTOR B to form the C3bB proenzyme, and promotes the dissociation of Bb from the C3bBb complex (COMPLEMENT C3 CONVERTASE, ALTERNATIVE PATHWAY).
The larger fragment generated from the cleavage of C5 by C5 CONVERTASE that yields COMPLEMENT C5A and C5b (beta chain + alpha' chain, the residual alpha chain, bound by disulfide bond). C5b remains bound to the membrane and initiates the spontaneous assembly of the late complement components to form C5b-8-poly-C9, the MEMBRANE ATTACK COMPLEX.
The COOH-terminal fragment of COMPLEMENT 2, released by the action of activated COMPLEMENT C1S. It is a SERINE PROTEASE. C2a combines with COMPLEMENT C4B to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).
A G-protein-coupled receptor that signals an increase in intracellular calcium in response to the potent ANAPHYLATOXIN peptide COMPLEMENT C5A.
Enzymes that activate one or more COMPLEMENT PROTEINS in the complement system leading to the formation of the COMPLEMENT MEMBRANE ATTACK COMPLEX, an important response in host defense. They are enzymes in the various COMPLEMENT ACTIVATION pathways.
Compounds that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host.
A screening assay for circulating COMPLEMENT PROTEINS. Diluted SERUM samples are added to antibody-coated ERYTHROCYTES and the percentage of cell lysis is measured. The values are expressed by the so called CH50, in HEMOLYTIC COMPLEMENT units per milliliter, which is the dilution of serum required to lyse 50 percent of the erythrocytes in the assay.
Serum proteins that inhibit, antagonize, or inactivate COMPLEMENT C1 or its subunits.
Molecular sites on or in B-lymphocytes, follicular dendritic cells, lymphoid cells, and epithelial cells that recognize and combine with COMPLEMENT C3D. Human complement receptor 2 (CR2) serves as a receptor for both C3dg and the gp350/220 glycoprotein of HERPESVIRUS 4, HUMAN, and binds the monoclonal antibody OKB7, which blocks binding of both ligands to the receptor.
Serum peptides derived from certain cleaved COMPLEMENT PROTEINS during COMPLEMENT ACTIVATION. They induce smooth MUSCLE CONTRACTION; mast cell HISTAMINE RELEASE; PLATELET AGGREGATION; and act as mediators of the local inflammatory process. The order of anaphylatoxin activity from the strongest to the weakest is C5a, C3a, C4a, and C5a des-arginine.
Serologic tests based on inactivation of complement by the antigen-antibody complex (stage 1). Binding of free complement can be visualized by addition of a second antigen-antibody system such as red cells and appropriate red cell antibody (hemolysin) requiring complement for its completion (stage 2). Failure of the red cells to lyse indicates that a specific antigen-antibody reaction has taken place in stage 1. If red cells lyse, free complement is present indicating no antigen-antibody reaction occurred in stage 1.
A serum protein which is important in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY. This enzyme cleaves the COMPLEMENT C3B-bound COMPLEMENT FACTOR B to form C3bBb which is ALTERNATIVE PATHWAY C3 CONVERTASE.
A plasma serine proteinase that cleaves the alpha-chains of C3b and C4b in the presence of the cofactors COMPLEMENT FACTOR H and C4-binding protein, respectively. It is a 66-kDa glycoprotein that converts C3b to inactivated C3b (iC3b) followed by the release of two fragments, C3c (150-kDa) and C3dg (41-kDa). It was formerly called KAF, C3bINF, or enzyme 3b inactivator.
A serum protein that regulates the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. It binds as a cofactor to COMPLEMENT FACTOR I which then hydrolyzes the COMPLEMENT C4B in the CLASSICAL PATHWAY C3 CONVERTASE (C4bC2a).
Endogenous proteins that inhibit or inactivate COMPLEMENT C3B. They include COMPLEMENT FACTOR H and COMPLEMENT FACTOR I (C3b/C4b inactivator). They cleave or promote the cleavage of C3b into inactive fragments, and thus are important in the down-regulation of COMPLEMENT ACTIVATION and its cytolytic sequence.
GPI-linked membrane proteins broadly distributed among hematopoietic and non-hematopoietic cells. CD55 prevents the assembly of C3 CONVERTASE or accelerates the disassembly of preformed convertase, thus blocking the formation of the membrane attack complex.
Important enzymes in the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. They cleave COMPLEMENT C3 and COMPLEMENT C5.
The N-terminal fragment of COMPLEMENT 2, released by the action of activated COMPLEMENT C1S.
Small glycoproteins found on both hematopoietic and non-hematopoietic cells. CD59 restricts the cytolytic activity of homologous complement by binding to C8 and C9 and blocking the assembly of the membrane attack complex. (From Barclay et al., The Leukocyte Antigen FactsBook, 1993, p234)
Venoms from snakes of the genus Naja (family Elapidae). They contain many specific proteins that have cytotoxic, hemolytic, neurotoxic, and other properties. Like other elapid venoms, they are rich in enzymes. They include cobramines and cobralysins.
The complex formed by the binding of antigen and antibody molecules. The deposition of large antigen-antibody complexes leading to tissue damage causes IMMUNE COMPLEX DISEASES.
An adrenal microsomal cytochrome P450 enzyme that catalyzes the 21-hydroxylation of steroids in the presence of molecular oxygen and NADPH-FERRIHEMOPROTEIN REDUCTASE. This enzyme, encoded by CYP21 gene, converts progesterones to precursors of adrenal steroid hormones (CORTICOSTERONE; HYDROCORTISONE). Defects in CYP21 cause congenital adrenal hyperplasia (ADRENAL HYPERPLASIA, CONGENITAL).
Important enzymes in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY. They cleave COMPLEMENT C3 and COMPLEMENT C5.
An endogenous 105-kDa plasma glycoprotein produced primarily by the LIVER and MONOCYTES. It inhibits a broad spectrum of proteases, including the COMPLEMENT C1R and the COMPLEMENT C1S proteases of the CLASSICAL COMPLEMENT PATHWAY, and the MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES. C1-INH-deficient individuals suffer from HEREDITARY ANGIOEDEMA TYPES I AND II.
The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B.
The destruction of ERYTHROCYTES by many different causal agents such as antibodies, bacteria, chemicals, temperature, and changes in tonicity.
A serine protease that is the complex of COMPLEMENT C3B and COMPLEMENT FACTOR BB. It cleaves multiple COMPLEMENT C3 into COMPLEMENT C3A (anaphylatoxin) and COMPLEMENT C3B in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY.
A serine protease that cleaves multiple COMPLEMENT 5 into COMPLEMENT 5A (anaphylatoxin) and COMPLEMENT 5B in the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. It is a complex of CLASSICAL PATHWAY C3 CONVERTASE (C4b2a) with an additional COMPLEMENT C3B, or C4b2a3b.
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
A serine protease that cleaves multiple COMPLEMENT 3 into COMPLEMENT 3A (anaphylatoxin) and COMPLEMENT 3B in the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. It is a complex of COMPLEMENT 4B and COMPLEMENT 2A (C4b2a).
A ubiquitously expressed complement receptor that binds COMPLEMENT C3B and COMPLEMENT C4B and serves as a cofactor for their inactivation. CD46 also interacts with a wide variety of pathogens and mediates immune response.
Proteins that bind to particles and cells to increase susceptibility to PHAGOCYTOSIS, especially ANTIBODIES bound to EPITOPES that attach to FC RECEPTORS. COMPLEMENT C3B may also participate.
Proteins that are present in blood serum, including SERUM ALBUMIN; BLOOD COAGULATION FACTORS; and many other types of proteins.
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
A serine protease that cleaves multiple COMPLEMENT C5 into COMPLEMENT C5A (anaphylatoxin) and COMPLEMENT C5B in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY. It is the complex of ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb) with an additional COMPLEMENT C3B, or C3bBb3b.
The engulfing and degradation of microorganisms; other cells that are dead, dying, or pathogenic; and foreign particles by phagocytic cells (PHAGOCYTES).
The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.
Complement activation triggered by the interaction of microbial POLYSACCHARIDES with serum MANNOSE-BINDING LECTIN resulting in the activation of MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES. As in the classical pathway, MASPs cleave COMPLEMENT C4 and COMPLEMENT C2 to form C3 CONVERTASE (C4B2A) and the subsequent C5 CONVERTASE (C4B2A3B) leading to cleavage of COMPLEMENT C5 and assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.
A 53-kDa protein that is a positive regulator of the alternate pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It stabilizes the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb) and protects it from rapid inactivation, thus facilitating the cascade of COMPLEMENT ACTIVATION and the formation of MEMBRANE ATTACK COMPLEX. Individuals with mutation in the PFC gene exhibit properdin deficiency and have a high susceptibility to infections.
A derivative of complement C5a, generated when the carboxy-terminal ARGININE is removed by CARBOXYPEPTIDASE B present in normal human serum. C5a des-Arg shows complete loss of spasmogenic activity though it retains some chemotactic ability (CHEMOATTRACTANTS).
An adhesion-promoting leukocyte surface membrane heterodimer. The alpha subunit consists of the CD11b ANTIGEN and the beta subunit the CD18 ANTIGEN. The antigen, which is an integrin, functions both as a receptor for complement 3 and in cell-cell and cell-substrate adhesive interactions.
The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments.
Granular leukocytes having a nucleus with three to five lobes connected by slender threads of chromatin, and cytoplasm containing fine inconspicuous granules and stainable by neutral dyes.
The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.
A cluster of convoluted capillaries beginning at each nephric tubule in the kidney and held together by connective tissue.
The clear portion of BLOOD that is left after BLOOD COAGULATION to remove BLOOD CELLS and clotting proteins.
Chronic glomerulonephritis characterized histologically by proliferation of MESANGIAL CELLS, increase in the MESANGIAL EXTRACELLULAR MATRIX, and a thickening of the glomerular capillary walls. This may appear as a primary disorder or secondary to other diseases including infections and autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Various subtypes are classified by their abnormal ultrastructures and immune deposits. Hypocomplementemia is a characteristic feature of all types of MPGN.
A class of immunoglobulin bearing mu chains (IMMUNOGLOBULIN MU-CHAINS). IgM can fix COMPLEMENT. The name comes from its high molecular weight and originally being called a macroglobulin.
A genus of trematode flukes belonging to the family Schistosomatidae. There are over a dozen species. These parasites are found in man and other mammals. Snails are the intermediate hosts.
A test used to determine whether or not complementation (compensation in the form of dominance) will occur in a cell with a given mutant phenotype when another mutant genome, encoding the same mutant phenotype, is introduced into that cell.
An immunoassay utilizing an antibody labeled with an enzyme marker such as horseradish peroxidase. While either the enzyme or the antibody is bound to an immunosorbent substrate, they both retain their biologic activity; the change in enzyme activity as a result of the enzyme-antibody-antigen reaction is proportional to the concentration of the antigen and can be measured spectrophotometrically or with the naked eye. Many variations of the method have been developed.
Strains of mice in which certain GENES of their GENOMES have been disrupted, or "knocked-out". To produce knockouts, using RECOMBINANT DNA technology, the normal DNA sequence of the gene being studied is altered to prevent synthesis of a normal gene product. Cloned cells in which this DNA alteration is successful are then injected into mouse EMBRYOS to produce chimeric mice. The chimeric mice are then bred to yield a strain in which all the cells of the mouse contain the disrupted gene. Knockout mice are used as EXPERIMENTAL ANIMAL MODELS for diseases (DISEASE MODELS, ANIMAL) and to clarify the functions of the genes.
Inflammation of the renal glomeruli (KIDNEY GLOMERULUS) that can be classified by the type of glomerular injuries including antibody deposition, complement activation, cellular proliferation, and glomerulosclerosis. These structural and functional abnormalities usually lead to HEMATURIA; PROTEINURIA; HYPERTENSION; and RENAL INSUFFICIENCY.
Thickening of the walls of small ARTERIES or ARTERIOLES due to cell proliferation or HYALINE deposition.
Antibodies produced by a single clone of cells.
The genetic region which contains the loci of genes which determine the structure of the serologically defined (SD) and lymphocyte-defined (LD) TRANSPLANTATION ANTIGENS, genes which control the structure of the IMMUNE RESPONSE-ASSOCIATED ANTIGENS, HUMAN; the IMMUNE RESPONSE GENES which control the ability of an animal to respond immunologically to antigenic stimuli, and genes which determine the structure and/or level of the first four components of complement.
Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN.
Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.
Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.
RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.
The relatively long-lived phagocytic cell of mammalian tissues that are derived from blood MONOCYTES. Main types are PERITONEAL MACROPHAGES; ALVEOLAR MACROPHAGES; HISTIOCYTES; KUPFFER CELLS of the liver; and OSTEOCLASTS. They may further differentiate within chronic inflammatory lesions to EPITHELIOID CELLS or may fuse to form FOREIGN BODY GIANT CELLS or LANGHANS GIANT CELLS. (from The Dictionary of Cell Biology, Lackie and Dow, 3rd ed.)
Established cell cultures that have the potential to propagate indefinitely.
The capacity of a normal organism to remain unaffected by microorganisms and their toxins. It results from the presence of naturally occurring ANTI-INFECTIVE AGENTS, constitutional factors such as BODY TEMPERATURE and immediate acting immune cells such as NATURAL KILLER CELLS.
Partial proteins formed by partial hydrolysis of complete proteins or generated through PROTEIN ENGINEERING techniques.
Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.
The species Oryctolagus cuniculus, in the family Leporidae, order LAGOMORPHA. Rabbits are born in burrows, furless, and with eyes and ears closed. In contrast with HARES, rabbits have 22 chromosome pairs.
Naturally occurring or experimentally induced animal diseases with pathological processes sufficiently similar to those of human diseases. They are used as study models for human diseases.
The insertion of recombinant DNA molecules from prokaryotic and/or eukaryotic sources into a replicating vehicle, such as a plasmid or virus vector, and the introduction of the resultant hybrid molecules into recipient cells without altering the viability of those cells.
The parts of a macromolecule that directly participate in its specific combination with another molecule.
The natural bactericidal property of BLOOD due to normally occurring antibacterial substances such as beta lysin, leukin, etc. This activity needs to be distinguished from the bactericidal activity contained in a patient's serum as a result of antimicrobial therapy, which is measured by a SERUM BACTERICIDAL TEST.
Differentiation antigens residing on mammalian leukocytes. CD stands for cluster of differentiation, which refers to groups of monoclonal antibodies that show similar reactivity with certain subpopulations of antigens of a particular lineage or differentiation stage. The subpopulations of antigens are also known by the same CD designation.
Electrophoresis in which a polyacrylamide gel is used as the diffusion medium.
A specific mannose-binding member of the collectin family of lectins. It binds to carbohydrate groups on invading pathogens and plays a key role in the MANNOSE-BINDING LECTIN COMPLEMENT PATHWAY.
Variant forms of the same gene, occupying the same locus on homologous CHROMOSOMES, and governing the variants in production of the same gene product.
Immunoglobulin molecules having a specific amino acid sequence by virtue of which they interact only with the ANTIGEN (or a very similar shape) that induced their synthesis in cells of the lymphoid series (especially PLASMA CELLS).
Proteins prepared by recombinant DNA technology.
An IgG autoantibody against the ALTERNATIVE PATHWAY C3 CONVERTASE, found in serum of patients with MESANGIOCAPILLARY GLOMERULONEPHRITIS. The binding of this autoantibody to C3bBb stabilizes the enzyme thus reduces the actions of C3b inactivators (COMPLEMENT FACTOR H; COMPLEMENT FACTOR I). This abnormally stabilized enzyme induces a continuous COMPLEMENT ACTIVATION and generation of C3b thereby promoting the assembly of MEMBRANE ATTACK COMPLEX and cytolysis.
Conjugated protein-carbohydrate compounds including mucins, mucoid, and amyloid glycoproteins.
Multi-subunit proteins which function in IMMUNITY. They are produced by B LYMPHOCYTES from the IMMUNOGLOBULIN GENES. They are comprised of two heavy (IMMUNOGLOBULIN HEAVY CHAINS) and two light chains (IMMUNOGLOBULIN LIGHT CHAINS) with additional ancillary polypeptide chains depending on their isoforms. The variety of isoforms include monomeric or polymeric forms, and transmembrane forms (B-CELL ANTIGEN RECEPTORS) or secreted forms (ANTIBODIES). They are divided by the amino acid sequence of their heavy chains into five classes (IMMUNOGLOBULIN A; IMMUNOGLOBULIN D; IMMUNOGLOBULIN E; IMMUNOGLOBULIN G; IMMUNOGLOBULIN M) and various subclasses.
Plasma glycoproteins that form a stable complex with hemoglobin to aid the recycling of heme iron. They are encoded in man by a gene on the short arm of chromosome 16.
A deoxyribonucleotide polymer that is the primary genetic material of all cells. Eukaryotic and prokaryotic organisms normally contain DNA in a double-stranded state, yet several important biological processes transiently involve single-stranded regions. DNA, which consists of a polysugar-phosphate backbone possessing projections of purines (adenine and guanine) and pyrimidines (thymine and cytosine), forms a double helix that is held together by hydrogen bonds between these purines and pyrimidines (adenine to thymine and guanine to cytosine).
A biosensing technique in which biomolecules capable of binding to specific analytes or ligands are first immobilized on one side of a metallic film. Light is then focused on the opposite side of the film to excite the surface plasmons, that is, the oscillations of free electrons propagating along the film's surface. The refractive index of light reflecting off this surface is measured. When the immobilized biomolecules are bound by their ligands, an alteration in surface plasmons on the opposite side of the film is created which is directly proportional to the change in bound, or adsorbed, mass. Binding is measured by changes in the refractive index. The technique is used to study biomolecular interactions, such as antigen-antibody binding.
Peptides whose amino and carboxy ends are linked together with a peptide bond forming a circular chain. Some of them are ANTI-INFECTIVE AGENTS. Some of them are biosynthesized non-ribosomally (PEPTIDE BIOSYNTHESIS, NON-RIBOSOMAL).
Glomerulonephritis associated with autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Lupus nephritis is histologically classified into 6 classes: class I - normal glomeruli, class II - pure mesangial alterations, class III - focal segmental glomerulonephritis, class IV - diffuse glomerulonephritis, class V - diffuse membranous glomerulonephritis, and class VI - advanced sclerosing glomerulonephritis (The World Health Organization classification 1982).
Autoantibodies directed against various nuclear antigens including DNA, RNA, histones, acidic nuclear proteins, or complexes of these molecular elements. Antinuclear antibodies are found in systemic autoimmune diseases including systemic lupus erythematosus, Sjogren's syndrome, scleroderma, polymyositis, and mixed connective tissue disease.
The degree of similarity between sequences of amino acids. This information is useful for the analyzing genetic relatedness of proteins and species.
Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.
Plasmids containing at least one cos (cohesive-end site) of PHAGE LAMBDA. They are used as cloning vehicles.
In vitro method for producing large amounts of specific DNA or RNA fragments of defined length and sequence from small amounts of short oligonucleotide flanking sequences (primers). The essential steps include thermal denaturation of the double-stranded target molecules, annealing of the primers to their complementary sequences, and extension of the annealed primers by enzymatic synthesis with DNA polymerase. The reaction is efficient, specific, and extremely sensitive. Uses for the reaction include disease diagnosis, detection of difficult-to-isolate pathogens, mutation analysis, genetic testing, DNA sequencing, and analyzing evolutionary relationships.
Proteins found in any species of bacterium.
Any of the processes by which nuclear, cytoplasmic, or intercellular factors influence the differential control (induction or repression) of gene action at the level of transcription or translation.
Measurable and quantifiable biological parameters (e.g., specific enzyme concentration, specific hormone concentration, specific gene phenotype distribution in a population, presence of biological substances) which serve as indices for health- and physiology-related assessments, such as disease risk, psychiatric disorders, environmental exposure and its effects, disease diagnosis, metabolic processes, substance abuse, pregnancy, cell line development, epidemiologic studies, etc.
A pathological process characterized by injury or destruction of tissues caused by a variety of cytologic and chemical reactions. It is usually manifested by typical signs of pain, heat, redness, swelling, and loss of function.
Transport proteins that carry specific substances in the blood or across cell membranes.
Serum serine proteases which participate in COMPLEMENT ACTIVATION. They are activated when complexed with the MANNOSE-BINDING LECTIN, therefore also known as Mannose-binding protein-Associated Serine Proteases (MASPs). They cleave COMPLEMENT C4 and COMPLEMENT C2 to form C4b2a, the CLASSICAL PATHWAY C3 CONVERTASE.
A group of inherited disorders of the ADRENAL GLANDS, caused by enzyme defects in the synthesis of cortisol (HYDROCORTISONE) and/or ALDOSTERONE leading to accumulation of precursors for ANDROGENS. Depending on the hormone imbalance, congenital adrenal hyperplasia can be classified as salt-wasting, hypertensive, virilizing, or feminizing. Defects in STEROID 21-HYDROXYLASE; STEROID 11-BETA-HYDROXYLASE; STEROID 17-ALPHA-HYDROXYLASE; 3-beta-hydroxysteroid dehydrogenase (3-HYDROXYSTEROID DEHYDROGENASES); TESTOSTERONE 5-ALPHA-REDUCTASE; or steroidogenic acute regulatory protein; among others, underlie these disorders.
The restriction of a characteristic behavior, anatomical structure or physical system, such as immune response; metabolic response, or gene or gene variant to the members of one species. It refers to that property which differentiates one species from another but it is also used for phylogenetic levels higher or lower than the species.
An individual in which both alleles at a given locus are identical.
Body organ that filters blood for the secretion of URINE and that regulates ion concentrations.
The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment.
Biologically active substances whose activities affect or play a role in the functioning of the immune system.
Elements of limited time intervals, contributing to particular results or situations.
The level of protein structure in which combinations of secondary protein structures (alpha helices, beta sheets, loop regions, and motifs) pack together to form folded shapes called domains. Disulfide bridges between cysteines in two different parts of the polypeptide chain along with other interactions between the chains play a role in the formation and stabilization of tertiary structure. Small proteins usually consist of only one domain but larger proteins may contain a number of domains connected by segments of polypeptide chain which lack regular secondary structure.
Histochemical localization of immunoreactive substances using labeled antibodies as reagents.
The number of copies of a given gene present in the cell of an organism. An increase in gene dosage (by GENE DUPLICATION for example) can result in higher levels of gene product formation. GENE DOSAGE COMPENSATION mechanisms result in adjustments to the level GENE EXPRESSION when there are changes or differences in gene dosage.
The genetic constitution of individuals with respect to one member of a pair of allelic genes, or sets of genes that are closely linked and tend to be inherited together such as those of the MAJOR HISTOCOMPATIBILITY COMPLEX.
Proteins which are found in membranes including cellular and intracellular membranes. They consist of two types, peripheral and integral proteins. They include most membrane-associated enzymes, antigenic proteins, transport proteins, and drug, hormone, and lectin receptors.
Antigens determined by leukocyte loci found on chromosome 6, the major histocompatibility loci in humans. They are polypeptides or glycoproteins found on most nucleated cells and platelets, determine tissue types for transplantation, and are associated with certain diseases.
Glycoproteins found on the membrane or surface of cells.
The sequential correspondence of nucleotides in one nucleic acid molecule with those of another nucleic acid molecule. Sequence homology is an indication of the genetic relatedness of different organisms and gene function.
The phenotypic manifestation of a gene or genes by the processes of GENETIC TRANSCRIPTION and GENETIC TRANSLATION.
Large, phagocytic mononuclear leukocytes produced in the vertebrate BONE MARROW and released into the BLOOD; contain a large, oval or somewhat indented nucleus surrounded by voluminous cytoplasm and numerous organelles.
The sum of the weight of all the atoms in a molecule.
The rate dynamics in chemical or physical systems.
Plasma glycoprotein clotted by thrombin, composed of a dimer of three non-identical pairs of polypeptide chains (alpha, beta, gamma) held together by disulfide bonds. Fibrinogen clotting is a sol-gel change involving complex molecular arrangements: whereas fibrinogen is cleaved by thrombin to form polypeptides A and B, the proteolytic action of other enzymes yields different fibrinogen degradation products.
The parts of a transcript of a split GENE remaining after the INTRONS are removed. They are spliced together to become a MESSENGER RNA or other functional RNA.
Lymphoid cells concerned with humoral immunity. They are short-lived cells resembling bursa-derived lymphocytes of birds in their production of immunoglobulin upon appropriate stimulation.
The presence of proteins in the urine, an indicator of KIDNEY DISEASES.
Technique using an instrument system for making, processing, and displaying one or more measurements on individual cells obtained from a cell suspension. Cells are usually stained with one or more fluorescent dyes specific to cell components of interest, e.g., DNA, and fluorescence of each cell is measured as it rapidly transverses the excitation beam (laser or mercury arc lamp). Fluorescence provides a quantitative measure of various biochemical and biophysical properties of the cell, as well as a basis for cell sorting. Other measurable optical parameters include light absorption and light scattering, the latter being applicable to the measurement of cell size, shape, density, granularity, and stain uptake.
The production of ANTIBODIES by proliferating and differentiated B-LYMPHOCYTES under stimulation by ANTIGENS.
Any member of the group of ENDOPEPTIDASES containing at the active site a serine residue involved in catalysis.
A gram-positive organism found in the upper respiratory tract, inflammatory exudates, and various body fluids of normal and/or diseased humans and, rarely, domestic animals.
A class of C-type lectins that target the carbohydrate structures found on invading pathogens. Binding of collectins to microorganisms results in their agglutination and enhanced clearance. Collectins form trimers that may assemble into larger oligomers. Each collectin polypeptide chain consists of four regions: a relatively short N-terminal region, a collagen-like region, an alpha-helical coiled-coil region, and carbohydrate-binding region.
Use of restriction endonucleases to analyze and generate a physical map of genomes, genes, or other segments of DNA.
A category of nucleic acid sequences that function as units of heredity and which code for the basic instructions for the development, reproduction, and maintenance of organisms.
Short sequences (generally about 10 base pairs) of DNA that are complementary to sequences of messenger RNA and allow reverse transcriptases to start copying the adjacent sequences of mRNA. Primers are used extensively in genetic and molecular biology techniques.
A plasma protein that circulates in increased amounts during inflammation and after tissue damage.
The genetic constitution of the individual, comprising the ALLELES present at each GENETIC LOCUS.
A positive regulatory effect on physiological processes at the molecular, cellular, or systemic level. At the molecular level, the major regulatory sites include membrane receptors, genes (GENE EXPRESSION REGULATION), mRNAs (RNA, MESSENGER), and proteins.
Lipid-containing polysaccharides which are endotoxins and important group-specific antigens. They are often derived from the cell wall of gram-negative bacteria and induce immunoglobulin secretion. The lipopolysaccharide molecule consists of three parts: LIPID A, core polysaccharide, and O-specific chains (O ANTIGENS). When derived from Escherichia coli, lipopolysaccharides serve as polyclonal B-cell mitogens commonly used in laboratory immunology. (From Dorland, 28th ed)
Cytochrome P-450 monooxygenases (MIXED FUNCTION OXYGENASES) that are important in steroid biosynthesis and metabolism.
Detection of RNA that has been electrophoretically separated and immobilized by blotting on nitrocellulose or other type of paper or nylon membrane followed by hybridization with labeled NUCLEIC ACID PROBES.
Lymphocytes responsible for cell-mediated immunity. Two types have been identified - cytotoxic (T-LYMPHOCYTES, CYTOTOXIC) and helper T-lymphocytes (T-LYMPHOCYTES, HELPER-INDUCER). They are formed when lymphocytes circulate through the THYMUS GLAND and differentiate to thymocytes. When exposed to an antigen, they divide rapidly and produce large numbers of new T cells sensitized to that antigen.
Single-stranded complementary DNA synthesized from an RNA template by the action of RNA-dependent DNA polymerase. cDNA (i.e., complementary DNA, not circular DNA, not C-DNA) is used in a variety of molecular cloning experiments as well as serving as a specific hybridization probe.
A method (first developed by E.M. Southern) for detection of DNA that has been electrophoretically separated and immobilized by blotting on nitrocellulose or other type of paper or nylon membrane followed by hybridization with labeled NUCLEIC ACID PROBES.
Non-antibody proteins secreted by inflammatory leukocytes and some non-leukocytic cells, that act as intercellular mediators. They differ from classical hormones in that they are produced by a number of tissue or cell types rather than by specialized glands. They generally act locally in a paracrine or autocrine rather than endocrine manner.
Degenerative changes in the RETINA usually of older adults which results in a loss of vision in the center of the visual field (the MACULA LUTEA) because of damage to the retina. It occurs in dry and wet forms.
A constitution or condition of the body which makes the tissues react in special ways to certain extrinsic stimuli and thus tends to make the individual more than usually susceptible to certain diseases.
Models used experimentally or theoretically to study molecular shape, electronic properties, or interactions; includes analogous molecules, computer-generated graphics, and mechanical structures.
A mass spectrometric technique that is used for the analysis of large biomolecules. Analyte molecules are embedded in an excess matrix of small organic molecules that show a high resonant absorption at the laser wavelength used. The matrix absorbs the laser energy, thus inducing a soft disintegration of the sample-matrix mixture into free (gas phase) matrix and analyte molecules and molecular ions. In general, only molecular ions of the analyte molecules are produced, and almost no fragmentation occurs. This makes the method well suited for molecular weight determinations and mixture analysis.
A variation of the PCR technique in which cDNA is made from RNA via reverse transcription. The resultant cDNA is then amplified using standard PCR protocols.
The lipid- and protein-containing, selectively permeable membrane that surrounds the cytoplasm in prokaryotic and eukaryotic cells.
The record of descent or ancestry, particularly of a particular condition or trait, indicating individual family members, their relationships, and their status with respect to the trait or condition.
Studies which start with the identification of persons with a disease of interest and a control (comparison, referent) group without the disease. The relationship of an attribute to the disease is examined by comparing diseased and non-diseased persons with regard to the frequency or levels of the attribute in each group.
Variation occurring within a species in the presence or length of DNA fragment generated by a specific endonuclease at a specific site in the genome. Such variations are generated by mutations that create or abolish recognition sites for these enzymes or change the length of the fragment.
The proportion of one particular in the total of all ALLELES for one genetic locus in a breeding POPULATION.
A common name used for the genus Cavia. The most common species is Cavia porcellus which is the domesticated guinea pig used for pets and biomedical research.
A method for the detection of very small quantities of antibody in which the antigen-antibody-complement complex adheres to indicator cells, usually primate erythrocytes or nonprimate blood platelets. The reaction is dependent on the number of bound C3 molecules on the C3b receptor sites of the indicator cell.
A species of gram-negative, facultatively anaerobic, rod-shaped bacteria (GRAM-NEGATIVE FACULTATIVELY ANAEROBIC RODS) commonly found in the lower part of the intestine of warm-blooded animals. It is usually nonpathogenic, but some strains are known to produce DIARRHEA and pyogenic infections. Pathogenic strains (virotypes) are classified by their specific pathogenic mechanisms such as toxins (ENTEROTOXIGENIC ESCHERICHIA COLI), etc.
A technique that combines protein electrophoresis and double immunodiffusion. In this procedure proteins are first separated by gel electrophoresis (usually agarose), then made visible by immunodiffusion of specific antibodies. A distinct elliptical precipitin arc results for each protein detectable by the antisera.
Potentially pathogenic bacteria found in nasal membranes, skin, hair follicles, and perineum of warm-blooded animals. They may cause a wide range of infections and intoxications.
The uptake of naked or purified DNA by CELLS, usually meaning the process as it occurs in eukaryotic cells. It is analogous to bacterial transformation (TRANSFORMATION, BACTERIAL) and both are routinely employed in GENE TRANSFER TECHNIQUES.
A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.
Either of the pair of organs occupying the cavity of the thorax that effect the aeration of the blood.
A chronic systemic disease, primarily of the joints, marked by inflammatory changes in the synovial membranes and articular structures, widespread fibrinoid degeneration of the collagen fibers in mesenchymal tissues, and by atrophy and rarefaction of bony structures. Etiology is unknown, but autoimmune mechanisms have been implicated.
Immunoglobulins produced in a response to BACTERIAL ANTIGENS.
The systematic study of the complete complement of proteins (PROTEOME) of organisms.
Test for tissue antigen using either a direct method, by conjugation of antibody with fluorescent dye (FLUORESCENT ANTIBODY TECHNIQUE, DIRECT) or an indirect method, by formation of antigen-antibody complex which is then labeled with fluorescein-conjugated anti-immunoglobulin antibody (FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT). The tissue is then examined by fluorescence microscopy.
A cytokine that stimulates the growth and differentiation of B-LYMPHOCYTES and is also a growth factor for HYBRIDOMAS and plasmacytomas. It is produced by many different cells including T-LYMPHOCYTES; MONOCYTES; and FIBROBLASTS.
The characteristic 3-dimensional shape of a protein, including the secondary, supersecondary (motifs), tertiary (domains) and quaternary structure of the peptide chain. PROTEIN STRUCTURE, QUATERNARY describes the conformation assumed by multimeric proteins (aggregates of more than one polypeptide chain).
Cells that line the inner and outer surfaces of the body by forming cellular layers (EPITHELIUM) or masses. Epithelial cells lining the SKIN; the MOUTH; the NOSE; and the ANAL CANAL derive from ectoderm; those lining the RESPIRATORY SYSTEM and the DIGESTIVE SYSTEM derive from endoderm; others (CARDIOVASCULAR SYSTEM and LYMPHATIC SYSTEM) derive from mesoderm. Epithelial cells can be classified mainly by cell shape and function into squamous, glandular and transitional epithelial cells.
The relationship between the chemical structure of a compound and its biological or pharmacological activity. Compounds are often classed together because they have structural characteristics in common including shape, size, stereochemical arrangement, and distribution of functional groups.
A large collection of DNA fragments cloned (CLONING, MOLECULAR) from a given organism, tissue, organ, or cell type. It may contain complete genomic sequences (GENOMIC LIBRARY) or complementary DNA sequences, the latter being formed from messenger RNA and lacking intron sequences.
The intracellular transfer of information (biological activation/inhibition) through a signal pathway. In each signal transduction system, an activation/inhibition signal from a biologically active molecule (hormone, neurotransmitter) is mediated via the coupling of a receptor/enzyme to a second messenger system or to an ion channel. Signal transduction plays an important role in activating cellular functions, cell differentiation, and cell proliferation. Examples of signal transduction systems are the GAMMA-AMINOBUTYRIC ACID-postsynaptic receptor-calcium ion channel system, the receptor-mediated T-cell activation pathway, and the receptor-mediated activation of phospholipases. Those coupled to membrane depolarization or intracellular release of calcium include the receptor-mediated activation of cytotoxic functions in granulocytes and the synaptic potentiation of protein kinase activation. Some signal transduction pathways may be part of larger signal transduction pathways; for example, protein kinase activation is part of the platelet activation signal pathway.
A latent susceptibility to disease at the genetic level, which may be activated under certain conditions.
A condition characterized by the recurrence of HEMOGLOBINURIA caused by intravascular HEMOLYSIS. In cases occurring upon cold exposure (paroxysmal cold hemoglobinuria), usually after infections, there is a circulating antibody which is also a cold hemolysin. In cases occurring during or after sleep (paroxysmal nocturnal hemoglobinuria), the clonal hematopoietic stem cells exhibit a global deficiency of cell membrane proteins.
A single nucleotide variation in a genetic sequence that occurs at appreciable frequency in the population.
Group of diseases mediated by the deposition of large soluble complexes of antigen and antibody with resultant damage to tissue. Besides SERUM SICKNESS and the ARTHUS REACTION, evidence supports a pathogenic role for immune complexes in many other IMMUNE SYSTEM DISEASES including GLOMERULONEPHRITIS, systemic lupus erythematosus (LUPUS ERYTHEMATOSUS, SYSTEMIC) and POLYARTERITIS NODOSA.

In vitro analysis of complement-dependent HIV-1 cell infection using a model system. (1/259)

Previous studies based on the use of human serum as a source of C have provided evidence for the C-dependent enhancement of cell infection by HIV-1. The present study was undertaken to distinguish C from other serum factors and to identify the proteins and the mechanisms involved in C-dependent cell infection by HIV-1. The classical C activation pathway was reconstituted from the proteins C1q, C1r, C1s, C4, C2, C3, factor H, and factor I; each were purified to homogeneity. A mixture of these proteins at physiological concentrations was shown to reproduce the ability of normal human serum to enhance the infection of MT2 cells by HIV-1 at low doses of virus. This enhancing effect was abolished when heat-inactivated serum and C2- or C3-depleted serum were used, and was restored upon addition of the corresponding purified proteins. A mixture of two synthetic peptides corresponding to positions 10-15 and 90-97 of human C receptor type 2 (CD21) as well as soluble CD4 both inhibited the C-dependent infection process. These data provide unambiguous evidence that HIV-1 triggers a direct activation of the classical C pathway in vitro and thereby facilitates the infection of MT2 cells at low doses of virus. These findings are consistent with a mechanism involving increased interaction between the virus opsonized by C3b-derived fragment(s) and the CD21 cell receptors and subsequent virus entry through CD4 receptors.  (+info)

Cutting edge: C1q protects against the development of glomerulonephritis independently of C3 activation. (2/259)

C1q-deficient (C1qa-/-) mice develop antinuclear Abs and glomerulonephritis (GN) characterized by multiple apoptotic bodies. To explore the contribution of C3 activation to the induction of spontaneous GN, C1qa-/- mice were crossed with factor B- and C2-deficient (H2-Bf/C2-/-) mice. GN was present in 64% of the 45 C1qa/H2-Bf/C2-/- mice compared with 8% of the 65 H2-Bf/C2-/- mice and none of the 24 wild-type controls. IgG was detected in the glomeruli of diseased C1qa/H2-Bf/C2-/- kidneys. However, glomerular staining for C3 was absent. Increased numbers of glomerular apoptotic bodies were detected in undiseased C1qa/H2-Bf/C2-/- kidneys. These findings support the hypothesis that C1q may play a role in the clearance of apoptotic cells without the necessity for C3 activation and demonstrate that the activation of C3 is not essential for the development of GN in this spontaneous model of lupus-like disease.  (+info)

Calcium-independent haemolysis via the lectin pathway of complement activation in the guinea-pig and other species*. (3/259)

We previously reported that complement-dependent haemolysis of sheep erythrocytes (E) coated with mannan (M) and sensitized with human mannan-binding lectin (MBL) via the lectin pathway in man occurs in Mg-EGTA and requires alternative pathway amplification. Calcium was required for MBL binding to E-M, but once the E-M-MBL intermediate was formed, MBL was retained and haemolysis occurred in the absence of calcium. Comparable or greater lectin pathway haemolysis in the absence of calcium was observed upon incubation of E-M-MBL in guinea-pig, rat, dog and pig sera, and was further investigated in the guinea-pig, in which titres were much higher ( approximately 14-fold) than in man, and in contrast to humans, greater than classical pathway haemolytic activity. As in human serum, no lysis was observed in C4- or C2-deficient guinea-pig serum until purified C4 or C2, respectively, were restored. However, lectin pathway haemolytic activity in the guinea-pig did not require the alternative pathway. Removal (>98%) of factor D activity by three sequential passages through Sephadex G-75, resulting in serum which retained a normal classical pathway but no alternative pathway haemolytic activity, did not reduce the ability of guinea-pig serum to mediate haemolysis via the lectin pathway. Further, the C3-convertase formed via the lectin pathway (E-M-MBL-C4,2) lysed in C2-deficient guinea-pig but not human serum chelated with EDTA, a condition which precludes alternative pathway amplification. Thus, lectin pathway haemolysis occurs efficiently in guinea-pig serum, in the absence of calcium and without requirement for alternative pathway amplification. The guinea-pig provides a model for studying the assembly and haemolytic function of a lectin pathway which contrasts with the lectin pathway of man, and allows for comparisons that may help clarify the role of this pathway in complement biology.  (+info)

IgG and complement-mediated tissue damage in the absence of C2: evidence of a functionally active C2-bypass pathway in a guinea pig model. (4/259)

In vitro complement-mediated lysis of heavily sensitized sheep erythrocytes by C4-deficient (C4D) guinea pig and C2-deficient (C2D) human sera was demonstrated some years ago. It was postulated that these "complement-bypass" pathways resulted from activation of C1 and components of the alternative pathway. We used normal, C2D, and C4D guinea pigs in a Forssman shock model to test the in vivo relevance of the C2- and C4-bypass pathways of complement activation. High concentrations of both anti-Forssman Ab and C2D or C4D guinea pig serum induced efficient lysis of sheep erythrocytes in vitro. The most efficient lysis was observed when IgG Ab and C2D guinea pig serum were used. Blocking either the classical pathway (treatments with EGTA-Mg2+ or soluble recombinant complement receptor type 1 (sCR1)) or the alternative pathway (treatment with heating at 50 degrees C, sCR1, or soluble recombinant CR1 lacking the first of the four long homologous repeat sequences (sCR1[desLHR-A])) inhibited lysis; both pathways were required for lysis of sheep erythrocytes by C2D and C4D guinea pig sera. i.v. injection of anti-Forssman Ab in normal guinea pigs resulted in rapid death from pulmonary shock, whereas C4D guinea pigs had no adverse effect. Surprisingly, C2D guinea pigs either died in a delayed fashion or had a sublethal reaction. sCR1 treatment prevented Forssman shock in both normal and C2D guinea pigs, whereas sCR1[desLHR-A] prevented Forssman shock only in C2D animals. Our results suggest that the C2-bypass pathway occurs in vivo to produce tissue damage. Activation of complement in the absence of C2 appears to be far more efficient than in the absence of C4.  (+info)

On the mechanism of cytolysis by complement: evidence on insertion of C5b and C7 subunits of the C5b,6,7 complex into phospholipid bilayers of erythrocyte membranes. (5/259)

The doughnut hypothesis of cytolysis by complement [Mayer, M. M. (1972) Proc. Nat. Acad. Sci. USA 69, 2954-2958] describes an annular structure made up of C5b-9 (complement factors C5b, C6, C7, C8, and C9) which becomes inserted in the lipid bilayer of the cell membrane, thus creating a hole. We now present initial explorations of this hypothesis. EAC1-6 and EAC1-7 (sheep erythrocytes carrying rabbit antibody and complement factors C1 through C6 or C1 through C7, respectively), prepared with either 125I-C3 or 125I-C5 were incubated with trypsin and the release of bound 125I was measured. In the case of 125I-C3, all of the radioactivity was released by trypsin from both intermediates. With 125I-C5, trypsin released all of the 125I from EAC1-6, but only 40-55% from EAC1-7. Possible reasons for resistance of the C5b subunit in EAC1-7 to tryptic digestion are discussed; in terms of the doughnut hypothesis it would be due to shielding by lipid molecules as a consequence of insertion into the lipid bilayer. In accord with this interpretation we have also found that C5b in EAC1-7, but not in EAC1-6, resists elution by 0.3 M NaC1. Similarly, we have found that 125I-C7 in EAC1-7 resists stripping by trypsin. Hence, we now propose the hypothesis that hydrophobic polypeptide chains from the C5b and the C7 subunits of C5b,6,7 complex become inserted in the phospholipid bilayer and that subsequent reactions with C8 and C9 open a channel across the membrane.  (+info)

New structural motifs on the chymotrypsin fold and their potential roles in complement factor B. (6/259)

Factor B and C2 are two central enzymes for complement activation. They are multidomain serine proteases and require cofactor binding for full expression of proteolytic activities. We present a 2.1 A crystal structure of the serine protease domain of factor B. It shows a number of structural motifs novel to the chymotrypsin fold, which by sequence homology are probably present in C2 as well. These motifs distribute characteristically on the protein surface. Six loops surround the active site, four of which shape substrate-binding pockets. Three loops next to the oxyanion hole, which typically mediate zymogen activation, are much shorter or absent. Three insertions including the linker to the preceding domain bulge from the side opposite to the active site. The catalytic triad and non-specific substrate-binding site display active conformations, but the oxyanion hole displays a zymogen-like conformation. The bottom of the S1 pocket has a negative charge at residue 226 instead of the typical 189 position. These unique structural features may play different roles in domain-domain interaction, cofactor binding and substrate binding.  (+info)

A Schistosoma protein, Sh-TOR, is a novel inhibitor of complement which binds human C2. (7/259)

Human complement regulatory (also called inhibitory) proteins control misdirected attack of complement against autologous cells. Trypanosome and schistosome parasites which survive in the host vascular system also possess regulators of human complement. We have shown Sh-TOR, a protein with three predicted transmembrane domains, located on the Schistosoma parasite surface, to be a novel complement regulatory receptor. The N-terminal extracellular domain, Sh-TOR-ed1, binds the complement protein C2 from human serum and specifically interacts with the C2a fragment. As a result Sh-TOR-ed1 pre-incubated with C2 inhibits classical pathway (CP)-mediated haemolysis of sheep erythrocytes in a dose-dependent manner. In CP-mediated complement activation, C2 normally binds to C4b to form the CP C3 convertase and Sh-TOR-ed1 has short regions of sequence identity with a segment of human C4b. We propose the more appropriate name for TOR of CRIT (complement C2 receptor inhibitory trispanning).  (+info)

Staphylococcus aureus opsonization mediated via the classical and alternative complement pathways. A kinetic study using MgEGTA chelated serum and human sera deficient in IgG and complement factors C1s and C2. (8/259)

Staphylococcus aureus opsonization was studied kinetically by: (1) determination of the uptake of [3H]-thymidine labelled bacteria by human PMN's; (2) fluorescent anti-C3 and anti-IgG staining of opsonized bacteria; and (3) measuring bacterial complement consumption. Maximum opsonization in normal serum occurred within 5 min of incubation. About 80% of staphylococci were then taken up by PMN's, and IgG and C3b could be detected on the bacterial surface. In the absence of a functional classical complement pathway, as in sera deficient in C1s and C2 and in MgEGTA chelated serum, maximal opsonization was only achieved after 30--60 min incubation. Opsonization in IgG deficient serum occurred at a rate similar to that found in C2 deficient or MgEGTA chelated serum. Opsonization was greatly enhanced when sera were reconstituted. It was concluded that in IgG deficient serum Staphylococcus aureus opsonization is mediated via the alternative complement pathway. Dilution of normal serum primarily affected the classical complement pathway, resulting in a decreased rate of opsonization. In normal serum IgG did not appear to be a rate-limiting factor. S. Aureus opsonization was best studied by the phagocytosis assay and the fluorescent-antibody technique. Measuring haemolytic complement consumption was found to be an insensitive indicator of bacterial complement activation and opsonization.  (+info)

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The Rate of Plasmodium vivax Infectivity within Gloucose-6-Phosphate Dehydrogenase (G6PD) Deficient Individuals in Hormozgan Province, Iran.
Purpose: : Complement activation is associated with the pathogenesis of retinal dystrophies such as age-related macular degeneration (AMD). We aimed to investigate the expression and localization of a crucial component of complement, C3, in the retina during aging and in a light-induced degenerative model of atrophic AMD. Methods: : In the degenerative model, SD rats were exposed to 1000 lux of light for up to 24hrs (1, 3, 6, 12, 17, and 24hrs), after which some animals were kept in dim light (5 lux) to recover (3 and 7 days). In the aging model, SD rats were born and reared in age groups corresponding to post-natal (P) days 100, 450, and 750. For both models at their respective time-points, animals were euthanized and retinas processed. The expression of C3 was assessed by qPCR (n=3), immunohistochemistry (n=3), and in situ hybridization (n=3). Counts were made of C3-expressing monocytes using in situ hybridization (n=3). In conjunction, photoreceptor apoptosis was assessed using TUNEL labeling ...
A separation assembly for initially keeping a first component separate from a second component includes a generally cylindrical body slidably supported within a container between the first and second components. The body includes a seal structure having an inner seal member that initially seals the first component from the second component. In response to a predetermined operating condition, the inner seal member allows the first component to flow through at least one flow path in the seal structure to mix with the second component. The body further includes a flow distributing member disposed adjacent the seal structure to evenly distribute the first component into the second component. The seal structure and the flow distributing member form a single unit.
A medical device including a catheter having a first component having a first color and a wall thickness and a second component having a second color different from the first color and a wall thickness. The first component and the second component are joined by a lap weld having a wall thickness such that the wall thickness of the lap weld is greater than the first component wall thickness and also greater than the second component wall thickness. The first component wall thickness is spaced apart from the lap weld and the second component wall thickness is spaced apart from the lap weld. The first component is disposed over the second component and the first color is selected from the group consisting of blue, green, orange, yellow, or purple.
An apparatus and method for implanting a prosthesis includes implanting a first component into a recess in a bone. The first component defines a main body defining a receiving portion and a locating bore. A second component is located into engagement with the first component, the second component defining a passage therethrough. A rod is inserted through the passage defined on the second component and into the locating bore of the first component. A handle associated with the rod is slidably actuated into contact with the second component to matingly lock the first component to the second component.
TY - JOUR. T1 - Complement gene variants in relation to autoantibodies to beta cell specific antigens and type 1 diabetes in the TEDDY Study. AU - Törn, Carina. AU - Liu, Xiang. AU - Hagopian, William. AU - Lernmark, Åke. AU - Simell, Olli. AU - Rewers, Marian. AU - Ziegler, Anette G.. AU - Schatz, Desmond. AU - Akolkar, Beena. AU - Onengut-Gumuscu, Suna. AU - Chen, Wei Min. AU - Toppari, Jorma. AU - Mykkänen, Juha. AU - Ilonen, Jorma. AU - Rich, Stephen S.. AU - She, Jin-Xiong. AU - Sharma, Ashok Kumar. AU - Steck, Andrea. AU - Krischer, Jeffrey. PY - 2016/6/16. Y1 - 2016/6/16. N2 - A total of 15 SNPs within complement genes and present on the ImmunoChip were analyzed in The Environmental Determinants of Diabetes in the Young (TEDDY) study. A total of 5474 subjects were followed from three months of age until islet autoimmunity (IA: n = 413) and the subsequent onset of type 1 diabetes (n = 115) for a median of 73 months (IQR 54-91). Three SNPs within ITGAM were nominally associated (p , ...
A measuring device for determining the relative offset between two components in a z-direction includes two measuring members. A first measuring member is affixable on a first component, and the second measuring member is affixable on a second component. Furthermore, the measuring device includes a sensor device for determining the relative position of the two measuring members. The first measuring member and the second measuring member are affixable on the first components at a rigid angle. At least one of the measuring members is able to be brought into adhesive contact with the first component or the second component. The measuring device includes support members for at least one measuring member so that the measuring member is able to assume a parking or an operating position. The measuring members are precisely and reproducibly aligned in space relative to each other in the parking position.
Pharmaceutical compositions which release the active ingredient slowly are based upon a growth factor or hormone as active ingredient and a means for effecting slow release of the active ingredient. Means for effecting slow release of the active ingredient comprise a conjugate or mixture of a first component and a second component. The first component is a protein other than the active ingredient for binding growth factors and hormones, and the second component is a biodegradable carrier.
A method for separating at least two discrete volumes of a composite liquid into at least a first component and a second component, comprising centrifuging at least two separation bags containing two discrete volumes of a composite liquid respectively, so as to separate therein the first and second components; transferring at least one fraction of a first separated component from the separation bags into satellite bags connected thereto respectively; detecting a characteristic of a component at determined location in each separation bag; and stopping transferring the at least one fraction of the first component from each separation bag into the first satellite bag connected thereto, upon detection of the characteristic of a component at the determined location.
Module Leader: José Zalabardo. Term: 1. Area: C. Assessment: Essay 4500. Shared: BA \ MA. The purpose of this course is to present some of the central doctrines of Wittgensteins Tractatus-: the Tractarian Account of Representation and Reality (TARR). We can characterise TARR as involving three main components.. The first component of TARR is the view that everyday propositions dont represent the world directly: they represent the world through the mediation of a class of postulated propositions, known as elementary propositions (Elementarsätze). Everyday propositions represent the world by being truth functions of elementary propositions.. The second component of TARR is an account of the nature of elementary propositions and of how they represent the world. On this account, an elementary proposition is a combination of items known as names. Names are referential expressions. An elementary proposition represents the referents of its names as combined with one another in the same way in which ...
Our study shows that TREM-2-deficient individuals have a striking defect in OC development, which results in impaired bone resorption in vitro. TREM-2-deficient OC precursors fail to fuse into multinucleated cells, do not develop extensive rearrangements of actin cytoskeleton, and show reduced or no expression of typical OC markers such as vitronectin and calcitonin receptors. TREM-2-deficient immature OCs do express TRAP; however, this molecule is also expressed in other bone marrow-derived cells. This block in OC development leads to impaired performance in dentin resorption assays, potentially reflecting inefficient bone resorption in vivo. It will be important to determine how this defect contributes to the development of cysts and spontaneous fractures in bones of Nasu-Hakola disease patients (9). Consistent with our results, defective development and resorptive function of OCs have been recently observed in DAP12-deficient mice (23). Thus, TREM-2/DAP12 is an essential element in a unique ...
AMD is strongly associated with the over-activation of complement pathways [16]. Supporting evidence comes from the detection of various complement proteins in the drusen of patients with AMD [9] and the close association between AMD and the alternative pathway. Furthermore, the association of genetic variants in complement genes, including the human factor B gene, is a risk factor for all forms of AMD [5,9,22,24]. In addition, it has been shown that polymorphisms of genes encoding the alternative pathway proteins CFH, factor B, and C3, as well as the classical pathway protein C2, are also associated with AMD [23,46]. Studies in animal models of choroidal neovascularization (CNV), a major pathologic association with wet AMD, also supports a role for complements in AMD. Furthermore, a targeted inhibitor specific for the AP of complement significantly reduces CNV and the physiologic consequences of CNV on retinal function [11,47]. Additional evidence for a role of the AP pathway in AMD exists. For ...
TY - JOUR. T1 - Risk of non-Hodgkin lymphoma in association with germline variation in complement genes. AU - Cerhan, James R. AU - Novak, Anne J. AU - Fredericksen, Zachary S.. AU - Wang, Alice H.. AU - Liebow, Mark. AU - Call, Timothy G.. AU - Dogan, Ahmet. AU - Witzig, Thomas Elmer. AU - Ansell, Stephen Maxted. AU - Habermann, Thomas Matthew. AU - Kay, Neil Elliot. AU - Slager, Susan L. PY - 2009/6. Y1 - 2009/6. N2 - Germline mutations in complement genes have been associated with susceptibility to infections and autoimmune diseases, conditions that are associated with non-Hodgkin lymphoma (NHL) risk. To test the hypothesis that common genetic variation in complement genes affect risk of NHL, we genotyped 167 single nucleotide polymorphisms (SNPs) from 31 genes in 441 NHL cases and 475 controls. Principal components (PC) and haplotype analyses were used for gene-level tests of NHL risk, while individual SNPs were modelled as having a log-additive effect. In gene level PC analyses, C2 (P = ...
Activation of the complement cascade is clearly implicated in the pathology of multiple sclerosis (MS). However, the extent and nature of its involvement in specific athological processes remains uncertain. We describe a detailed immunohistochemical study to localise a strategically selected set of complement proteins, activation products and regulators in brain and spinal cord tissue of 17 patients with progressive MS, examining 35 different plaques, and 16 control donors, including 9 with CNS disease. Plaques were consistently positive for complement proteins (C3, factor B, C1q), activation products (C3b, iC3b, C4d, TCC) and regulators (factor H, C1-inhibitor, clusterin), suggesting continuing local complement synthesis, activation and regulation despite no other evidence of on-going inflammation. Complement immunolabelling was most apparent in plaque and peri-plaque areas but also present in normal appearing white matter and cortical areas to a greater extent than in control tissue. Cellular ...
An external mixer assembly is provided which externally mixes and delivers a first and a second component of a biological adhesive to tissues or organs for sealing wounds, stopping bleeding and the like. The first and second components are mixed immediately after exiting from separate outlet ports disposed in fluid communication with component reservoirs. In on embodiment, the external mixer assembly includes a housing having a housing head for enclosing therein a first reservoir containing the first component, and a second reservoir containing the second component. The housing further includes a discharge nozzle defining a longitudinal axis for enclosing therein a conduit assembly having a first and a second conduit in communication with the first and second reservoir, respectively. A deflector assembly is connected to the discharge nozzle. The deflector assembly includes a deflector plate to provide a space for initial mixing of the first and second components. The deflector plate is oriented in
A method of percutaneously implanting a first component and a second component of an orthopaedic assembly into a body of a patient includes the steps of securing a first instrument to the first component, and advancing the first component into the body of the patient. The first instrument is advanced into the body of the patient such that a portion of the first instrument extends out of the body. A second instrument is secured to the second component, and the second component is advanced into the body of the patient. The second instrument is advanced into the body of the patient such that a portion of the second instrument extends out of the body. A third instrument is advanced into contact with both the first instrument and the second instrument so as to position the first component and the second component in a predetermined position relative to one another. An instrument assembly for percutaneously implanting an orthopaedic assembly is also disclosed.
The IUPHAR/BPS Guide to Pharmacology. complement factor B - S1: Chymotrypsin. Detailed annotation on the structure, function, physiology, pharmacology and clinical relevance of drug targets.
A method for ablation in which a portion of atrial tissue around the pulmonary veins of the heart is ablated by a first elongated ablation component and a second elongated ablation component movable relative to the first ablation component and having means for magnetically attracting the first and second components toward one another. The magnetic means draw the first and second components toward one another to compress the atrial tissue therebetween, along the length of the first and second components and thereby position the device for ablation of the tissue.
Influence of the Gαq-protein on myocardial infarct size of the bone marrow-engrafted Gαq-knockout-mouse in a ischemia/reperfusion-model Platelets play a key role in the pathogenesis of an acute myocardial infarction. Thrombus formation, which is mediated by the activation of platelets, results in the occlusion of the coronary artery. On the other hand, agglutinated platelets in the small myocardial vessels impair the myocardial microcirculation. Due to their pro-inflammatory effects, platelets also contribute to the pathogenesis of ischemia/reperfusion injury. The myocardium of Gαq-deficient mice showed a reduced susceptibility to ischemia and reperfusion compared to that of wild-type mice (HEUER, in preparation). Activation of platelets lacking the Gαq-protein is markedly impaired, resulting not only in an increased haemophilia but also in a decreased thrombophilia of Gαq-deficient mice. A bone marrow transplantation was performed to investigate whether the impaired platelet-activation is ...
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Yet consump- tion of rude quantities of booze by ALDH2-deficient individuals triggers symp- toms of acetaldehyde intoxication that include dizziness, nausea, hypotension and palpitations. The mass of proteins in a proteome can top the number of genes expressed in an being, taking into consideration the admissibility opportunity of protein expressed by means of surrogate splicing or with bizarre posttranslational modifications. Marks M, poet T, Abadi M, et al cheap 3 ml bimat with visa symptoms thyroid. The continuation of a drug discovery program close to the integration of more potent and discriminatory inhibitors of parasitic GSK-3s is workable because judgemental differences abide in the ATP-binding take between parasite and human GSK-3 (Ojo et al. Girls who take enunciated contraceptives mainly be undergoing deeply steady 28-day cycles, with lighter bleeding than those who do not take contraceptives. It gave me focus, ongoing goals, and the same dignity safe 100mg allopurinol gastritis ...
A method and apparatus for separation, concentration, and/or applying a biological or bio-engineered fluid. Generally, the fluid application device includes a sprayer body to enable the application of the fluid and a container adaptable to enable the separation of the fluid into at least a first component and a second component. The container is releasably coupled to the nozzle. The nozzle is adapted to withdraw at least one of the first component or the second component from the container after the fluid has been separated to apply the fluid to a selected site.
A dental model and related systems and methods, including a first component representing a portion of a patients jaw and a second component that is demountably attachable to the first component, and a second component representing a dental structure of interest, such as the remaining portion of a tooth or a dental implant.
Cleavage of C5 requires complement fragment C3b which binds C5 and renders it susceptible to cleavage by the C4b,2a complex. Includes former EC 3.4.21.44. Complement component C2a is in peptidase family S1 (trypsin family). Links to other databases: BRENDA, EXPASY, KEGG, MEROPS, Metacyc, PDB, CAS registry number: 56626-15-4. References 1. Kerr, M.A. The second component of human complement. Methods Enzymol. 80 (1980) 54-64. [PMID: 7043188]. 2. Müller-Eberhard, H.J. Molecular organization and function of the complement system. Annu. Rev. Biochem. 57 (1988) 321-347. [PMID: 3052276]. ...
A relation is a set of ordered pairs. The set consisting of the first components of each ordered pair is called the domain and the set consisting of the second components of each ordered pair is called the range. Consider the following set of ordered pairs. The first numbers in each pair are the first five natural numbers. The second number in each pair is twice that of the first.. $$\{(1,\text{ }2),\text{ }(2,\text{ }4),\text{ }(3,\text{ }6),\text{ }(4,\text{ }8),\text{ }(5,\text{ }10)\}$$. The domain is \(\{1,\text{ }2,\text{ }3,\text{ }4,\text{ }5\}\). The range is \(\{2,\text{ }4,\text{ }6,\text{ }8,\text{ }10\}\).. Note that each value in the domain is also known as an input value, or independent variable, and is often labeled with the lowercase letter \(x\). Each value in the range is also known as an output value, or dependent variable, and is often labeled lowercase letter \(y\).. A function \(f\) is a relation that assigns a single value in the range to each value in the domain. In ...
Controlled Labs Orange OxiMega was designed to help you establish a strong, healthful, foundation on which to base your training, diet, and supplementation. Orange OxiMega is comprised of two products in every box. The first is a very high potency Nordic fish oil, the second component is a true sports greens product.
Space time is made up of an inactive energy field in a grid structure, it only becomes active when energy acts on it. A second component of space time are...
Information will flow in our hyper-connected business world but knowledge, which could be infinitely more important to a business, is inherently individually focused. To get that knowledge out and shared we have to overcome several obstacles. As with many enterprise problems we address today this issue has two basic components, culture and technology. By culture I mean creating an environment where sharing individual knowledge to a broader group is the accepted standard of behavior. Once thats done then the second component kicks in, what technology can facilitate effective knowledge sharing?. How do you create a knowledge sharing culture? Looking at the reasons individuals dont choose to share knowledge yields some ideas. Employees protect their knowledge when they believe that its in their best interest to do so. This could be based on the concept that knowledge is property and must be protected or that employees are valued for what they know, which leads to protecting that knowledge, or ...
Protein delivery is an interesting technique that complements gene transfection and RNA interference for studying protein function and cellular pathways.
Looking for online definition of Complement factor b in the Medical Dictionary? Complement factor b explanation free. What is Complement factor b? Meaning of Complement factor b medical term. What does Complement factor b mean?
TY - JOUR. T1 - Coelomocytes express SpBf, a homologue of factor B, the second component in the sea urchin complement system. AU - Smith, L. Courtney. AU - Shih, Chi Schin. AU - Dachenhausen, Sebastian G.. PY - 1998/12/15. Y1 - 1998/12/15. N2 - A homologue of factor B, SpBf, has been cloned and sequenced from an LPS-activated coelomocyte cDNA library from the purple sea urchin, Strongylocentrotus purpuratus. The deduced amino acid sequence and domain structure show significant similarity to the vertebrate Bf/C2 family proteins. SpBf is a mosaic protein, composed of five short consensus repeats, a von Willebrand Factor domain, and a serine protease domain. It has a deduced molecular mass of 91 kDa, with a conserved cleavage site for a putative factor D protease. It has ten consensus recognition sites for N- linked glycosylation. Amino acids involved in both Mg2+ binding and in serine protease activity in the vertebrate C2/Bf proteins are conserved in SpBf. Phylogenetic analysis of SpBf indicates ...
article{28d5c2ad-b244-4d8a-b7d9-b8f6b4fb3b95, abstract = {,p,Severe alpha-1-antitrypsin (AAT) deficiency (PiZZ) is a risk factor for liver disease, but the prevalence of liver cirrhosis and hepatocellular cancer in PiZZ adults is unknown. The risk of liver disease in adults with moderate AAT deficiency (PiSZ) is also unknown. A cohort of 127 PiZZ, 2 PiZnull, 54 PiSZ, and 1 PiSnull individuals were identified by the Swedish national neonatal AAT screening program between 1972 and 1974, when all 200, 000 newborn infants in Sweden were screened for AAT deficiency. The cohort has been followed up since birth. Our aim was to study liver function and signs of liver disease in this cohort at 37 to 40 years of age in comparison with a matched, random sample of control subjects identified from the population registry. Eighty seven PiZZ, 32 PiSZ, and 92 control subjects (PiMM) answered a questionnaire on medication and alcohol consumption and provided blood samples. Liver stiffness was assessed by ...
In this study, we showed that blocking complement activation accelerates the early healing rate in a mouse model of cutaneous wound healing. We also found that the components of the complement system responsible for this effect include C3, C5, and signaling through C5aR1, but not C5aR2 or C3aR. Furthermore, reconstitution of C3-deficient animals with purified human C3 or serum from C3+/+ mice abrogated the effect, confirming the involvement of complement in the process. The absence of these molecules resulted in a reduction in the intensity of inflammation involved in the initial events of healing. We postulate that the reduced inflammation allowed the process to advance faster to the subsequent events of healing (proliferation, maturation), thus accelerating the whole process. Moreover, we observed an increase of vascularization accompanied by a significantly higher presence of mast cells in complement-deficient mice.. A major role of complement effectors is to attract, activate, and control ...
Dr. Brownstein clearly lays out what we would expect to find in iodine deficient individuals. When iodine is deficient nodules form in key organs leading to pre-cancerous conditions and then eventually to full-blown cancer. He says, Iodines main job is to maintain a normal architecture of those tissues. With iodine deficiency, the first thing that happens is you get cystic formation in the breasts, the ovaries, uterus, thyroid, prostate and, lets throw in the pancreas in here as well, which is also increasing at epidemic rates - pancreatic cancer. Cysts start to form when iodine deficiency is there. If it goes on longer, they become nodular and hard. If it goes on longer, they become hyperplastic tissue, which is the precursor to cancer. I say thats the iodine deficiency continuum ...
Dr. Brownstein clearly lays out what we would expect to find in iodine deficient individuals. When iodine is deficient nodules form in key organs leading to pre-cancerous conditions and then eventually to full-blown cancer. He says, Iodines main job is to maintain a normal architecture of those tissues. With iodine deficiency, the first thing that happens is you get cystic formation in the breasts, the ovaries, uterus, thyroid, prostate and, lets throw in the pancreas in here as well, which is also increasing at epidemic rates - pancreatic cancer. Cysts start to form when iodine deficiency is there. If it goes on longer, they become nodular and hard. If it goes on longer, they become hyperplastic tissue, which is the precursor to cancer. I say thats the iodine deficiency continuum ...
Welcome to Episode 10: Supplements; Do you need them? In todays episode, Nav and I discuss supplements. We cover: Navs rationale for why he takes them The usual suspects that can be deficient Individual differences and testing protocols Lets start with the obligatory disclaimer: We are not advising you to …. Continue reading. ...
In this webinar, Mark Dysinger, M.S., Development Scientist III fromAlexion Pharmaceuticals will present A Gyrolab Assay for the Quantitation of Free Complement Protein C5a in Human Plasma.
First we like to confirm complement deficiency (C3, C4, CH50). If complement dysregulation is apparent, we have then been getting Factor H and I assays done on those patients to try and confirm and cause for it. Of course, as we start to understand more about complement dysregulation, more of this pathway will become subject of laboratory assessment ...
Functional complement assays were developed to find patients suffering from complement deficiency. Today these assays are also widely used in drug development of new complement targeted drugs.
2100- This course is the first component of the introductory graduate sequence designed to provide an overview of cellular and molecular aspects of neuroscience. This course covers protein chemistry, regulation of gene expression, nerve cell biology, signal transduction, development, and neurogenesis in a lecture format.. 2101- This course is the second component of the introductory graduate sequence designed to provide an overview of cellular and molecular aspects of neuroscience. This course covers the electrical properties of neurons, signal propagation in nerve cells, and synaptic transmission.. Prerequisites: A background in basic biology and permission of the instructor are required.. Note for CMU students: Section 2 ofthe PCHE Cross Registration Request Form provides a space for students to enroll in a primary choice (course), and a secondary choice in case the primary is not available. Please register for the NROSCI sections as your primary chioce and the MSNBIO sections as your ...
Vessels selected from crucibles, pans, open cups and saggars essentially comprising of two components, from which (A) one component being a monolithic ceramic material, (B) the second component being a ceramic matrix composite, and wherein component (A) is the inner part, or inlay.
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highlighting the link between complement gene expression and IQ, in both schizophrenic patients and healthy controls. The study demonstrated a broad association between variations in complement gene expression and perturbed neurodevelopment. We were particularly interested to see that the final sentence of the paper stated that When complement gene-sets are taken as a whole, their relevance is to neurodevelopment, not illness . Their conclusion rightly touches on the emerging understanding of non-immune roles for complement in diverse biological processes (Hawksworth et al., 2018) .The complement group of proteins is ancient in evolutionary terms. Recognised complement components are found strewn throughout the animal kingdom including in rudimentary diploblastic species (Kimura et al., 2009) . In the human adult, complement functions as an essential pillar of the innate immune system. It is activated by various noxious stimuli and results in the production of the anaphylatoxins and membrane attack
Objective: Complement proteins have been associated with atherosclerosis and cardiovascular risk factors. Recent data suggest a potential role of complement protein C3 in clot stability with hypofibrinolytic and prothrombotic features. Women after menopause are at greater risk of cardiovascular disease and have significantly higher levels of C3 compared with younger women. To better understand the association between complement proteins and atherosclerosis we evaluated the cross-sectional associations between complement proteins C3 and C4 and hemostatic markers (factor VIIc, fibrinogen, plasminogen activator inhibitor-1 (PAI-1) antigen and tissue plasminogen activator (tPA) antigen) in a sample of midlife women.. Design: Pilot data from the Study of Womens Health Across the Nation (SWAN) Pittsburgh site were used. Both C3 and C4 were measured using frozen serum specimens by immunoturbidimetric assay. Data for hemostatic markers were available in SWAN Core data (factor VIIc and fibrinogen were ...
A growing body of evidence has recently accumulated about the pathogenic role of the complement system in AAV (1). In 2007, Xiao et al. (4) demonstrated how infusion of ANCA antibodies in wild-type mice could induce glomerular lesions typical of pauci-immune NCGN; interestingly, no lesion was observed when ANCA were injected in mice knocked out for C5 or complement factor B, whereas RPGN fully developed in C4-knockout mice; because C5 belongs to the terminal part of the complement cascade and complement factor B belongs to the cAP, while C4 is a key molecule of the classic pathway, these findings clearly showed that cAP plays a central role in experimental AAV (4). In keeping with this, Gou et al. showed that patients with active AAV have serologic signs of activation of the cAP (augmented serum levels of activated complement proteins, such as C3a, C5a, sC5b9, and Bb), which correlated with acute-phase reactants, number of crescents, and severity of kidney histology (9). The same group ...
Case of recurrent bacterial infections - investigation, diagnosis and treatment of Complement Deficiency and study of immunology of defects in lytic activity
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Factor B antibody, Internal (complement factor B) for FACS, IHC-P, WB. Anti-Factor B pAb (GTX80605) is tested in Human samples. 100% Ab-Assurance.
The Japanese researchers selected planar ions to build up self-organized materials in which the charged components are stacked in an alternating fashion. The first component is a planar complex made from a small inorganic ion and an organic receptor (receptor-anion complex). The critical structural element of the receptor contains two pyrroles bound into what is known as a -conjugated environment. This means that some of the electrons are freely mobile as an electron cloud over a large area of the molecule. The ligand surrounds the anion on three sides. The second component is a disk-shaped organic cation made from an aromatic ring system, which also has an electron cloud. Because of the electrostatic attraction between oppositely charged ions, and also attractive interactions between the electron clouds, these anions and cations always stack themselves into alternating columnar units ...
2100- This course is the first component of the introductory graduatesequence designed to provide an overview of cellular and molecularaspects of neuroscience. This course covers nerve cell biology, proteinchemistry, regulation of gene expression, receptor function, and secondmessenger signaling in a lecture format. A conference designed todevelop critical reading skills will cover primary literaturecorresponding to material covered in each block. Students will beexpected to read and discuss original scientific literature.. 2101- This course is the second component of the introductory graduatesequence designed to provide an overview of cellular and molecularaspects of neuroscience. This course covers the electrical propertiesof neurons, synaptic transmission and neural development.. Prerequisites: A background in basic biology and permission of the instructor is required.. Note for CMU students: Section 2 ofthe PCHE Cross Registration Request Form provides a space for studentsto enroll in a ...
0042] Having established the principles and signals used in neighbor level compensation, extension to higher-order neighbor level compensation is fairly straightforward. This applies to situations in which two or more modules at different hierarchy levels share more than one input channel in common. For example, there might be a three-input module sharing two inputs with a two-input module. A signal component common to all three inputs will also be common to both inputs of the two-input module, and without compensation, will be rendered at different positions by each module. More generally, there may be a signal component common to all three inputs and a second component common to only the two-input module inputs, requiring that their effects be separated as much as possible for proper rendering of the output soundfield. Consequently, the three-input common signal effects, as embodied in the common input levels described above, should be subtracted from the inputs before the two-input ...
Prior studies of the strategic interaction between taxpayers and the tax authority have focused on reported net taxable income and on audit policies designed to discover potential misstatement of that single item. This study analyzes the impact of component reporting requirements on taxpayer incentives to misstate their tax liability. It also allows the tax authority to tailor its audit policy to consider all tax return information. In particular, the model permits the tax authority to audit return components sequentially: the investigation of a second component is conditional on the results of the first components audit. It is found that partitioning taxable income into a multi-component report reduces overall tax evasion and increases tax authority net revenue collections relative to a single-report model of net taxable income. However, the impact on predicted evasion is not uniform across taxpayers. While some taxpayers reduce evasion, others with multiple opportunities to evade are more ...
In this overview, we initial present the general Homes of wood packaging for direct foods Get hold of: picket substance and its mechanical, Bodily, and normal chemical Attributes, using wooden packaging inside the foodstuff sector, and many samples of the notion of wood packaging by buyers. In the second component, we describe the microbiological position of normal wood for the timber used in the manufacture of picket packaging and methods of microbiological Evaluation of picket product accessible to date ...
An index into M is called an address.. The function pw takes a quantity of width w and returns it with its bytes permuted.. F is a stack of pairs of naturals. FP is the index of the top-most item in F. FS(i) and FN(i) denote respectively the first and second component of the ith item of F. A stack position is a natural p in the range 1… FN(FP).. S holds items of two sorts: a register is a word created by NEW(), and a chunk is a quantity of arbitrary size created by NEW(c) (see section 5.7). The stack items are held in M at word-aligned addresses.. Sp, where p is a stack position, denotes S[FS(FP)+p−1]; &Sp denotes the address of Sp. SP is an abbreviation for FS(FP)+FN(FP)−1.. ...
It complements or replaces the existing compilers (C1/C2 in HotSpot). In contrast to those existing compilers, the GraalVM ...
"Mannan-binding lectin activates C3 and the alternative complement pathway without involvement of C2". J Clin Invest 116: 1425- ... Watford WT, Ghio AJ, Wright JR (2000). "Complement-mediated host defense in the lung". Am J Physiol Lung Cell Mol Physiol 279: ... Watford WT, Wright JR, Hester CG, Jiang H, Frank MM (2001). "Surfactant Protein A Regulates Complement Activation". J Immunol ... Alvarez-Dominguez C, Carrasco-Marin E, Leyva-Cobian F (1993). "Role of Complement Component Clq in Phagocytosis of Listeria ...
... complement factors B, C2, CR3 and CR4; the integrins (I-domains); collagen types VI, VII, XII and XIV; and other extracellular ... ANTXR1; ANTXR2; BF; C2; CACHD1; CACNA2D1; CACNA2D2; CACNA2D3; CACNA2D4; CFB; CLCA1; CLCA2; CLCA4; COCH; COL12A1; COL14A1; ... "The secondary structure of the von Willebrand factor type A domain in factor B of human complement by Fourier transform ...
... inherited complement deficiencies (esp C2 deficiency), scleroderma, Celiac disease. The histomorphologic differential diagnosis ... Type III is very rare, it is characterized by a mixture of subepithelial and subendothelial immune and/or complement deposits. ... It is believed to be associated with the classical complement pathway. Also called recently as 'C3 nephropathy' The preferred ... DDD is associated with deposition of complement C3 within the glomeruli with little or no staining for immunoglobulin. The ...
Suppose its complement in the plane has countably many connected components C1, C2, C3, ... and suppose: the diameter of Ci ...
"Interferon-gamma induces biosynthesis of complement components C2, C4 and factor H by human proximal tubular epithelial cells ...
When a single gene deficiency does cause lupus, it is usually attributed to the complement protein genes C1, C2, or C4. The ...
Also, C4 and C2 cleavage goes unchecked, resulting in auto-activation of the complement system. In its most common form, it ... This way, C1-inhibitor prevents the proteolytic cleavage of later complement components C4 and C2 by C1 and MBL. Although named ... The activation of the complement cascade can cause damage to cells, therefore the inhibition of the complement cascade can work ... which triggers the complement cascade. Activation of the complement cascade attracts phagocytes that leak peroxide and other ...
"A molecular map of the human major histocompatibility complex class III region linking complement genes C4, C2 and factor B". ... Complement component 4 Complement component 4B HLA A1-B8-DR3-DQ2 haplotype Complement system Complement deficiency ... Complement C4-A is a protein that in humans is encoded by the C4A gene. This gene encodes the acidic form of complement factor ... "Entrez Gene: C4A complement component 4A (Rodgers blood group)". Dawkins RL, Uko G, Christiansen FT, Kay PH (Sep 1983). "Low C4 ...
This characteristic distinguishes the C5 step, along with the C2 step, as potentially rate-limiting in the complement reaction ... The complement component C5 can be also activated by fluid phase C5 convertase. C5 is activated by CVFBb in the presence of ... However, unlike C2, C5 remains firmly cell-bound during the decay process and apparently undergoes an alteration in situ which ... The target of C5 convertase is complement protein C5. C5 is a two-chain (α, β) plasma glycoprotein (Mr = 196,000). C5 and C3 ...
Von Willebrand factor, type C (VWFC or VWC)is a protein domain is found in various blood plasma proteins: complement factors B ... C2, CR3 and CR4; the integrins (I-domains); collagen types VI, VII, XII and XIV; and other extracellular proteins. Although the ... "The secondary structure of the von Willebrand factor type A domain in factor B of human complement by Fourier transform ...
This gene complements the ultraviolet sensitivity of xeroderma pigmentosum group C cells and encodes a protein with a C2 domain ... 1990). "Isolation by polymerase chain reaction of a cDNA whose product partially complements the ultraviolet sensitivity of ...
The gene cluster was discovered when genes (specifically those of complement components C2, C4, and factor B) were found in ... However, birds (chicken and quail) have only a single gene, which codes for a complement component gene (C4). In fishes, the ...
The changes in the immune system are complex and include increased expression of complement related genes (C1QB, C2, SERPING1 ...
This enzyme catalyses the following chemical reaction Selective cleavage after Arg223 in complement component C2 (-Ser-Leu-Gly- ... Matsushita M, Fujita T (December 1992). "Activation of the classical complement pathway by mannose-binding protein in ... Arg-Lys-Ile-Gln-Ile) and after Arg76 in complement component C4 (-Gly-Leu-Gln-Arg-Ala-Leu-Glu-Ile) This mannan-binding lectin ( ... "A second serine protease associated with mannan-binding lectin that activates complement". Nature. 386 (6624): 506-10. doi: ...
... components of the complement system (such as C2, C4, and B factor), cytokines (such as TNF-α, LTA, and LTB), and heat shock ...
MASP-2 is activated to cleave complement components C4 and C2 into C4a, C4b, C2a, and C2b. MASP1 (protein) Mannan-binding ... MASP-2 is involved in the complement system. MASP-2 is very similar to the C1s molecule, of the classical complement pathway, ... The Ra-reactive factor (RARF) is a complement-dependent bactericidal factor that binds to the Ra and R2 polysaccharides ... Petersen SV, Thiel S, Jensenius JC (2001). "The mannan-binding lectin pathway of complement activation: biology and disease ...
Here, for consistency, we shall call all large fragments of complement b, so the larger fragment of C2 will be designated C2b. ... Polymorphisms of complement component 3, complement factor B, and complement factor I, as well as deletion of complement factor ... The complement system, also known as complement cascade, is a part of the immune system that enhances (complements) the ability ... Three biochemical pathways activate the complement system: the classical complement pathway, the alternative complement pathway ...
The mechanism of complement deficiency consists of: C2: In regard to C2 deficiency, about 5 different mutations in the C2 gene ... "Complement Deficiencies. What are complement deficiencies?". patient.info. Retrieved 31 December 2017. "Complement Deficiencies ... One of the most common mutations deletes 28 DNA nucleotides from the C2 gene. Therefore, no C2 protein which can help make C3- ... Complement deficiency is an immunodeficiency of absent or suboptimal functioning of one of the complement system proteins. ...
Analysis of complement C1 inhibitor levels may play a role in diagnosis. C4 and C2 are complementary components. There are ... Measure: serum complement factor 4 (C4), C1 inhibitor (C1-INH) antigenic protein, C1 inhibitor (C1-INH) functional level if ... In this type, atypical C1-inhibitor proteins are produced which are less capable of suppressing activation of the complement ... which normally suppress activation of the complement system. The resultant over-stimulation of this system leads to the ...
"Production and interferon-gamma-mediated regulation of complement component C2 and factors B and D by the astroglioma cell line ... Complement factor B is a protein that in humans is encoded by the CFB gene. This gene encodes complement factor B, a component ... "Entrez Gene: CFB complement factor B". Ambrus JL, Peters MG, Fauci AS, Brown EJ (March 1990). "The Ba fragment of complement ... Christie DL, Gagnon J (January 1983). "Amino acid sequence of the Bb fragment from complement Factor B. Sequence of the major ...
Complement C2 is a protein that in humans is encoded by the C2 gene. The protein encoded by this gene is part of the classical ... "Entrez Gene: C2 complement component 2". Krishnan V, Xu Y, Macon K, Volanakis JE, Narayana SV (2009). "The structure of C2b, a ... Deficiency of C2 has been associated with certain autoimmune diseases. In the classical and lectin pathways of complement ... Johnson CA, Densen P, Hurford RK, Colten HR, Wetsel RA (May 1992). "Type I human complement C2 deficiency. A 28-base pair gene ...
It interacts with protein C2; the same protease invoked earlier, C1s, then cleaves C2 into two parts, termed C2a and C2b, with ... Complement component 4A Complement component 4B HLA A1-B8-DR3-DQ2 haplotype Complement system Complement deficiency Sekar A, ... Complement component 4 (C4), in humans, is a protein involved in the intricate complement system, originating from the human ... "A molecular map of the human major histocompatibility complex class III region linking complement genes C4, C2 and factor B". ...
July 2003). "Immunoglobulin deficiencies and susceptibility to infection among homozygotes and heterozygotes for C2 deficiency ... Complement 2 deficiency is a type of complement deficiency caused by any one of several different alterations in the structure ... of complement component 2. It has been associated with an increase in infections. It can present similarly to systemic lupus ...
... chain are important for C2 binding". Journal of Immunology. 165 (5): 2518-27. doi:10.4049/jimmunol.165.5.2518. PMID 10946278. ... Complement component 4 Complement component 4A HLA A1-B8-DR3-DQ2 haplotype Complement system Complement deficiency ... Complement component 4B (Chido blood group) is a protein that in humans is encoded by the C4B gene. This gene encodes the basic ... "Entrez Gene: Complement component 4B (Chido blood group)". Retrieved 2012-01-27. CS1 maint: discouraged parameter (link) Yu CY ...
C1s cleaves C4 and C2, which eventually leads to the production of the classical pathway C3-convertase. C1q - another part of ... Complement component 1s (EC 3.4.21.42, C1 esterase, activated complement C1s, complement C overbar 1r, C1s) is a protein ... "Entrez Gene: C1S Complement component 1, s subcomponent". Luo C, Thielens NM, Gagnon J, Gal P, Sarvari M, Tseng Y, Tosi M, ... Complement+C1s at the US National Library of Medicine Medical Subject Headings (MeSH) Human C1S genome location and C1S gene ...
Arribas J, Arizti P, Castaño JG (1994). "Antibodies against the C2 COOH-terminal region discriminate the active and latent ... Complement component 5 is involved in the complement system. It is cleaved into C5a and C5b: C5a plays an important role in ... Complement component 5 is the fifth component of complement, which plays an important role in inflammatory and cell killing ... 2008). "Structure of and influence of a tick complement inhibitor on human complement component 5". Nat Immunol. 9 (7): 753-60 ...
... involved in complement activation but may play a role as an amplifier of complement activation by cleaving complement C2 or by ... MASP-1 is involved in the lectin pathway of the complement system and is responsible for cleaving C4 and C2 into fragments to ... MASP-1 is a serine protease that functions as a component of the lectin pathway of complement activation. The complement ... Takada F, Takayama Y, Hatsuse H, Kawakami M (Oct 1993). "A new member of the C1s family of complement proteins found in a ...
One form of C3-convertase, also known as C4b2a, is formed by a heterodimer of activated forms of C4 and C2. It catalyzes the ... Complement component 3, often simply called C3, is a protein of the immune system. It plays a central role in the complement ... "Entrez Gene: C3 complement component 3". Sahu A, Lambris JD (Apr 2001). "Structure and biology of complement protein C3, a ... In the alternative complement pathway, C3 is cleaved by C3bBb, another form of C3-convertase composed of activated forms of C3 ...
Zipfel, P. F., Hallström, T., & Riesbeck, K. (2013). Human complement control and complement evasion by pathogenic microbes- ... C1r ja C1s-i aktivatsioonile koos C1s esteraasi formuleerumisega järgneb C4 ja C2 lõhustumine, mis vabastab väikseid peptiide ... 1,0 1,1 1,2 1,3 Rus, H., Cudrici, C., & Niculescu, F. (2005). The role of the complement system in innate immunity. Immunologic ... 7,0 7,1 Lambris, J. D., Ricklin, D., & Geisbrecht, B. V. (2008). Complement evasion by human pathogens. Nature Reviews. ...
Complement deficiencies are the result of a lack of any of these proteins. They may predispose to infections but also to ... C2 deficiency (lupus-like syndrome, vasculitis, polymyositis, pyogenic infections) C3 deficiency (recurrent pyogenic infections ... The complement system is part of the innate as well as the adaptive immune system; it is a group of circulating proteins that ... MASP2 deficiency Complement receptor 3 (CR3) deficiency Membrane cofactor protein (CD46) deficiency Membrane attack complex ...
Complement:. Varied by design type. The Type C4-class ship were the largest cargo ships built by the United States Maritime ... Type C2 ship. *Type C3 ship. *T2 tanker. *Liberty ship. *Victory ship ...
FC(F)(F)c1cc(cc(c1)C(F)(F)F)CN(C(=O)OC)[[email protected]@H]3c2c(ccc(c2)C(F)(F)F)N(C(=O)OCC)[[email protected]@H](C3)CC ... Drugs that interfere with the action of these peptides would aid in lowering cholesterol levels by complementing the action of ...
Corrocher R, Tedesco F, Rabusin P, De Sandre G (1975). "Effect of human erythrocyte stromata on complement activation". Br. J. ...
C-2 Greyhound of the US Navy catches the wire aboard Charles de Gaulle in 2002. ... The ship carries a complement of Dassault Rafale M and E‑2C Hawkeye aircraft, EC725 Caracal and AS532 Cougar helicopters for ... In event of war CdG is expected to operate close to full complement of 40 aircraft. CdG air group took part in combat ... US Navy F-18 Hornets and C-2 Greyhounds conduct regularly qualification traps and launches from CdG. In June 2011 two US Navy C ...
It was the first C-2 loss since 2005,[79] and the first fatal crash since 1973. Eight of the 11 were rescued.[80][81][82][83][ ... Complement:. *Ship's company: 3,532. *Air wing: 2,480. Sensors and processing systems:. *SPS-48E 3-D air search radar ... "US Navy C-2 Greyhound crashes in Philippine Sea". Flight Global. 22 November 2017. Archived from the original on 22 November ...
Here, for consistency, we shall call all large fragments of complement b, so the larger fragment of C2 will be designated C2b. ... complement factor B, and complement factor I, as well as deletion of complement factor H-related 3 and complement factor H- ... Complement deficiencyEdit. Main article: Complement deficiency. It is thought that the complement system might play a role in ... Three biochemical pathways activate the complement system: the classical complement pathway, the alternative complement pathway ...
... rear wheelarch flares complemented the HF-derived wide front wings and bumper, sported by all HPE versions. This meant the HPE ... Fiat C2. Related. Fiat Bravo (2007). Powertrain. Engine. *1.4 L Fire I4 (turbocharged petrol) ... a sportier front bumper complementing 2.8 cm (1.1 in) wider front wings, black side skirts, specific 15 inch 7-spoke alloy ...
Media related to Complement membrane attack complex at Wikimedia Commons. *Complement+Membrane+Attack+Complex at the US ... The membrane attack complex (MAC) or terminal complement complex (TCC) is a structure typically formed on the surface of ... Reid K. B. M., The complement system, in: B. D. Hames and D. M. Glover (eds.), Molecular Immunology, Oxford: IRL Press, 1988, ... MAC is composed of a complex of four complement proteins (C5b, C6, C7, and C8) that bind to the outer surface of the plasma ...
1 is C♯, 2 is D♮ and so on up to 11, which is B♮. The C above this is not 12, but 0 again (12 − 12 = 0). Thus arithmetic modulo ...
The radars, C2 centres, TEL's and missiles will be codeveloped by Israel and India. In turn, IAI and its Israeli partners have ... The MFCR is an active phased array radar and complements the Swordfish Long Range Tracking Radar, for intercepting ballistic ... Along with the Akash SAM, the LRSAM fills a longer range requirement and both types will complement each other. Each unit of ... The Nag will complement the existing Russian 9M113 Konkurs Anti-tank guided missile and European missile MILAN in Indian usage ...
During and after the war, the very light, ​1⁄4-ton jeeps were complemented by the ​3⁄4-ton Dodge WC and Korea War M37 models. ... C2. *AN/USQ-163 Falconer. *AN/GSQ-272 Sentinel. Ground-based. radar. *AN/FPQ-16 Perimeter Acquisition Attack Characterization ...
C1QL1: encoding protein complement component 1, q subcomponent-like 1. *CCDC144A: encoding protein Coiled-coil domain- ...
replace or complement in some plant taxa the starch as storage carbohydrate ...
Sengeløv H (1996). "Complement receptors in neutrophils.". Crit. Rev. Immunol. 15 (2): 107-31. PMID 8573284. ... C: C1Q/C1R/C1S - C4 (C4a) - C2. L: MASP1/MASP2 - MBL. A: Faktor B - Faktor D - Faktor P/Properdin ... "Function, structure and therapeutic potential of complement C5a receptors". British Journal of Pharmacology 152 (4): 429-48. ...
Proteîna C1 a çalak, proteîna C2 û C4 ê çalak dike. C2 û C4 ên çalak yek dibin û proteîna C3yê çalak dikin. C3 ya çalak jî C5ê ... a b https://www.britannica.com/science/complement-immune-system-component *↑ a b c d e f g h i OpenStax, Microbiology, openstax ... Sîstema temamker an jî sîstema tewawker (bi îngilîzî: complement system)[1] beşek ji sîstema bergiriya giştî ye. Sîstema ...
... there are several different kinds of regulatory proteins that disrupt the complement activation process: *Complement Receptor 1 ... Complement Factor H can inhibit the formation of the C3 convertase by competing with factor B for binding to C3b;[1] accelerate ... "Inhibition of the alternative complement pathway by antisense oligonucleotides targeting complement factor B improves lupus ... CFHR5 (Complement Factor H-Related protein 5) is able to bind to act as a cofactor for factor I, has decay accelerating ...
The human basigin protein contains 269 amino acids that form two heavily glycosylated C2 type immunoglobulin-like domains at ...
Tschopp J, Masson D, Stanley KK (1986). "Structural/functional similarity between proteins involved in complement- and ... autosómica recesiva causada pola substitución de dous dos aminoácidos situados no dominio C2: T435M. Anteriormente, críase que ... "Functional assessment of perforin C2 domain mutations illustrates the critical role for calcium-dependent lipid binding in ...
Within the Czech Republic, Common Czech is represented by dark yellow (C1) and Moravian dialects by medium yellow (C2) and ... Where budu has a noun or adjective complement it means "I will be", for example, budu šťastný (I will be happy).[75] Some verbs ... Additionally prepositions and some verbs require their complements to be declined in a certain case.[57] The locative case is ...
... na C4b pa se veže molekula C2, kar odkrije mesto za delovanje C1s, ki razcepi C2 na C2a in C2b. Fragmenta C2b ter C4b se vežeta ... Označujemo jih z veliko črko C (kratica za complement) in številko. Ko signal sproži spremembo enega od začetnih proencimov v ... MBP). Ta ob vezavi aktivira serinsko proteazo MASP (MBP-associated serine protease), ki deluje na beljakovini C2 in C4, ti dve ... ta je homologen C2), pri čemer se ji spremeni konformacija in postane dovzetna za razcep z proteazo faktor D, ki se nahaja v ...
... complement receptors, Fc receptors, B cell receptors and T cell receptors.[12] ...
In 1985, Fripp began using a tuning he called "New Standard tuning"[42] (C2-G2-D3-A3-E4-G4), which would also become ... With the more pop-inspired Belew as main songwriter (complementing Fripp as main instrumental composer) the band took on a new ...
Decay accelerating factor (DAF/CD55), a regulatory protein of the complement cascade, interacts with the first and second EGF- ... 8 C2,8 40.22 cM. Start. 83,723,251 bp[2]. End. 83,741,326 bp[2]. ...
Atazanavir was marketed in 2003[18] and is an azapeptide protease inhibitor[18] designed to fit the C2-symmetry of the enzyme ... The potency of inhibitors bearing hydrophobic groups complementing these areas is therefore increased.[29] Some residues in the ... Ritonavir, a peptidomimetic HIV protease inhibitor, was marketed in 1996.[18] It was designed to fit the C2-symmetry in the ... The HIV protease is a C2-symmetric homodimeric enzyme consisting of two 99 amino acid monomers. Each monomer contributes an ...
Complement B/C2 (IPR011360). Short name: Compl_C2_B Overlapping homologous superfamilies *von Willebrand factor A-like domain ... This family contains two mammalian proteins, complement C2 and complement factor B, which, respectively, have analogous roles ... in non-mammalian species are often more or less equally related to mammalian C2 and B and may be designated as complement B/C2 ... New structural motifs on the chymotrypsin fold and their potential roles in complement factor B.. EMBO J. 19 164-73 2000 ...
ELISA for the measurement of Human Complement C2 in Human Cell culture supernatant, Saliva, Milk, Serum, Plasma, Cerebral ... Human Complement C2 ELISA Kit is a Sandwich (quantitative) ... Cynomolgus C2 could be detected with complement C2 human ELISA ... Cynomolgus C2 detected with complement C2 human ELISA kit (ab154132). Average Good 4/5 (Ease of Use) ... Defects in C2 are the cause of complement component 2 deficiency (C2D) [MIM:217000]. A deficiency of the complement classical ...
C2a, a serine protease, then combines with complement factor C4b to generate the C3 or C5 convertase. ... Component C2 which is part of the classical pathway of the complement system is cleaved by activated factor C1 into two ... IPR011360 Compl_C2_B. IPR037568 Complement_C2. IPR009003 Peptidase_S1_PA. IPR001314 Peptidase_S1A. IPR035976 Sushi/SCR/CCP_sf. ... IPR011360 Compl_C2_B. IPR037568 Complement_C2. IPR009003 Peptidase_S1_PA. IPR001314 Peptidase_S1A. IPR035976 Sushi/SCR/CCP_sf. ...
Mannan-binding lectin activates C3 and the alternative complement pathway without involvement of C2.. Selander B1, Mårtensson U ... MBL-dependent C2 bypass activation could be particularly important in various inherited and acquired complement deficiency ... The serum was reconstituted with MBL (2 mg/l) and C2 (6.5 mg/l) as indicated. PNHS was used as a control. MBL-dependent C2 ... Mannan-binding lectin activates C3 and the alternative complement pathway without involvement of C2 ...
... complement component 2) for ICC/IF, WB. Anti-Complement C2 pAb (GTX105404) is tested in Human samples. 100% Ab-Assurance. ... CO2 antibody, DKFZp779M0311 antibody, C2 antibody, complement C2 antibody, complement component C2 antibody, C3/C5 convertase ... C2. 717. Q5JP69, B4DV20, P06681. 217000, 603075. NP_001269386. 56.47. Homo sapiens. C2. 717. Q5JP69, B4DV20, P06681. 217000, ... Activated C1 cleaves C2 into C2a and C2b. The serine proteinase C2a then combines with complement factor 4b to create the C3 or ...
Adolescence, C2, Cochlear implantation, Complement deficiency, Immunological, Meningitis. in International Journal of Pediatric ... Delayed cochlear implantation in post-meningitic deafness and hereditary complement C2 deficiency. Di Berardino, Federica ; ... A possible immunodeficiency was investigated, revealing hereditary complement C2 deficiency (C2D). Given the insufficient ... A possible immunodeficiency was investigated, revealing hereditary complement C2 deficiency (C2D). Given the insufficient ...
Effects of vitamin D3 and IFN-gamma on the synthesis of the second complement component, C2, by a human myeloid leukemia (HL-60 ... Effects of vitamin D3 and IFN-gamma on the synthesis of the second complement component, C2, by a human myeloid leukemia (HL-60 ... Effects of vitamin D3 and IFN-gamma on the synthesis of the second complement component, C2, by a human myeloid leukemia (HL-60 ... Effects of vitamin D3 and IFN-gamma on the synthesis of the second complement component, C2, by a human myeloid leukemia (HL-60 ...
Complement C2 antibody LS-C722609 is a PE-conjugated rabbit polyclonal antibody to human Complement C2. Validated for IHC and ... Complement C2 antibody LS-C722609 is a PE-conjugated rabbit polyclonal antibody to human Complement C2. Validated for IHC and ... Complement C2 antibody LS-C722609 is a PE-conjugated rabbit polyclonal antibody to human Complement C2. Validated for IHC and ... Complement C2 antibody was raised against recombinant C2 (Ile245-His451) expressed in E. coli. ...
Complement C2 antibody LS-C442556 is an FITC-conjugated rabbit polyclonal antibody to Complement C2 from human, mouse, rat and ... Complement C2 antibody LS-C442556 is an FITC-conjugated rabbit polyclonal antibody to Complement C2 from human, mouse, rat and ... Complement C2 antibody LS-C442556 is an FITC-conjugated rabbit polyclonal antibody to Complement C2 from human, mouse, rat and ... Complement C2 antibody was raised against synthetic peptide located between aa201-250 of human C2 (P06681, NP_000054). Percent ...
C2), transcript variant 1 as transfection-ready DNA - 10 µg - OriGene - cdna clones ... Myc-DDK-tagged ORF clone of Homo sapiens complement component 2 ( ... Myc-DDK-tagged ORF clone of Homo sapiens complement component 2 ... home , products , origene , myc-ddk-tagged orf clone of homo sapiens complement component 2 (c2), transcript variant 1 as ... TrueORF Myc-DDK-tagged ORF clone of Homo sapiens complement component 2 (C2), transcript variant 1 as transfection-ready DNA ...
Association study of complement C2, C3 and factor B, and age-related macular degeneration in a Greek population ... Susceptibility to advanced age-related macular degeneration and alleles of complement factor H, complement factor B, complement ... Association study of complement factor H, C2, CFB, and C3 and age-related macular degeneration in a Han Chinese population. ... Complement C3, C2, and factor B gene polymorphisms and age-related macular degeneration in a Greek cohort study. European ...
C2 : Patient Preparation: Fasting preferred but not required Supplies: Aliquot Tube, 5 mL (T465) Collection Container/Tube: Red ... If the C2 result is less than 15 U/mL, then complement C3 and C4 will be performed at an additional charge. ... This test is for assessment of complement C2 and includes assessment of C3 and C4 as reflex testing. Unless a deficiency has ... A deficiency of an individual component of the complement cascade will result in an undetectable total complement level. ...
Test Code C2AG C2 Complement, Antigen, Serum Reporting Name. C2 Complement, Antigen, S ... Although rare, C2 deficiency is the most common inherited complement deficiency. Homozygous C2 deficiency has an estimated ... The total complement assay (COM / Complement, Total, Serum) should be used as a screen for suspected complement deficiencies ... Low C2 levels in the presence of low C3 and C4 values are consistent with a complement-consumptive process. ...
The results of this study indicated that rSjTOR-ed1 could inhibit complement hemolysis and bind to complement C2, and it is a ... Haemolytic assays and ELISA revealed that rSjTOR-ed1 could inhibit complement hemolysis and bind to complement C2. Purified ... Schistosomes can survive in the host vascular system by immune evasion, regulating the host complement cascade. Schistosoma ... japonicum tetraspanning orphan receptor (SjTOR) is a complement regulator, which is a tegument membrane protein. To date there ...
... hemolytic complement (CH50), with reflex testing to C3 and C4, if appropriate ... C2 : Investigation of a patient with a low (absent) ... Test ID: C2 C2 Complement, Functional, with Reflex, Serum. ... If the C2 result is less than 15 U/mL, then complement C3 and C4 will be performed at an additional charge. ... Investigation of a patient with a low (absent) hemolytic complement (CH50), with reflex testing to C3 and C4, if appropriate ...
Complement C2, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene ... GeneCards Summary for C2 Gene C2 (Complement C2) is a Protein Coding gene. Diseases associated with C2 include Complement ... View all R&D Systems C2 (Complement Component C2b) Products. *View all R&D Systems C2 (Complement Component C2) Proteins and ... View all 2 R&D Systems C2 (Complement Component C2) Primary Antibodies*Human Complement Component C2 Biotinylated Antibody ( ...
Complement C2 receptor inhibitor trispanning: a novel human complement inhibitory receptor. Authors. Lange, S., Inal, J., Hui ... Complement C2 receptor inhibitor trispanning: a novel human complement inhibitory receptor. The Journal of Immunology. 174 (1 ... Complement C2 receptor inhibitor trispanning: a novel human complement inhibitory receptor. Lange, S., Inal, J., Hui Kwok-M, ... We describe complement C2 receptor inhibitor trispanning (CRIT), a novel human complement regulatory receptor, expressed on ...
Diagnosis of complete deficiency of C2 Lennart ruedsson MD/PhD, Section of Microbiology, Immunology and Glycobiology Dept of ... Defects of the Complement System Defects of the Complement System Missing or reduced elements of the complement system, a ... Levels of complement proteins C2, C3, and C4 were within normal ranges. he serum concentration of C1q was below the detection ... Download "Case Report: Complement and infection susceptibility Diagnosis of complete deficiency of C2" ...
The C2 gene provides instructions for making the complement component 2 protein. Learn about this gene and related health ... Complement component 2 deficiency. At least five mutations in the C2 gene have been found to cause complement component 2 ... Complement component C2, inhibiting a latent serine protease in the classical pathway of complement activation. Biochemistry. ... URL of this page: https://medlineplus.gov/genetics/gene/c2/ C2 gene. complement C2 ...
The complement consists of at least 35 or more plasma proteins and cell surface receptors/regulators, which can be activated by ... The complement consists of at least 35 or more plasma proteins and cell surface receptors/regulators, which can be activated by ... In this context, the complement, as one of the first line of host defense against infection was shown to play an important role ... Nevertheless, the parasite uses a sequence of events in order to escape from complement-mediated lysis. In fact, several T. ...
C2: complement C2. *C3: complement C3. *C8A: complement C8 alpha chain. *C8B: complement C8 beta chain ...
SPAPLUS Complement C2 Kit by Binding Site "Great kit! Does so much more than people & clinicians know in monitoring and ...
SPAPLUS Complement C2 Kit by Binding Site "Great kit! Does so much more than people & clinicians know in monitoring and ...
Complement C2. A. 509. Homo sapiens. Mutation(s): 1 Gene Names: C2, hCG_43694. EC: 3.4.21.43. ... Complement component C2a, the catalytic fragment of C3- and C5-convertase of human complement. *DOI: 10.2210/pdb2ODP/pdb ... Formation of these convertases requires the Mg(2+)-dependent binding of C2 to C4b, and the subsequent cleavage of C2 by C1s or ... Formation of these convertases requires the Mg(2+)-dependent binding of C2 to C4b, and the subsequent cleavage of C2 by C1s or ...
Association Between Complement Factor C2/C3/CFB/CFH Polymorphisms and Age-Related Macular Degeneration: A Meta-Analysis.. Lu F1 ... Several previous studies have assessed the contribution of polymorphisms in genes encoding the complement factors C2/C3/CFB/CFH ... Our findings from this analysis confirmed the protective role of C2/CFB/CFH polymorphisms in the development of AMD, but showed ... We conducted a meta-analysis to systematically review the potential association between complement factor polymorphisms and AMD ...
Expanded complement of Niemann-Pick type C2-like protein genes in Clonorchis sinensis suggests functions beyond sterol binding ... Expanded complement of Niemann-Pick type C2-like protein genes in Clonorchis sinensis suggests functions beyond sterol binding ... Thus, here we curated the NPC2-like protein gene complement in C. sinensis, and predicted their conserved and/or divergent ... previous work indicated that Niemann-pick type C2 (NPC2)-like sterol-binding proteins might be integral in the host-parasite ...
C2 and Factor B, soon after the C2 poly(A) site. A comparison of the C2 termination signal with a functionally similar sequence ... In the case of the C2 termination sequence a protein binds within it, and is partially responsible for the termination effect. ... that transcriptional termination occurs between the closely spaced human complement genes, ... Transcriptional termination between the closely linked human complement genes C2 and factor B: common termination factor for C2 ...
Includes B- (humoral) or T-lymphocyte deficiency; complement deficiencies, particularly C1, C2, C3, and C4 deficiency; and ...
Includes B- (humoral) or T-lymphocyte deficiency; complement deficiencies, particularly C1, C2, C3, and C4 deficiency; and ...
  • Several previous studies have assessed the contribution of polymorphisms in genes encoding the complement factors C2/C3/CFB/CFH with the risk of age-related macular degeneration (AMD), however the results have been inconsistent. (cdc.gov)
  • Transcriptional termination between the closely linked human complement genes C2 and factor B: common termination factor for C2 and c-myc? (ox.ac.uk)
  • We have demonstrated, using a combination of nuclear run-off and poly(A) site competition assays, that transcriptional termination occurs between the closely spaced human complement genes, C2 and Factor B, soon after the C2 poly(A) site. (ox.ac.uk)
  • Several genes not involved in the complement cascade have also been implicated. (medscape.com)
  • [ 21 ] Such rare variants have been described in the complement factor H ( CFH ), complement factor I ( CFI ), complement factor 9 ( C9 ), and complement factor 3 ( C3) genes. (medscape.com)
  • When a single gene deficiency does cause lupus, it is usually attributed to the complement protein genes C1 , C2 , or C4 . (wikipedia.org)
  • The two genes -- dubbed Factor H and Factor B -- are involved in regulating the alternative complement cascade (ACC), which responds to infection and other triggers by stimulating an inflammatory response, Dr. Allikmets and colleagues noted in the March 6 online issue of Nature Genetics . (medpagetoday.com)
  • Variation in factor B (BF) and complement component 2 (C2) genes is associated with age-related macular degeneration. (medpagetoday.com)
  • The complement protein C4 exists as two isotypes, C4A and C4B, encoded by genes in the major histocompatibility complex (MHC) class III region. (lu.se)
  • To investigate how potentially functional genetic variants are coinherited on each of four common complement factor H ( CFH ) and CFH -related gene haplotypes and to measure expression of these genes in eye and liver tissues. (arvojournals.org)
  • Unless a deficiency has already been identified, initial assessment should begin with the total complement assay (COM / Complement, Total, Serum), which is a screen for suspected complement deficiencies and should be performed before ordering individual complement component assays. (mayocliniclabs.com)
  • The total complement assay (COM / Complement, Total, Serum) should be used as a screen for suspected complement deficiencies before ordering individual complement component assays. (testcatalog.org)
  • Patients with deficiencies of the early complement proteins are unable to clear immune complexes or to generate lytic activity. (testcatalog.org)
  • In addition, low or undetectable levels of complement may be due to acquired deficiencies or complement consumption (eg, as a consequence of infectious or autoimmune processes). (testcatalog.org)
  • he symptoms of increased susceptibility for infections may have many different causes and among these are complement deficiencies. (docplayer.net)
  • Thus, clinical indications for possible complete complement deficiencies include, among others, recurrent mild or serious bacterial infections as well as autoimmune diseases (AID). (frontiersin.org)
  • Therefore, complement deficiencies disorders impair the immune system's ability to defend the body against foreign or abnormal cells that invade or attack it ( 3 - 5 ). (frontiersin.org)
  • Complement tests, most commonly C3 and C4, are used to determine whether deficiencies or abnormalities in the complement system are causing, or contributing to, a person's disease or condition. (labcorp.com)
  • Other complement components are ordered as needed to look for deficiencies. (labcorp.com)
  • Also found with Sjögren syndrome , rheumatoid arthritis , inherited complement deficiencies (esp C2 deficiency), scleroderma , Celiac disease . (rug.nl)
  • There are deficiencies of each of the individual components of complement. (primaryimmune.org)
  • Patients with complement deficiencies encounter clinical problems that depend on the role of the specific complement protein in normal function. (primaryimmune.org)
  • Congenital complement C1q, C2 or C4 deficiencies are associated with increased risk of vasculitis. (oncologynurseadvisor.com)
  • Abcam's Complement C2 Human in vitro ELISA (Enzyme-Linked Immunosorbent Assay) kit is designed for the quantitative measurement of Complement C2 in CSF, plasma, serum, saliva, and cell culture supernatants. (abcam.com)
  • C3 deposition onto wells coated with O antigen-specific BO, CO, and DO following incubation in PNHS or C2-deficient serum. (nih.gov)
  • Reconstitution of undiluted serum with C1q (70 mg/l, not shown) or D (1 mg/l) together with P (25 mg/l) did not promote C3 deposition, showing that the CO antigen did not activate complement in the absence of recognition proteins. (nih.gov)
  • Component C2 is a serum glycoprotein that functions as part of the classical pathway of the complement system. (genetex.com)
  • Serum complement component C2 binds to the N-terminal extracellular domain 1 of CRIT, which, in peptide form, blocks C3 convertase formation and complement-mediated inflammation. (westminster.ac.uk)
  • The complement system as understood today is a multimolecular system composed of more than 32 proteins and consisting of serum proteins, serosal proteins, and cell membrane receptors that bind to complement fragments. (medscape.com)
  • The complement system consists of 7 serum and 9 membrane regulatory proteins, 1 serosal regulatory protein, and 8 cell membrane receptors that bind complement fragments. (medscape.com)
  • Complement is the term used to describe a group of serum proteins that are critically important in our defense against infection. (primaryimmune.org)
  • HAE is diagnosed by the patient's appearance, family history, blood testing for serum C4 levels, and other complement levels such as C1, C2 and C4. (medicinenet.com)
  • Low serum complement levels can be observed due to an artifact if tests are not performed on fresh sera or sera that are not stored at low temperature. (oncologynurseadvisor.com)
  • Complement was first described as a heat-labile component in serum with bactericidal activity. (springer.com)
  • To date, most, if not all, of the serum proteins that constitute the complement system have been identified and their activities characterized. (springer.com)
  • The complement system is a complex cascade involving proteolytic cleavage of serum glycoproteins often activated by cell receptors. (sigmaaldrich.com)
  • A Complement C2 specific antibody has been precoated onto 96-well plates and blocked. (abcam.com)
  • Standards or test samples are added to the wells and subsequently a Complement C2 specific biotinylated detection antibody is added and then followed by washing with wash buffer. (abcam.com)
  • Immunofluorescence analysis of methanol-fixed A431, using Complement C2(GTX105404) antibody at 1:200 dilution. (genetex.com)
  • Complement C2 antibody LS-C722609 is a PE-conjugated rabbit polyclonal antibody to human Complement C2. (lsbio.com)
  • Complement C2 antibody was raised against recombinant C2 (Ile245-His451) expressed in E. coli. (lsbio.com)
  • The antibody is a rabbit polyclonal antibody raised against C2. (lsbio.com)
  • Complement C2 antibody LS-C442556 is an FITC-conjugated rabbit polyclonal antibody to Complement C2 from human, mouse, rat and other species. (lsbio.com)
  • Complement C2 antibody was raised against synthetic peptide located between aa201-250 of human C2 (P06681, NP_000054). (lsbio.com)
  • ARGX-117 is a next-generation complement-targeting antibody against C2, an important component of both the classical and lectin pathways in the complement cascade. (marketwatch.com)
  • Mouse polyclonal antibody raised against a full-length human C2 protein. (abnova.com)
  • Western Blot analysis of C2 expression in transfected 293T cell line ( H00000717-T01 ) by C2 MaxPab polyclonal antibody. (abnova.com)
  • Mouse monoclonal antibody raised against a partial recombinant C2. (abnova.com)
  • Complement C3b may be an opsonin for antigen-antibody complexes which helps prevent damage from the formation of large, insoluble immune aggregates. (sigmaaldrich.com)
  • Subsequent binding of the antibody to complement C1q subunits of C1 result in catalytically active C1s subunits. (sigmaaldrich.com)
  • Mannan-binding lectin activates C3 and the alternative complement pathway without involvement of C2. (nih.gov)
  • [ 3 ] The existence of multiple, complement-related AMD risk alleles has lent further support to this theory and has shed light on the role of uncontrolled alternative complement pathway activation in this disease. (medscape.com)
  • Most cases are associated with the dysregulation of the alternative complement pathway . (rug.nl)
  • These proteins are composed of three regions, an N-terminal three-module complement control protein domain, a von Willebrand factor A domain, and a C-terminal serine protease domain. (ebi.ac.uk)
  • The structures of the von Willebrand factor A and serine protease domains from human complement factor B ( P00751 ) have been analysed [ PMID: 15016353 , PMID: 10637221 ]. (ebi.ac.uk)
  • C2a, a serine protease, then combines with complement factor 4b to generate the C3 or C5 convertase. (abcam.com)
  • Lectin pathway activation of C3 is known to involve target recognition by mannan-binding lectin (MBL) or ficolins and generation of classical pathway C3 convertase via cleavage of C4 and C2 by MBL-associated serine protease 2 (MASP-2). (nih.gov)
  • Complement component C2, inhibiting a latent serine protease in the classical pathway of complement activation. (medlineplus.gov)
  • The multi-domain serine protease C2 provides the catalytic activity for the C3 and C5- convertases of the classical and lectin pathways of complement activation. (rcsb.org)
  • The protein encoded by this gene is part of the classical pathway of the complement system, acting as a multi-domain serine protease. (wikipedia.org)
  • Complement protein C1r is a serine protease that combines with C1q and C1s to form C1, the first component of the classical pathway of the complement system. (quidel.com)
  • Unlike C1 inhibitor, which inhibits the cleavage of C4 and C2, CRIT only blocks C2 cleavage but, in so doing, shares with C1 inhibitor the same functional effect, of preventing classical pathway C3 convertase formation. (westminster.ac.uk)
  • It is thought that cleavage of C2 by C1s, while bound to C4b, results in a conformational rotation of C2b whereas the released C2a fragment may retain most of its original structure. (wikipedia.org)
  • In the alternate pathway complement C3 undergoes spontaneous cleavage resulting in complement B binding to C3b. (sigmaaldrich.com)
  • Haemolytic assays and ELISA revealed that rSjTOR-ed1 could inhibit complement hemolysis and bind to complement C2. (biomedcentral.com)
  • The Tricarboxylic Acid Cycle Pathway complements our catalog of research reagents including antibodies and ELISA kits against ME1, ME2, ME3, CS, FH. (novusbio.com)
  • The Metabolic Process Pathway complements our catalog of research reagents including antibodies and ELISA kits against INS, C2, CAT, IGF1, CYP3A4. (novusbio.com)
  • 26 Complement Factor B (CFB) ELISA Kits from 8 manufacturers are available on www.antibodies-online.com. (antibodies-online.com)
  • Enzyme-linked immunosorbent assay (ELISA) for complement components, cytokine IL-12 and western blot for C3 activation were performed on CSF and plasma samples. (biomedcentral.com)
  • We investigated C3 activation in C2-deficient human sera and in sera with other defined defects of complement to assess other mechanisms through which MBL might recruit complement. (nih.gov)
  • Recombinant protein encompassing a sequence within the center region of human Complement C2. (genetex.com)
  • Effects of vitamin D3 and IFN-gamma on the synthesis of the second complement component, C2, by a human myeloid leukemia (HL-60) cell line. (jimmunol.org)
  • We describe complement C2 receptor inhibitor trispanning (CRIT), a novel human complement regulatory receptor, expressed on hemopoietic cells and a wide range of tissues throughout the body. (westminster.ac.uk)
  • A comparison of the C2 termination signal with a functionally similar sequence downstream of the human alpha 2 globin gene reveals that both signals function in an orientation dependent manner, with subfragments of the whole signal displaying partial effects. (ox.ac.uk)
  • Classical Complement Pathway Component C1q: Purification of Human C1q, Isolation of C1q Collagen-Like and Globular Head Fragments and Production of Recombinant C1q-Derivatives. (springer.com)
  • Type I human complement C2 deficiency. (wikipedia.org)
  • Primary structure of human complement component C2. (wikipedia.org)
  • Alternatively spliced transcripts of the human complement C2 gene. (semanticscholar.org)
  • The solution structure of the 16th CCP module from human complement factor H has been determined by a combination of 2-dimensional nuclear magnetic resonance spectroscopy and restrained simulated annealing. (embl-heidelberg.de)
  • The secondary structure of the von Willebrand factor type A domain in factor B of human complement by Fourier transform infrared spectroscopy. (ebi.ac.uk)
  • Inhibition of human complement by beta-glycyrrhetinic acid. (nih.gov)
  • To determine whether interference with complement functions may contribute to the immunomodulatory activity of beta-glycyrrhetinic acid, its effects on the classical and alternative activation pathways of human complement were investigated. (nih.gov)
  • It was also established that naturally occurring steroids, e.g. hydrocortisone and cortisone, did not inhibit human complement activity under similar conditions. (nih.gov)
  • Full length native complement component C2 protein isolated from human plasma. (abcam.com)
  • Borsos, T., Rapp, H. J.: Immune hemolysis: A simplified method for the preparation of EAC'4 with guinea pig or with human complement. (springer.com)
  • Quidel's purified human complement components are useful in a wide variety of immunochemical and research applications. (quidel.com)
  • C2 (AAH29781, 20 a.a. ~ 328 a.a) full-length human protein. (abnova.com)
  • The purpose of this study was to evaluate the baseline complement component profiles in human cerebrospinal fluid (CSF) and plasma from non-HIV patients with CM, and therefore to provide insights of possible roles of the complement system in CM. (biomedcentral.com)
  • Investigations from animal model and human patients have shown the importance of the complement system against cryptococcal infections [ 10 ]. (biomedcentral.com)
  • Binding sites for transcription factors, for example, a MAZ binding site downstream of the human complement C2 gene ( 6 ), also have pausing effects which lead to termination, although this is by no means ubiquitous among all protein binding sites. (asm.org)
  • Ten years have passed since the association between variation in the human complement factor H ( CFH ) gene and risk of age-related macular degeneration (AMD) was discovered, 1 - 3 yet this knowledge has not yet been translated into preventive measures for the aging population or treatments for patients with AMD. (arvojournals.org)
  • Defects in C2 are the cause of complement component 2 deficiency (C2D) [MIM:217000]. (abcam.com)
  • Patients with complement component 2 deficiency are also reported to have recurrent or invasive infections. (abcam.com)
  • At least five mutations in the C2 gene have been found to cause complement component 2 deficiency. (medlineplus.gov)
  • More than 90 percent of people with complement component 2 deficiency have a mutation that deletes 28 DNA building blocks (nucleotides) from the C2 gene. (medlineplus.gov)
  • It is unclear how complement component 2 deficiency leads to increased susceptibility to autoimmune disorders. (medlineplus.gov)
  • It is likely that other factors, both genetic and environmental, play a role in the variability of the signs and symptoms of complement component 2 deficiency. (medlineplus.gov)
  • A deficiency of an individual component of the complement cascade will result in an undetectable total complement level. (mayocliniclabs.com)
  • The end result of the complement activation cascade is the formation of the lytic membrane attack complex (MAC). (testcatalog.org)
  • The absence of early components (C1, C2, C4, C3) of the complement cascade results in the inability of immune complexes to activate the cascade. (testcatalog.org)
  • Schistosomes can survive in the host vascular system by immune evasion, regulating the host complement cascade. (biomedcentral.com)
  • The complement is composed of a complex proteolytic cascade of more than 40 soluble and membranous proteins that interact in a various range of function. (frontiersin.org)
  • Inactive circulating complement proteins can be rapidly activated in a cascade fashion by three different activation pathways leading to a common terminal pathway. (frontiersin.org)
  • Basic and clinical studies suggest that activation of complements in the common cascade, for example, complement component 3 (C3) and C5, is involved in the development of septic shock. (hindawi.com)
  • Different initiators activate each pathway but all converge to complement protein C3 and are followed by a common cascade (C5-9), resulting in the deposition of a membrane-attack-complex on targets and the release of chemoattractants (C3a and C5a) for inflammatory cells. (hindawi.com)
  • The complement system functions as an interactive sequence, with one reaction leading to another in the form of a cascade. (medscape.com)
  • Ischaemic infarction may also cause initiation of the complement cascade. (sigmaaldrich.com)
  • The activation process results in the generation of peptides that are chemotactic for neutrophils and that bind to immune complexes and complement receptors. (testcatalog.org)
  • The complement consists of at least 35 or more plasma proteins and cell surface receptors/regulators, which can be activated by three pathways: classical (CP), lectin (LP), and alternative (AP). (frontiersin.org)
  • In this chapter, the structure and function of the complement receptors on the macrophage and related cells are described. (springer.com)
  • The structure of C2b, a fragment of complement component C2 produced during C3 convertase formation. (medlineplus.gov)
  • An inherited complement component deficiency will result in an undetectable complement level. (testcatalog.org)
  • Homozygous C2 deficiency has an estimated prevalence ranging from 1 in 10,000 to 1 in 40,000 (the prevalence of heterozygotes is 1:100 to 1:50). (testcatalog.org)
  • However, discoid lupus erythematosus or systemic lupus erythematosus (SLE) occurs in approximately one-third of patients with homozygous C2 deficiency. (testcatalog.org)
  • Of particular interest was the finding of a great-aunt who also had homozygous C2 deficiency. (annals.org)
  • When a foreign invader is detected, the complement pathway is turned on (activated) and the complement component 2 protein attaches (binds) to a similar protein called complement component 4. (medlineplus.gov)
  • In the case of the C2 termination sequence a protein binds within it, and is partially responsible for the termination effect. (ox.ac.uk)
  • Component C2 which is part of the classical pathway of the complement system is cleaved by activated factor C1 into two fragments: C2b and C2a. (abcam.com)
  • Complement proteins are components of the innate immune system. (testcatalog.org)
  • The classic pathway of the complement system is composed of a series of proteins that are activated in response to the presence of immune complexes. (testcatalog.org)
  • The complement system presents a powerful defense against infection and is tightly regulated to prevent damage to self by functionally equivalent soluble and membrane regulators. (westminster.ac.uk)
  • valuation and Diagnosis Analysis of complement function with the Complement system screen ieslab showed no activity for the classical pathway and the lectin pathway but normal activity for the alternative pathway. (docplayer.net)
  • urther analysis of the immune system included complement and immunoglobulins. (docplayer.net)
  • valuation and Diagnosis Analysis of the immune system was performed including immunoglobulins and complement. (docplayer.net)
  • This protein helps regulate a part of the body's immune response known as the complement system. (medlineplus.gov)
  • The complement system is a group of proteins that work together to destroy foreign invaders (such as bacteria and viruses), trigger inflammation, and remove debris from cells and tissues. (medlineplus.gov)
  • Together, these proteins form a complex called C3 convertase, which triggers further activation of the pathway, allowing the proteins of the complement system to participate in an immune response. (medlineplus.gov)
  • Without this protein to form C3 convertase, activation of the complement system is stalled. (medlineplus.gov)
  • Researchers speculate that the dysfunctional complement system is unable to distinguish what it should attack, and it sometimes attacks normal tissues, leading to autoimmunity. (medlineplus.gov)
  • Alternatively, the dysfunctional complement system may perform partial attacks on invading molecules, which leaves behind foreign fragments that are difficult to distinguish from the body's tissues, so the complement system sometimes attacks the body's own cells. (medlineplus.gov)
  • The complement system is crucial for defense against pathogens and the removal of dying cells or immune complexes. (frontiersin.org)
  • The complement system is a key component of innate immunity and contributes to the elimination of pathogens, dying host cells and abnormal molecular structures ( 1 , 2 ). (frontiersin.org)
  • While complement is an important defense system against bacterial infection, earlier clinical observations suggest that activation of complement factors is associated with detrimental effects in septic shock, such as multiorgan damages and poor outcome [ 5 - 8 ]. (hindawi.com)
  • There are three pathways in the complement system: classical, alternative, and lectin. (hindawi.com)
  • Rare genetic variants in the complement system have also been found to play an important role in AMD. (medscape.com)
  • Evaluation of his complement system showed an absence of functional and antigenic C7, compatible with a complete deficiency of the seventh component of complement. (annals.org)
  • The purple sea urchin, Strongylocentrotus purpuratus, possesses a non-adaptive immune system including elements homologous to C3 and factor B (Bf) of the vertebrate complement system. (semanticscholar.org)
  • Coelomocytes express SpBf, a homologue of factor B, the second component in the sea urchin complement system. (semanticscholar.org)
  • Echinoderm immunity and the evolution of the complement system. (semanticscholar.org)
  • The ancestral complement system in sea urchins. (semanticscholar.org)
  • The complement control protein (CCP) modules (also known as short consensus repeats SCRs or SUSHI repeats) contain approximately 60 amino acid residues and have been identified in several proteins of the complement system. (embl-heidelberg.de)
  • These modules have been identified more than 140 times in over 20 proteins, including 12 proteins of the complement system. (embl-heidelberg.de)
  • It may also be ordered when a health practitioner suspects that someone may have an immune complex-related condition and wants to check the status of the person's complement system. (labcorp.com)
  • In the case of Factor B, a third gene -- involved in control of another part of the immune system, the classical complement pathway -- also seems to be involved. (medpagetoday.com)
  • The complement system consists of more than 30 proteins, present in blood and tissues, as well as other proteins anchored on the surfaces of cells. (primaryimmune.org)
  • The primary functions of the complement system are to protect from infection, to remove particulate substances, (like damaged or dying cells, microbes or immune complexes) and to help modulate adaptive immune responses. (primaryimmune.org)
  • As part of the innate immune system, complement acts immediately to start the process of removal and resolution of the problem. (primaryimmune.org)
  • Complement works with the inflammatory cells of the innate immune system and those of adaptive or acquired immunity. (primaryimmune.org)
  • A unique feature of the AP is the presence of the only positive regulator in the complement system, Properdin. (primaryimmune.org)
  • Götze, O., Müller-Eberhard, H.-J.: The C3-activator system: an alternate pathway of complement activation. (springer.com)
  • What cleaves the centra protine of the complement system? (brainscape.com)
  • The relative contribution of the complement system in protection and pathogenesis during CM remains largely unknown. (biomedcentral.com)
  • The activity of the complement system in CSF was increased in non-HIV patients with CM. C1q, MBL and FB are the important participants in the complement activation in CM. The relative contribution of each of the specific complement pathways and complement cascades in protection and inflammation resolution against CM warrant further investigation. (biomedcentral.com)
  • The complement system is well known as a major component of the host innate immune defense system against infection. (biomedcentral.com)
  • Activation of the complement system in response to invading pathogens is initiated through the classical (CP), alternative (AP) and lectin (LP) pathways. (biomedcentral.com)
  • The complement system has the inhibitory proteins to regulate the location and efficacy of complement activation. (biomedcentral.com)
  • It has been suggested that the complement system is of central importance in host defense against bacterial meningitis. (biomedcentral.com)
  • Dysfunction of the complement system (a central part of the innate immune system) is key to the etiology of AMD. (arvojournals.org)
  • 4 The complement system comprises a series of proteins, C1 to C9, which are sequentially activated or inhibited by regulatory molecules, including complement factor H (FH), factor D (FD), and factor I (FI). (arvojournals.org)
  • Mice lacking hemolytic complement activity in their sera were sensitive to C.neoformans [ 11 ]. (biomedcentral.com)
  • A deficiency of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus erythematosus. (abcam.com)
  • These include Common Variable Immune Deficiency (CVID) , Wiskott-Aldrich Syndrome (WAS) , IgA deficiency , Good Syndrome, Hyper IgM Syndrome , Idiopathic T-cell Lymphopenia (ICL) and Complement disorders . (primaryimmune.org)
  • Once activated, several relevant complement functions are generated which include opsonization and phagocytosis of particles or microorganisms and cell lysis. (frontiersin.org)
  • This family contains two mammalian proteins, complement C2 and complement factor B, which, respectively, have analogous roles in the classical and alternative pathways of complement activation. (ebi.ac.uk)
  • Strongylocentrotus purpuratus (Purple sea urchin) has an atypical factor B with a five-module complement control protein domain. (ebi.ac.uk)
  • Crystal structure of the A domain from complement factor B reveals an integrin-like open conformation. (ebi.ac.uk)
  • New structural motifs on the chymotrypsin fold and their potential roles in complement factor B. (ebi.ac.uk)
  • They may also have symptoms that suggest autoimmune disease and complement deficiency may be an etiologic factor in the development of autoimmune disease. (testcatalog.org)
  • Association Between Complement Factor C2/C3/CFB/CFH Polymorphisms and Age-Related Macular Degeneration: A Meta-Analysis. (cdc.gov)
  • We conducted a meta-analysis to systematically review the potential association between complement factor polymorphisms and AMD. (cdc.gov)
  • Our findings from this analysis confirmed the protective role of C2/CFB/CFH polymorphisms in the development of AMD, but showed that the single nucleotide polymorphism in C3 was a high-risk factor for AMD. (cdc.gov)
  • The molecular genetics and polymorphism of C2 and factor B". British Medical Bulletin. (wikipedia.org)
  • The common coding variant Y402H in the complement factor H ( CFH ) gene was the first identified. (medscape.com)
  • Diversity of complement factor B/C2 in the common carp (Cyprinus carpio): three isotypes of B/C2-A expressed in different tissues. (semanticscholar.org)
  • Two diverged complement factor B/C2-like cDNA sequences from a teleost, the common carp (Cyprinus carpio). (semanticscholar.org)
  • Ehrlich and Morgan termed this factor complement. (medscape.com)
  • Binding of factor H to C3b increases its inactivation by factor I. Properdin stabilizes it, preventing its inactivation by factors H and I. The alternate pathway does not result in a truly nonspecific activation of complement because it requires specific types of compounds for activation. (medscape.com)
  • Four genetic biomarkers were examined in MAHALO, including complement factor H (CFH), C3, C2/CFB and CFI. (roche.com)
  • Two variants of the so-called C2 gene are almost always inherited with the protective haplotypes of Factor B. (medpagetoday.com)
  • Key fluid phase complement regulators include Factor H (FH), Factor I (FI), C4-binding protein (C4BP) and C1 inhibitor (C1INH). (biomedcentral.com)
  • In fact, several T. cruzi molecules are known to interfere in the initiation of all three pathways and in the assembly of C3 convertase, a key step in the activation of complement. (frontiersin.org)
  • In this review, we aim to present an overview on the strategies used by T. cruzi in order to circumvent the activation of complement and, consequently, its biological effects. (frontiersin.org)
  • Deficiency of C2 has been reported to associated with certain autoimmune diseases and SNPs in this gene have been associated with altered susceptibility to age-related macular degeneration. (genetex.com)
  • Severe or multiple infections-mainly meningococcal infections-or severe autoimmune diseases (AID) in particular with childhood onset are the main features associated with complement deficiency (CD) ( 6 - 10 ). (frontiersin.org)
  • Deficiency of C2 has been associated with certain autoimmune diseases. (wikipedia.org)
  • Complement testing may be ordered when a person has unexplained inflammation or edema or symptoms of an autoimmune disorder such as SLE. (labcorp.com)
  • Decreased complement levels also are associated with an increased risk of developing an autoimmune disease. (labcorp.com)
  • The C2 deficiency may be a critical defect in the host defenses to infection that predisposed to the development of autoimmune disease. (annals.org)
  • 1 Diagnosis of complete deficiency of C2 Lennart ruedsson MD/PhD, Section of Microbiology, Immunology and Glycobiology Dept of Laboratory Medicine, Lund University, Lund, he patient was a 14-year-old girl who has healthy parents and a healthy 11-year-old sister. (docplayer.net)
  • urther analysis with measurement of individual components in the classical pathway (C1q, C2 and C4) revealed complete deficiency of C2. (docplayer.net)
  • Complete deficiency of C2 as diagnosed here is rare, found in about 1/ individuals. (docplayer.net)
  • 3 Diagnosis of complete deficiency of C2 Lennart ruedsson MD/PhD, Section of Microbiology, Immunology and Glycobiology Dept of Laboratory Medicine, Lund University, Lund, A girl of 8 years of age had a sudden onset of febrile illness with headache and was taken to hospital. (docplayer.net)
  • Complete deficiency of C2 is associated with increased susceptibility for infections with encapsulated bacteria such as pneumococci and also increased risk to develop SL. (docplayer.net)
  • Among its related pathways are Complement and coagulation cascades and Immune response Lectin induced complement pathway . (genecards.org)
  • MBL-dependent C2 bypass activation could be particularly important in various inherited and acquired complement deficiency states. (nih.gov)
  • Isoform 2 may have an inhibitory effect on the activation of the lectin pathway of complement or may cleave IGFBP5 . (rcsb.org)
  • Activated C1 cleaves C2 into C2a and C2b. (genetex.com)
  • This then acts similarly to C1 esterase and cleaves C2 and C4 to form C2aC4b, which is the C3 esterase that cleaves C3 to form C3b. (medscape.com)
  • A complex of complements C5b,C6, C7, and C8 mediates the polymerization of up to eighteen C9 molecules into a tube-like membrane attack complex that is inserted into the plasma membrane of an unwanted organism such as of gram-negative bacteria and viral infected cells. (sigmaaldrich.com)
  • Nevertheless, the parasite uses a sequence of events in order to escape from complement-mediated lysis. (frontiersin.org)
  • The complement control protein (CCP) modules (also known as short consensus repeats) are defined by a consensus sequence within a stretch of about 60 amino acid residues. (embl-heidelberg.de)
  • Here, using direct sequencing analysis of the coding region we report the pathogenic variants spectrum that underlie the total or subtotal complement deficiency in 212 patients. (frontiersin.org)
  • The results of this study indicated that rSjTOR-ed1 could inhibit complement hemolysis and bind to complement C2, and it is a potential vaccine candidate that protects against S. japonicum infection. (biomedcentral.com)
  • Functions in the lectin pathway of complement, which performs a key role in innate immunity by recognizing pathogens through patterns of sugar moieties and neutralizing them. (rcsb.org)
  • Complement activation by CRP may help limit the inflammatory response by providing opsonization with minimal generation of C5a and C5b-9. (nih.gov)
  • Schistosoma japonicum tetraspanning orphan receptor (SjTOR) is a complement regulator, which is a tegument membrane protein. (biomedcentral.com)
  • Ab blockage of cellular CRIT reduces inhibition of cytolysis, indicating that CRIT is a novel complement regulator protecting autologous cells. (westminster.ac.uk)
  • Our data demonstrated that the CSF levels of complement components of C1q, FB, MBL as well as complement pathway factors sC5b-9 and complement regulator FH were all elevated in patients with CM as compared to the controls, CSF C3 breakdown products iC3b were found in both CSF and plasma samples of the CM patients. (biomedcentral.com)
  • Complement factors C3a, C5a and C4 can induce vasodilatation, increased capillary permeability, and expression of leukocyte adhesion molecules. (sigmaaldrich.com)
  • Moreover, T. cruzi promotes secretion of plasma membrane-derived vesicles from host cells, which prevent the activity of C3 convertase C4b2a and thereby may hinder complement. (frontiersin.org)
  • Membranoproliferative glomerulonephritis ( MPGN ) is a type of glomerulonephritis caused by deposits in the kidney glomerular mesangium and basement membrane ( GBM ) thickening, [2] activating complement and damaging the glomeruli. (rug.nl)
  • The Terminal Pathway (TP) is the final set of steps in the complement activation process that forms a membrane lesion or hole (membrane attack complex or MAC) that kills susceptible bacteria or other cells that activate complement on their surfaces. (primaryimmune.org)
  • This infers resistance to complement-mediated cell lysis, allowing parasite survival and infection. (genecards.org)
  • In this context, the complement, as one of the first line of host defense against infection was shown to play an important role in recognizing T. cruzi metacyclic trypomastigotes and in controlling parasite invasion. (frontiersin.org)
  • An important step during T. cruzi infection is when intracellular trypomastigotes are release to bloodstream where they may be target by complement. (frontiersin.org)
  • Hereditary C2 deficiency in Sweden: frequent occurrence of invasive infection, atherosclerosis, and rheumatic disease. (medlineplus.gov)
  • Because it shares antigenic determinants with the C2-binding complement component C4 and can act as a decoy C2-binding receptor, TOR can regulate complement activation by interfering with the formation of C3 convertase (C4b2a) [ 9 , 15 , 17 ]. (biomedcentral.com)
  • C2b is the smallest , enzymatically active fragment which is incorporated into the C3 convertase in this pathway, C4b2b (NB: some sources now refer to the larger fragment of C2 as C2b, making the C3 convertase C4b2b, whereas older sources refer to the larger fragment of C2 as C2a, making the C3 convertase C4b2a). (wikipedia.org)
  • The two activated C1s subunits are then able to catalyze the assembly of the C3 convertase (complement C4b2a) from complements C2 and C4. (sigmaaldrich.com)
  • Sera from 3 C2-deficient patients were used in the kinetic experiments. (nih.gov)
  • Patients with SLE and a C2 deficiency frequently have a normal anti-ds DNA titer. (testcatalog.org)
  • 1 Genetic polymorphisms in the complement pathway have been implicated in the development of AMD including GA. In the MAHALO study, 57 percent of genotype samples collected from 93 patients were positive for the CFI biomarker. (roche.com)
  • In the MAHALO study, most patients had a background of CFH and C2/CFB. (roche.com)
  • Functions as an endopeptidase and may activate MASP2 or C2 or directly activate C3 the key component of complement reaction. (rcsb.org)
  • Homologs in non-mammalian species are often more or less equally related to mammalian C2 and B and may be designated as complement B/C2. (ebi.ac.uk)
  • We find that four-cysteine, zinc-bound (C4) and two-cysteine, zinc-free (C2) DksA proteins are able to mediate appropriate stringent control in Salmonella and that thiol-based sensing of reactive species is conserved among C2 and C4 orthologues. (asm.org)
  • This results in inhibition of the classical and lectin pathway of complement activation, probably due to interference with binding of C2a to C4b such that C3 convertase cannot be formed. (genecards.org)
  • The sushi domain is also known as the complement controle protein (CCP) module or the short consensus repeat (SCR). (embl-heidelberg.de)