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.

The N-terminal CUB-epidermal growth factor module pair of human complement protease C1r binds Ca2+ with high affinity and mediates Ca2+-dependent interaction with C1s. (1/112)

The Ca2+-dependent interaction between complement serine proteases C1r and C1s is mediated by their alpha regions, encompassing the major part of their N-terminal CUB-EGF-CUB (where EGF is epidermal growth factor) module array. In order to define the boundaries of the C1r domain(s) responsible for Ca2+ binding and Ca2+-dependent interaction with C1s and to assess the contribution of individual modules to these functions, the CUB, EGF, and CUB-EGF fragments were expressed in eucaryotic systems or synthesized chemically. Gel filtration studies, as well as measurements of intrinsic Tyr fluorescence, provided evidence that the CUB-EGF pair adopts a more compact conformation in the presence of Ca2+. Ca2+-dependent interaction of intact C1r with C1s was studied using surface plasmon resonance spectroscopy, yielding KD values of 10.9-29.7 nM. The C1r CUB-EGF pair bound immobilized C1s with a higher KD (1.5-1.8 microM), which decreased to 31.4 nM when CUB-EGF was used as the immobilized ligand and C1s was free. Half-maximal binding was obtained at comparable Ca2+ concentrations ranging from 5 microM with intact C1r to 10-16 microM for C1ralpha and CUB-EGF. The isolated CUB and EGF fragments or a CUB + EGF mixture did not bind C1s. These data demonstrate that the C1r CUB-EGF module pair (residues 1-175) is the minimal segment required for high affinity Ca2+ binding and Ca2+-dependent interaction with C1s and indicate that Ca2+ binding induces a more compact folding of the CUB-EGF pair.  (+info)

Alzheimer's beta-amyloid peptides can activate the early components of complement classical pathway in a C1q-independent manner. (2/112)

beta-Amyloid (beta-A) accumulates in the brain of patients with Alzheimer's disease (AD) and is presumably involved in the pathogenesis of this disease, on account of its neurotoxicity and complement-activating ability. Although assembly of beta-A in particular aggregates seems to be crucial, soluble non-fibrillar beta-A may also be involved. Non-fibrillar beta-A does not bind C1q, so we investigated alternative mechanisms of beta-A-dependent complement activation in vitro. On incubation with normal human plasma, non-fibrillar beta-A 1-42, and truncated peptide 1-28, induced dose-dependent activation of C1s and C4, sparing C3, as assessed by densitometric analysis of immunostained membrane after SDS-PAGE and Western blotting. The mechanism of C4 activation was not dependent on C1q, because non-fibrillar beta-A can still activate C1s and C4 in plasma genetically deficient in C1q (C1qd). In Factor XII-deficient plasma (F.XIId) the amount of cleaved C4 was about 5-10% less that in C1qd and in normal EDTA plasma; the reconstitution of F.XIId plasma with physiologic concentrations of F.XII resulted in an increased (8-15%) beta-A-dependent cleavage of C4. Thus our results indicate that the C1q-independent activation of C1 and C4 can be partially mediated by the activation products of contact system. Since the activation of contact system and of C4 leads to generation of several humoral inflammatory peptides, non-fibrillar beta-A might play a role in initiating the early inflammatory reactions leading to a multistep cascade contributing to neuronal and clinical dysfunction of AD brain.  (+info)

Antibody-independent classical complement pathway activation and homologous C3 deposition in xeroderma pigmentosum cell lines. (3/112)

Of human malignantly transformed cell lines, xeroderma pigmentosum (XP) cell lines were found to be highly susceptible to homologous complement (C): cells were opsonized by C3 fragments on incubation with diluted normal human serum. C3 fragment deposition on XP cells was Ca2+-dependent and occurred on live cells but not UV-irradiated apoptotic cells. (Ca2+ is required for activation of the classical C pathway via C1q and the lactin pathway via mannose binding lectin (MBL), and the surface of apoptotic cells usually activates the alternative C pathway.) In this study we tested which of the pathways participates in XP cell C3 deposition. In seven cell lines that allowed C3 deposition (i), Clq was shown to be essential but MBL played no role in C activation, (ii) Cls but not MASP bound XP cells for activation, (iii) no antibodies recognizing XP cells were required for homologous C3 deposition, and (iv) the alternative pathway barely participated in C3 deposition. Furthermore, the levels of C-regulatory proteins for host cell protection against C, decay-accelerating factor (DAF, CD55) and membrane cofactor protein (MCP, CD46), were found to be relatively low in almost all XP cell lines compared with normal cells. These results indicate that XP cells activate the classical C pathway in an antibody-independent manner through the expression of a molecule which directly attracts C1q in a C-activating form, and that relatively low levels of DAF and MCP on XP cells facilitate effective C3 deposition. The possible relationship between the pathogenesis of XP and our findings is discussed.  (+info)

A novel PCR-based technique using expressed sequence tags and gene homology for murine genetic mapping: localization of the complement genes. (4/112)

The complement system is a cascade of serum proteins and receptors which forms a vital arm of innate immunity and enhances the adaptive immune response. This work establishes the chromosomal localization of four key genes of the murine complement system. Mapping was performed using a novel and rapid PCR restriction length polymorphism method which was developed to exploit the murine expressed sequence tag (EST) database. This technique circumvents the laborious cDNA or genomic cloning steps of other mapping methods by relying on EST data and the prediction of exon-intron boundaries. This method can be easily applied to the genes of other systems, ranging from the interests of the individual researcher to large-scale gene localization projects. Here the complement system, probably one of the most well-characterized areas of immunology, was used as a model system. It was shown that the C3a receptor C1r and C1s genes form an unexpected complement gene cluster towards the telomeric end of chromosome 6. The second mannose binding lectin-associated serine protease gene was mapped to the telomeric end of chromosome 4, which is distinct from other complement-activating serine proteases. These results provide new insights into the evolution of this group of proteins.  (+info)

Crystal structure of the catalytic domain of human complement c1s: a serine protease with a handle. (5/112)

C1s is the highly specific modular serine protease that mediates the proteolytic activity of the C1 complex and thereby triggers activation of the complement cascade. The crystal structure of a catalytic fragment from human C1s comprising the second complement control protein (CCP2) module and the chymotrypsin-like serine protease (SP) domain has been determined and refined to 1.7 A resolution. In the areas surrounding the active site, the SP structure reveals a restricted access to subsidiary substrate binding sites that could be responsible for the narrow specificity of C1s. The ellipsoidal CCP2 module is oriented perpendicularly to the surface of the SP domain. This arrangement is maintained through a rigid module-domain interface involving intertwined proline- and tyrosine-rich polypeptide segments. The relative orientation of SP and CCP2 is consistent with the fact that the latter provides additional substrate recognition sites for the C4 substrate. This structure provides a first example of a CCP-SP assembly that is conserved in diverse extracellular proteins. Its implications in the activation mechanism of C1 are discussed.  (+info)

Interaction of C1q and mannan-binding lectin (MBL) with C1r, C1s, MBL-associated serine proteases 1 and 2, and the MBL-associated protein MAp19. (6/112)

Mannan-binding lectin (MBL) and C1q activate the complement cascade via attached serine proteases. The proteases C1r and C1s were initially discovered in a complex with C1q, whereas the MBL-associated serine proteases 1 and 2 (MASP-1 and -2) were discovered in a complex with MBL. There is controversy as to whether MBL can utilize C1r and C1s or, inversely, whether C1q can utilize MASP-1 and 2. Serum deficient in C1r produced no complement activation in IgG-coated microwells, whereas activation was seen in mannan-coated microwells. In serum, C1r and C1s were found to be associated only with C1q, whereas MASP-1, MASP-2, and a third protein, MAp19 (19-kDa MBL-associated protein), were found to be associated only with MBL. The bulk of MASP-1 and MAp19 was found in association with each other and was not bound to MBL or MASP-2. The interactions of MASP-1, MASP-2, and MAp19 with MBL differ from those of C1r and C1s with C1q in that both high salt concentrations and calcium chelation (EDTA) are required to fully dissociate the MASPs or MAp19 from MBL. In the presence of calcium, most of the MASP-1, MASP-2, and MAp19 emerged on gel-permeation chromatography as large complexes that were not associated with MBL, whereas in the presence of EDTA most of these components formed smaller complexes. Over 95% of the total MASPs and MAp19 found in serum are not complexed with MBL.  (+info)

The cleavage of two C1s subunits by a single active C1r reveals substantial flexibility of the C1s-C1r-C1r-C1s tetramer in the C1 complex. (7/112)

The activation of the C1s-C1r-C1r-C1s tetramer in the C1 complex, which involves the cleavage of an Arg-Ile bond in the catalytic domains of the subcomponents, is a two-step process. First, the autolytic activation of C1r takes place, then activated C1r cleaves zymogen C1s. The Arg463Gln mutant of C1r (C1rQI) is stabilized in the zymogen form. This mutant was used to form a C1q-(C1s-C1rQI-C1r-C1s) heteropentamer to study the relative position of the C1r and C1s subunits in the C1 complex. After triggering the C1 by IgG-Sepharose, both C1s subunits are cleaved by the single proteolytically active C1r subunit in the C1s-C1rQI-C1r-C1s tetramer. This finding indicates that the tetramer is flexible enough to adopt different conformations within the C1 complex during the activation process, enabling the single active C1r to cleave both C1s, the neighboring and the sequentially distant one.  (+info)

The complement component C1s is the protease that accounts for cleavage of insulin-like growth factor-binding protein-5 in fibroblast medium. (8/112)

Cultured fibroblasts secrete an 88-kDa serine protease that cleaves insulin-like growth factor binding protein-5 (IGFBP-5). Because IGFBP-5 has been shown to regulate IGF-I actions, understanding the chemical identity and regulation of this protease is important for understanding how IGF-I stimulates anabolic functions. The protease was purified from human fibroblast-conditioned medium by hydrophobic interaction, lectin affinity, and heparin Sepharose affinity chromatography followed by SDS-polyacrylamide gel electrophoresis. An 88-kDa band was excised and digested with lysyl-endopeptidase. Sequencing of the high pressure liquid chromatography-purified peptides yielded the complement components C1r and C1s. To confirm that C1r/C1s accounted for the proteolytic activity in the medium, immunoaffinity chromatography was performed. Most of the protease activity adhered to the column, and the eluant was fully active in cleaving IGFBP-5. SDS-polyacrylamide gel electrophoresis with silver staining showed two bands, and IGFBP-5 zymography showed a single 88-kDa band. Amino acid sequencing confirmed that the 88-kDa band contained only C1r and C1s. C1r in the fibroblast medium underwent autoactivation, and the activated form cleaved C1s. C1s purified from the conditioned medium cleaved C(4), a naturally occurring substrate. The purified protease cleaved IGFBP-5 but had no activity against IGFBP-1 through -4. C1 inhibitor, a protein known to inhibit activated C1s, was shown to inhibit the cleavage of IGFBP-5 by the protease in the conditioned medium. In summary, human fibroblasts secrete C1r and C1s that actively cleave IGFBP-5. The findings define a mechanism for cleaving IGFBP-5 in the culture medium, thus allowing release of IGF-I to cell surface receptors.  (+info)

The subcomponents C1r and C1s and their activated forms C-1r and C-1s were each found to have mol.wts. in dissociating solvents of about 83000. The amino acid compositions of each were similar, but there were significant differences in the monosaccharide analyses of subcomponents C1r and C1s, whether activated or not. Subcomponents C1r and C1s have only one polypeptide chain, but subcomponents C-1r and C-1s each contain two peptide chains of approx. mol.wts. 56000 (a chain) and 27000 (b chain). The amino acid analyses of the a chains from each activated subcomponent are similar, as are those of the b chains. The N-terminal amino acid sequence of 29 residues of the C-1s a chain was determined, but the C-1r a chain has blocked N-terminal amino acid. The 20 N-terminal residues of both b chains are similar, but not identical, and both show obvious homology with other serine proteinases. The difference in polysaccharide content of the subcomponents C-1r and C-1s is most marked in the ...
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Activation of the C1 complex is under control of the C1-inhibitor. It forms a proteolytically inactive stoichiometric complex with the C1r or C1s proteases. May play a potentially crucial role in regulating important physiological pathways including complement activation, blood coagulation, fibrinolysis and the generation of kinins. Very efficient inhibitor of FXIIa. Inhibits chymotrypsin and kallikrein (By similarity).
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Futaba 2HR ideal for use on Tamiya and other R/C cars, Servo is not included. requires 4 x AA Cell batteries.The new Futaba 2HR radio is designed for those car, boat and glider modellers that need a high quality yet affordable 2 channel 2.4GHz system, and includes steering dual rates, servo reversin ...
Originally Posted by Bishop Things like carbon to replace alloy is one of the few ways you can drop weight, but like everything just because you can
Bu t r yap t r c lar, UV veya g r n r a maruz kald klar nda k sa s rede k rle irler. K rle me h z n iddetine ve dalga boyuna ba l d r. I n yap t r c tabakas na ula abilmesi i in, yap t r lan par alardan en az birinin k ge iren bir malzeme olmas gereklidir. Bu nedenle cam ve effaf plastik t r malzemelerin yap t r lmas nda tercih edilirler.. zel r nler ...
Such are the petty games and I am glad I kept out of the fray not interfering in this at all and lets, time take its course. The old lady had commented on my lack of interest in the matter by not going to her house to ask her to return! I was not party to the incident so why should I interfere, and she is welcome to withhold her employment, and I will be the beneficiary not having to deal with personal vendettas.I only want performance for just pay, and I will not interfere in their lives.I am sure I have not seen the end of this as I am sure this lady who has been at the farm for over 25 years will try her best to get this girl off the place, and will try to influence my father, in the matter.It will be interesting to see the next move, and what sordid story will be cooked up to achieve this end. In the meantime I am trying to write a skit for a comedy, based completely on this story as I only see the comedy unfolding in this otherwise tragic case. Their is a lot more to this story with this ...
Such are the petty games and I am glad I kept out of the fray not interfering in this at all and lets, time take its course. The old lady had commented on my lack of interest in the matter by not going to her house to ask her to return! I was not party to the incident so why should I interfere, and she is welcome to withhold her employment, and I will be the beneficiary not having to deal with personal vendettas.I only want performance for just pay, and I will not interfere in their lives.I am sure I have not seen the end of this as I am sure this lady who has been at the farm for over 25 years will try her best to get this girl off the place, and will try to influence my father, in the matter.It will be interesting to see the next move, and what sordid story will be cooked up to achieve this end. In the meantime I am trying to write a skit for a comedy, based completely on this story as I only see the comedy unfolding in this otherwise tragic case. Their is a lot more to this story with this ...
Os 40 Fs found in: OS ENGINES FS 40S-C 40 Econo Power Manufacturer/Model By Series, OS ENGINES FS 40S-C 40 Ceramic MX Manufacturer/Model By Series, OS..
KOYUNCUOĞLU, MERAL; Görücü, Gözde; SAYGILI, UĞUR; Bilkay Görken, İlknur; Özen, Emek; USLU, TURHAN; Erten, Oktay (Dokuz Eylül Üniversitesi Tıp Fakültesi , 2002) ...
Several of the above proteins consist of a catalytic domain together with several CUB domains interspersed by calcium-binding EGF domains. Some CUB domains appear to be involved in oligomerisation and/or recognition of substrates and binding partners. For example, in the complement proteases, the CUB domains mediate dimerisation and binding to collagen-like regions of target proteins (e.g. C1q for C1r/C1s). The structure of CUB domains consists of a beta-sandwich with a jelly-roll fold. Almost all CUB domains contain four conserved cysteines that probably form two disulphide bridges (C1-C2, C3-C4). The CUB1 domains of C1s and Map19 have calcium-binding sites [ (PUBMED:17446170) ]. ...
Several of the above proteins consist of a catalytic domain together with several CUB domains interspersed by calcium-binding EGF domains. Some CUB domains appear to be involved in oligomerisation and/or recognition of substrates and binding partners. For example, in the complement proteases, the CUB domains mediate dimerisation and binding to collagen-like regions of target proteins (e.g. C1q for C1r/C1s). The structure of CUB domains consists of a beta-sandwich with a jelly-roll fold. Almost all CUB domains contain four conserved cysteines that probably form two disulphide bridges (C1-C2, C3-C4). The CUB1 domains of C1s and Map19 have calcium-binding sites [(PUBMED:17446170)].. ...
Well admit to not having had the best luck with pint-sized R/C choppers, but the Black Stealth 3-Channel R/C Helicopter ($30) is here to change...
Local violinist, composer, and founder of The Happy Maladies Eddy Kwon draws inspiration from Eric K M Clarks Deprivation Music for his work a r c h i p e l a g o. Performed by a string quartet, each musicians score is the exact same piece of music-but with...
It wasnt that long ago since three new PicooZ R/C Helicopters saw the light of day. Now yet another Silverlit micro chopper has been released. Currently touted
A R C H I V E S O F M E T A L L U R G Y A N D M A T E R I A L S Volume Issue 2 DOI: /v G. WNUK EXPERIMENTAL STUDY ON THERMODYNAMICS OF THE Cu-Ni-Sn- LIQUID ALLOYS BADANIA
LiveRC.com features the latest news, pictures, and videos in the radio control car industry. LiveRC.com also features exclusive live audio and video from various races around the world.
Bitkisel ve Hayvansal Yetiştiricilik, Hastalıkları, Zararlıları, Besin Noksanlıkları, Kanunları, Desteklemeleri, Mutfakta ve Sanayide kullanımı, Tarım marketi, Tarım Market
Bitkisel ve Hayvansal Yetiştiricilik, Hastalıkları, Zararlıları, Besin Noksanlıkları, Kanunları, Desteklemeleri, Mutfakta ve Sanayide kullanımı, Tarım marketi, Tarım Market
Tıbbi Biyokimya dalında Doktora öğrencisi Sercan Kapancık doğal bağışıklığın bir parçası olan pozitif yüklü (katyonik) anti-mikrobiyal peptitlerin kanser
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The Hong Kong-based WowWee company has announced the launch of their FlyTech Bladestar, a radio-controilled indoor flying machine that is said to change
Whew, what a day today! I made it to my first competitive cup with my newly acquired HPI RS4 MT and two mates Fantuzzi and Chief.Geronimo in Modified Monster class. We were all quite sure that all effort made will be pointless against those semi-professional racers with better equipment and more skills, but it ended not so bad at all! At the end of the day we were exhausted as if we performed a marathon, but left location with a big big smile in our faces. All hurdles had to be taken from fried escs to completely wrong car setups and hours of disassemble-assemble work, but hey it was worth it: it was tons and tons of fun!! Thats how it ended on paper: 8th (me), 9th (Fantuzzi) and 14th (Chief_Geronimo ...
Lee, S-C., Chen, S-C., Chingtai, T., Chiang, C. E., Wen, Z. C., Chen, Y-J., Yu, W-L., Fong, A. N., Huang, J-S., Cheng, J. J. & Chang, M. S., 九月 1997, 於: American Heart Journal. 134, 3, p. 387-94 8 p.. 研究成果: 雜誌貢獻 › 文章 › 同行評審 ...
Makrohematuriya (sərt) sidiyin rənginin gözlə görünəcək dərəcədə qanla və ya qan laxtası ilə dəyişməsidir. O, qırmızıdan qəhvəyi rəngə qədər sidiklə və ya aşkar qan şəklində təzahür oluna bilər. Cəmi 1 ml qan 1 litr sidiyin rəngini dəyişə bilər. Makrohematuriya hətta müvəqqəti və ya asimptomatik ...
Bioizmir Uluslararası Sağlık Teknolojileri Geliştirici ve Hızlandırıcı Uygulama ve Araştırma Merkezi Merkez Danışma Kurulu Başkanı, Mart 2018- Ağustos ...
Related Occupations for DWA by U.S. Department of Labor, Employment and Training Administration is licensed under a Creative Commons Attribution 4.0 International License ...
100D: CRYSTAL STRUCTURE OF THE HIGHLY DISTORTED CHIMERIC DECAMER R(C)D(CGGCGCCG)R(G)-SPERMINE COMPLEX-SPERMINE BINDING TO PHOSPHATE ONLY AND MINOR GROOVE TERTIARY BASE-PAIRING
by Brian Robinson, 9/27/98. I don t know if R C Lewontin s name features in the vast pool of knowledge above, but let me drop it in now to watch the ripples (even if the stone itself sinks!). You ll recall that quite some years ago he co-wrote a book, Not In Our Genes . It s a very long time since I read it but my feeling persists that it was not always easy to be sure what exactly the authors were saying (was it Bertrand Russell who said that you can never be sure exactly what you re saying until you know precisely what it is you are denying?). The general message, I think, was that it was quite wrong to look to genes to answer questions about human behaviour and intelligence. If that is a garbled account, please correct me, but it would seem extraordinary if genes were not to have a controlling influence on the brain when they exert so much control on every other organ in the body. But I ve just been reading his 1993 book, The Doctrine of DNA: Biology as ideology which, excepting one chapter, ...
Aickin, C C. and Thomas, R C., Micro-electrode measurement of the intracellular ph and buffering power of mouse soleus muscle fibres. (1977). Subject Strain Bibliography 1977. 3416 ...
Meier, H; Allen, R C.; and Hoag, W G., Separation and purification of clotting factors from inbred mice. (1963). Subject Strain Bibliography 1963. 791 ...
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LiveRC.com features the latest news, pictures, and videos in the radio control car industry. LiveRC.com also features exclusive live audio and video from various races around the world.
If this is your first visit, be sure to check out the FAQ by clicking the link above. You may have to register before you can post: click the register link above to proceed. To start viewing messages, select the forum that you want to visit from the selection below ...
chains in the Genus database with same CATH superfamily 4HJ7 A; 3MEF A; 2JA6 H; 3KOJ A; 4B3M Q; 4JI8 Q; 1AE2 A; 1Z47 A; 3CW2 C; 5BR8 Q; 4X62 L; 4LF7 Q; 1KRT A; 4A3G G; 1O7I A; 4X65 L; 1N34 L; 3FP9 A; 1N8R C; 3IRB A; 2VQF L; 3A74 A; 3KF6 A; 4BY1 H; 1P8L A; 1JE5 A; 4ME3 A; 4LF9 Q; 5IYB G; 1E1O A; 4A3L G; 2NVQ H; 1VQ7 A; 2ZM6 Q; 2WB1 G; 4A3J H; 1LTL A; 3GTG H; 2OCE A; 3KDF B; 3I7F A; 1X3G A; 1QUQ B; 3AQQ A; 2JEA I; 2N8N A; 4HJ9 A; 1L1O A; 2CWA A; 4JI6 Q; 3GTK H; 4A3B H; 4JV5 L; 3RLF A; 4KHP Q; 5GAH C; 2B3G A; 3EIV A; 2ZTE A; 3G4S A; 4DR5 L; 4JI3 L; 4C3I H; 2NVX H; 4DPG A; 2IX1 A; 4K0K Q; 5EAY A; 4PMW A; 1I5F A; 2OQ0 A; 1XMQ Q; 1Y1W H; 4LF6 Q; 3UE0 A; 2ASB A; 1MKH A; 4JI7 L; 3G48 A; 4A3D H; 4TQU S; 3BZC A; 2UXD L; 1XPU A; 2K5V A; 4YWL A; 4GLV B; 4DV3 Q; 3I56 A; 4JOI C; 1EIF A; 5C3E H; 4X66 L; 2RF4 A; 2VL6 A; 2YV5 A; 1X3E A; 2BH2 A; 2I5M X; 5H1S E; 5GAD C; 1I94 Q; 1PYS B; 2NZH A; 2VUM G; 1VQA A; 2PNH A; 2D62 A; 5HY6 A; 1TWF H; 1E22 A; 4DR4 Q; 3BU2 A; 1P16 A; 2D0O A; 2H5X A; 2CKZ B; 2Z0S A; 4RMF A; ...
Mace H A , Peterson R C , The Canadian Mineralogist , 33 (1995) p.7-11, The crystal structure of fichtelite, a naturally occuring hydrocarbon ...
Y V Bogdanova, C J Owen, A N Fazakerley, R C Fear (MSSL), M G G T Taylor (ESTEC), M W Dunlop, J A Davies (RAL), A D Lahiff (MSSL), F Pitout (MPI), C M Carr (IC), H Laakso (ESTEC), Z Pu (Beijing), Q -G Zong (Boston), S E Milan (Leicester), C Shen (CSSAR), H Rème (CESR), and A Balogh (IC), Plasma layers around dayside subsolar magnetopause and inside the cusp region under northward IMF: conjugated Cluster and Double Star observations ...
0. Clustering using convex fusion penalties - An R/C++ implementation of the clusterpath algorithm described in Hocking et al. 2011, for robust convex clustering using sparsity-inducing fusion penalties ...
0. Clustering using convex fusion penalties - An R/C++ implementation of the clusterpath algorithm described in Hocking et al. 2011, for robust convex clustering using sparsity-inducing fusion penalties ...
Centro Interdipartimentale di Ricerca sul Cambiamento Politico Centre for the Study of Political Change C I R C a P ON THE RELATIONSHIP BETWEEN PARTY ANG GOVERNMENT Maurizio Cotta N. 6/1999 Università
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Gaisl, Thomas; Giunta, Cecilia; Bratton, Daniel J; Sutherland, Kate; Schlatzer, Christian; Sievi, Noriane; Franzen, Daniel; Cistulli, Peter A; Rohrbach, Marianne; Kohler, Malcolm (2017). Obstructive sleep apnoea and quality of life in Ehlers-Danlos syndrome: a parallel cohort study. Thorax, 72(8):729-735.. Kapferer-Seebacher, Ines; Pepin, Melanie; Werner, Roland; Aitman, Timothy J; Nordgren, Ann; Stoiber, Heribert; Thielens, Nicole; Gaboriaud, Christine; Amberger, Albert; Schossig, Anna; Gruber, Robert; Giunta, Cecilia; Bamshad, Michael; Björck, Erik; Chen, Christina; Chitayat, David; Dorschner, Michael; Schmitt-Egenolf, Marcus; Hale, Christopher J; Hanna, David; Hennies, Hans Christian; Heiss-Kisielewsky, Irene; Lindstrand, Anna; Lundberg, Pernilla; Mitchell, Anna L; Nickerson, Deborah A; Reinstein, Eyal; Rohrbach, Marianne; Romani, Nikolaus; Schmuth, Matthias; et al (2016). Periodontal Ehlers-Danlos syndrome is caused by mutations in C1R and C1S, which encode subcomponents C1r and C1s of ...
Sharma Clinic in Sector-12, Gurgaon. Book Appointment, Consult Doctors Online, View Doctor Fees, Contact Number, Address for Sharma Clinic - Dr. R C Sharma | Lybrate
Buy Tamiya The Grasshopper R/C Model Kit 1:10 from the Model Kits range at Hobbycraft. Free UK Delivery over £20 and Free Returns.
Open205R-C is an STM32 development board designed for the STM32F205RBT6 microcontroller, consists of the mother board and the MCU core board Core205R. The
A series of [(R′‐C^N^C‐R′′)Pt(L)] complexes with doubly deprotonated cyclometalated R′‐C^N^C‐R′′ ligands (R′‐C^N^C‐R′′=2,6‐diphenylpyridine derivatives ...
The main objective of this study was to enhance teachers skills in the use of Role-play technique to develop speaking skills at Dalun R/C Basic schools. This has become necessary following pupils inability to communicate well in the English language. For this reason, teachers were trained on how to use role-play to develop speaking skills. Population for the study was made of teachers of Dalun R/C basic schools. The study employed action research design and it was done in three fac.... ...
The main objective of this study was to enhance teachers skills in the use of Role-play technique to develop speaking skills at Dalun R/C Basic schools. This has become necessary following pupils inability to communicate well in the English language. For this reason, teachers were trained on how to use role-play to develop speaking skills. Population for the study was made of teachers of Dalun R/C basic schools. The study employed action research design and it was done in three fac.... ...
Gentaur molecular products has all kinds of products like :search , Zyagen \ SERPING1 purified MaxPab Antibody \ APO-000710-B01P for more molecular products just contact us
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A cooling arrangement is disclosed for a vehicle having a first component, a first duct, and a cooling fan configured to deliver air through the first duct to the first component when the cooling fan
Janu r havi szak rt nk, Prof. Dr. K ri Gy rgy v laszolt az olvas k s a Daganatok.hu k rd seire a r kkutat s leg jabb eredm nyeivel s az egy nre szabott ter pi kkal kapcsolatban. Kider l t bbek k z tt, hogy milyen r kt pusokra l teznek m r c lzott gy gyszerek, ezek hogyan hatnak, vannak-e mell khat saik, s az is, hogy a legvesz lyesebb r kkelt k nt emlegetett doh nyf st pontosan hogyan k ros tja a sejteket. Az olvas i k rd seket n hol r vid tett form ban, ltal nos tva adjuk k zre, hogy mindenki sz m ra hasznos inform ci val szolg ljunk.
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Basciano L C , Peterson R C , Roeder P L , The Canadian Mineralogist , 36 (1998) p.1203-1210, Description of schoenfliesite, MgSn(OH)6, and roxbyite, Cu1.72S, from a 1375 BC shipwreck,, and rietveld neutron-diffraction refinement of synthetic schoenfliesite, wickmanite,, MnSn(OH)6, and burtite, CaSn(OH)6, Locality: a 1375 BC shipwreck ...
Szerb Orthodox Templom. 8000.Székesfehérvár Rác u.16.. Templom 1771-ben épült. Külső homlokzata barokk stílusú, a belső bizánci stilusban készült. Sok értékes freskó,ikon látható a templomegyüttesben,mely 1989-ben EUROPA NOSTRE fődíjat kapott. Templomunk szomszédjában található a Tájház, mely a szerb családok mindennapjait mutatja be, továbbá megtalálható még a Kézmüves Mesterek Háza is. Vezetés óránként, ill. igény szerint indul. Szept. 19-én 10h. Liturgia.. Szo. 9-12h. 13-17h. Vasárnap: 10-17h ig várjuk a látogatókat.. ...
Sorry to bug again but can someone check my answers please thank you. 1. Simplify the expression 3w-10w A. 13w B. -7w C. -7 D. 7w 2. Y+2y+3z A. 2y+3z B. 3y+3z C. 2y^2+3z D. 6yz 3. 6r+r-5r A. 2r B. 1r+r C. 0r D. 7r-5r 4. 5x+2(x+6) A. 7x+6 B. 7x^2+12 C. 7x+12 D. 7x (x+6) 5. -3m+... ...
ONEONTA, 08/19/14 - State Senator James L. Seward (R/C/I-Oneonta) has received top marks from leading business advocacy group, Unshackle Upstate.
I thought you might be interested in looking at [résolu] Problème dautonomie sur Macbook Pro Retina (3h30).. https://consomac.fr/forums/topic/2605-r%C3%A9solu-probl%C3%A8me-dautonomie-sur-macbook-pro-retina-3h30/?do=findComment&comment=30406 ...
I thought you might be interested in looking at [résolu] Problème dautonomie sur Macbook Pro Retina (3h30).. https://consomac.fr/forums/topic/2605-r%C3%A9solu-probl%C3%A8me-dautonomie-sur-macbook-pro-retina-3h30/?do=findComment&comment=30407 ...
Dopo mesi di non-aggiornamento xD ho deciso di trasferirmi su Livejournal. Questo vuol dire che {a r c o b a l e n o} non verrà più aggiornato, ma rimarrà come archivio di tutti i miei vecchi scleri.... ...
Following Beckys advice, I made up the pass the parcel (with Rubys old Cbeebies magazines as wrapping) with a simple forfeit in each layer (admittedly I also put a little sweet in too!). They were very simple tasks that I felt a 3 or 4 year old could manage easily, and we were on hand to help. However, it soon became clear that these kids had no clue how to play pass the parcel. I dont think most of them had ever done it before - how sad is that? It was also made more difficult by me having no idea how many would turn up as most hadnt RSVPd and lots of people arrived late - some breezing in an hour late ...
"Molecular cloning of cDNA for human complement component C1s. The complete amino acid sequence". European Journal of ...
... prevents complement-enhanced activation of autoimmune human B cells in vitro. () World Health Organization (2017). " ... 31 (4). Nikitin PA, Rose EL, Byun TS, Parry GC, Panicker S (February 2019). "C1s Inhibition by BIVV009 (Sutimlimab) Prevents ... Complement-Enhanced Activation of Autoimmune Human B Cells In Vitro". Journal of Immunology. 202 (4): 1200-1209. doi:10.4049/ ...
C1q associates with C1r and C1s in order to yield the first component of the serum complement system. Deficiency of C1q has ... Busby TF, Ingham KC (1990). "NH2-terminal calcium-binding domain of human complement C1s- mediates the interaction of C1r- with ... Complement C1q subcomponent subunit A is a protein that in humans is encoded by the C1QA gene. This gene encodes a major ... "Entrez Gene: C1QA complement component 1, q subcomponent, A chain". Human C1QA genome location and C1QA gene details page in ...
The CUB domain (for complement C1r/C1s, Uegf, Bmp1) is a structural motif of approximately 110 residues found almost ... Bork P (Apr 1991). "Complement components C1r/C1s, bone morphogenic protein 1 and Xenopus laevis developmentally regulated ... Mammalian complement-activating component of Ra-reactive factor (RARF), a protease that cleaves the C4 component of complement ... Mammalian complement subcomponents C1s/C1r, which form the calcium-dependent complex C1, the first component of the classical ...
The human complement subcomponent C1q associates with C1r and C1s in order to yield the first component of the serum complement ... Busby TF, Ingham KC (1990). "NH2-terminal calcium-binding domain of human complement C1s- mediates the interaction of C1r- with ... C1QBP, GHABP1, SF2p32, gC1Q-R, gC1qR, p32, complement component 1, q subcomponent binding protein, complement C1q binding ... Complement component 1 Q subcomponent-binding protein, mitochondrial is a protein that in humans is encoded by the C1QBP gene.[ ...
Takada F, Takayama Y, Hatsuse H, Kawakami M (Oct 1993). "A new member of the C1s family of complement proteins found in a ... This protein is not directly involved in complement activation but may play a role as an amplifier of complement activation by ... MASP-1 is a serine protease that functions as a component of the lectin pathway of complement activation. The complement ... MASP-1 is involved in the lectin pathway of the complement system and is responsible for cleaving C4 and C2 into fragments to ...
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 ... "Lectin complement pathway gene profile of donor and recipient determine the risk of bacterial infections after orthotopic liver ...
... complement c1r MeSH D08.811.277.300.290 - complement c1s MeSH D08.811.277.300.450 - complement factor d MeSH D08.811.277.352. ... complement factor b MeSH D08.811.277.656.300.760.200 - complement factor d MeSH D08.811.277.656.300.760.210 - complement factor ...
DSE Complement pathway: C1R, C1S Intracellular processes: SLC39A13, ZNF469, PRDM5 Variations in these genes usually alter the ... Molecular testing may reveal mutations in C1R or C1S genes affecting the C1r protein. Cardiac-valvular EDS (cvEDS) is ... Group E disorders are characterized by defects in the complement pathway. Group F are disorders of intracellular processes, and ...
... complement subcomponent C1r EC 3.4.21.42: complement subcomponent C1s EC 3.4.21.43: classical-complement-pathway C3/C5 ... Now EC 3.4.21.43, classical-complement-pathway C3/C5 convertase EC 3.4.21.45: complement factor I EC 3.4.21.46: complement ... factor D EC 3.4.21.47: alternative-complement-pathway C3/C5 convertase EC 3.4.21.48: cerevisin EC 3.4.21.49: hypodermin C EC ... convertase EC 3.4.21.44: Transferred entry: complement component C5 convertase. ...
... s contain the following four domains: N-terminal CUB domain (for complement C1r/C1s, Uegf, Bmp1) Coagulation factor 5 ...
The MASPs are very similar to C1r and C1s molecules of the classical complement pathway, respectively. When the carbohydrate- ... The lectin pathway or lectin complement pathway is a type of cascade reaction in the complement system, similar in structure to ... Additional resources on Endothelial Injury Syndrome Classical complement pathway Alternative complement pathway Mannan-binding ... In contrast to the classical complement pathway, the lectin pathway does not recognize an antibody bound to its target. The ...
"Activation of the classical complement pathway by mannose-binding protein in association with a novel C1s-like serine protease ... This enzyme catalyses the following chemical reaction Selective cleavage after Arg223 in complement component C2 (-Ser-Leu-Gly- ... Arg-Lys-Ile-Gln-Ile) and after Arg76 in complement component C4 (-Gly-Leu-Gln-Arg-Ala-Leu-Glu-Ile) This mannan-binding lectin ( ... "The structure of MBL-associated serine protease-2 reveals that identical substrate specificities of C1s and MASP-2 are realized ...
... activated complement C1s, complement C overbar 1r, C1s) is a protein involved in the complement system. C1s is part of the C1 ... complement activation, lectin pathway. • complement activation. • regulation of complement activation. Sources:Amigo / QuickGO ... "Selective complement C1s deficiency caused by homozygous four-base deletion in the C1s gene". Human Genetics. 103 (4): 415-8. ... Sim RB (1981). "The human complement system serine proteases C1r and C1s and their proenzymes". Methods in Enzymology. 80 Pt C ...
... irreversibly binds to and inactivates C1r and C1s proteases in the C1 complex of classical pathway of complement. ... 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 ... Inhibition of the complement cascade can decrease this damage. C1-inhibitor is contained in the human blood; it can, therefore ...
It is the first component of the classical complement pathway and is composed of the subcomponents C1q, C1r and C1s. The C1 ... Active C1r cleaves the C1s molecules, activating them. Active C1s splits C4 and then C2, producing C4a, C4b, C2a and C2b. The ... The C1 complex (complement component 1, C1) is a protein complex involved in the complement system. ... 2001). "The complement system and innate immunity". Immunobiology: The Immune System in Health and Disease. New York: Garland ...
C1q associates with C1r and C1s in order to yield the C1 complex (C1qr2s2), the first component of the serum complement system ... The complement component 1q (or simply C1q) is a protein complex involved in the complement system, which is part of the innate ... Activation of the C1 complex initiates the classical complement pathway of the complement system. The antibodies IgM and all ... C1q is a subunit of the C1 enzyme complex that activates the serum complement system. C1q comprises 6 A, 6 B and 6 C chains. ...
Linkage analysis and population genetics of the C1S subcomponent of the first complement component". Complement and ... C1r along with C1q and C1s form the C1 complex, which is the first component of the serum complement system. C1r is an enzyme ... Sim RB (1981). "The human complement system serine proteases C1r and C1s and their proenzymes". Methods in Enzymology. 80 Pt C ... Busby TF, Ingham KC (May 1990). "NH2-terminal calcium-binding domain of human complement C1s- mediates the interaction of C1r- ...
C1-inhibitor plays the role of inactivating C1r and C1s to prevent further downstream classical complement activity. C1- ... Alternative complement pathway - another complement system pathway Lectin pathway - another complement system pathway Noris, ... The classical complement pathway is one of three pathways which activate the complement system, which is part of the immune ... Activation of the complement pathway through the classical, lectin or alternative complement pathway is followed by a cascade ...
Classical complement pathway C1Q complex - C1R / C1S C4 - C4a C2 Mannan-binding lectin pathway MASP1 / MASP2 Mannan-binding ... system Complement system Classical complement pathway Mannan-binding lectin pathway Alternate complement pathway Complement ... see complement proteins section) Collectins Mannan-binding lectin (MBL) Surfactant protein A (SP-A) Surfactant protein D (SP-D ... Complement receptor of the immunoglobulin family) Anaphylatoxin receptors C3a receptor C5a receptor (CD88) C5AR2 Fc receptor Fc ...
... the resultant C4bC2 complex is then cleaved by C1s or MASP2 into C2a and C2b. It is thought that cleavage of C2 by C1s, while ... 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 ... Johnson CA, Densen P, Hurford RK, Colten HR, Wetsel RA (May 1992). "Type I human complement C2 deficiency. A 28-base pair gene ...
... that are not involved in coagulation such as trypsin and the C1s subunit of the enzyme C1 involved in the classical complement ... Ogston D, Murray J, Crawford GP (1976). "Inhibition of the activated Cls subunit of the first component of complement by ...
The classical pathway of complement activation is initiated when the C1 complex, made up of C1r and C1s serine proteases, ... 12th European Meeting on Complement in Human Disease12th European Meeting on CHD12th European Meeting on Complement in Human ... C3a is one of the proteins formed by the cleavage of complement component 3; the other is C3b. C3a is a 77 residue ... Anaphylatoxins are small complement peptides that induce proinflammatory responses in tissues. C3a is primarily regarded for ...
... 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 ...
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 ...
All forms of HAE lead to abnormal activation of the complement system, and all forms can cause swelling elsewhere in the body, ... This serine protease inhibitor (serpin) normally inhibits the association of C1r and C1s with C1q to prevent the formation of ... In this analysis, it is usually a reduced complement factor C4, rather than the C1-INH deficiency itself, that is detected. The ... In hereditary angioedema, bradykinin formation is caused by continuous activation of the complement system due to a deficiency ...
As a member of the LDLR family, LRP1 contains cysteine-rich complement-type repeats, EGF (gene) repeats, β-propeller domains, a ... LRP1 has been shown to interact with: A2-Macroglobulin, β-amyloid precursor protein, APBB1, APOE, Aprotinin, C1S/C1q inhibitor ... and eleven cysteine-rich complement-type repeats, respectively. These repeats bind extracellular matrix proteins, growth ... CALR, CD44, Chylomicron, Circumsporozoite protein, Collectin, Complement C3, CTGF, DLG4, Elastase, Factor IXa, Factor VIIa, ...
Complement deficiencies are the result of a lack of any of these proteins. They may predispose to infections but also to ... C1q deficiency (lupus-like syndrome, rheumatoid disease, infections) C1r deficiency (idem) C1s deficiency C4 deficiency (lupus- ... 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 ...
C1r ja C1s-i aktivatsioonile koos C1s esteraasi formuleerumisega järgneb C4 ja C2 lõhustumine, mis vabastab väikseid peptiide ... Zipfel, P. F., Hallström, T., & Riesbeck, K. (2013). Human complement control and complement evasion by pathogenic microbes- ... 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. ...
Watford WT, Ghio AJ, Wright JR (2000). "Complement-mediated host defense in the lung". Am J Physiol Lung Cell Mol Physiol 279: ... MASP-3 vzniká alternativním sestřihem genu MASP-1/3.) MASP-2 a MASP-3 vytváří tetramerní komplexy připomínající C1r a C1s, ... Watford WT, Wright JR, Hester CG, Jiang H, Frank MM (2001). "Surfactant Protein A Regulates Complement Activation". J Immunol ... Klasická cesta aktivace komplementu je spuštěna komplexem C1 (C1q, C1r, C1s), rozpozná-li Fc oblast imunoglobulinu navázaného ...
The C1-complex is composed of 1 molecule of C1q, 2 molecules of C1r and 2 molecules of C1s, or C1qr2s2. This occurs when C1q ... 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 ...
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. ...
... is a member of the immunoglobulin superfamily, with a structure related to the putative primordial form of the family. As members of the immunoglobulin superfamily play fundamental roles in intercellular recognition involved in various immunologic phenomena, differentiation, and development, basigin is thought also to play a role in intercellular recognition (Miyauchi et al., 1991; Kanekura et al., 1991).[9][10] It has a variety of functions. In addition to its metalloproteinase-inducing ability, basigin also regulates several distinct functions, such as spermatogenesis, expression of the monocarboxylate transporter and the responsiveness of lymphocytes.[6] Basigin is a type I integral membrane receptor that has many ligands, including the cyclophilin (CyP) proteins Cyp-A and CyP-B and certain integrins.[11][12][13] It is expressed by many cell types, including epithelial cells, endothelial cells and leukocytes. The human basigin protein contains 269 amino acids that form two heavily ...
... complement c1q MeSH D12.776.124.486.274.050.280 - complement c1r MeSH D12.776.124.486.274.050.290 - complement c1s MeSH D12.776 ... complement c1r MeSH D12.776.124.486.274.860.290 - complement c1s MeSH D12.776.124.486.274.860.387 - complement c3-c5 ... complement c3 MeSH D12.776.124.486.274.250.250 - complement c3a MeSH D12.776.124.486.274.250.260 - complement c3b MeSH D12.776. ... complement c4 MeSH D12.776.124.486.274.350.250 - complement c4a MeSH D12.776.124.486.274.350.260 - complement c4b MeSH D12.776. ...
A Type C1-S-AY-1 ship, she was completed in November 1943. She was transferred to the MoWT under the terms of lend lease ... She could carry 1,458 troops in addition to her complement of 250 officers and men. Armament consisted 1 x 4-inch gun, 1 x 12 ...
In the classical pathway, the complement component-hereafter abbreviated by the "C" preceding the protein number- termed C1s, a ... 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 ... All three pathways converge at a step in which complement protein C3 is cleaved into proteins C3a and C3b, which results in a ...
They then cleave C1s (another serine protease). The C1r2s2 component now splits C4 and then C2, producing C4a, C4b, C2a, and ... 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 two main proposed types of EGF-like domains are the human EGF-like (hEGF) domain and the complement C1r-like (cEGF) domain ... Below is a list of human proteins containing the EGF-like domain: AGC1; AGRIN; AREG; ATRN; ATRNL1; BCAN; BMP1; BTC; C1S; CASPR4 ... Circolo A, Garnier G, Volanakis JE (2003). "A novel murine complement-related gene encoding a C1r-like serum protein". ... C1r is a highly specific serine protease initiating the classical pathway of complement activation during immune response. Both ...
Complement C1s subcomponent. Complement C1s subcomponent, EC 3.4.21.42 (C1 esterase) (Complement component 1 subcomponent s) [ ... Cleaved into: Complement C1s subcomponent heavy chain; Complement C1s subcomponent light chain] ... tr,H0Y5D1,H0Y5D1_HUMAN Complement C1s subcomponent (Fragment) OS=Homo sapiens OX=9606 GN=C1S PE=1 SV=1 ... IPR035708, Complement_C1s_subcomponent. IPR000859, CUB_dom. IPR001881, EGF-like_Ca-bd_dom. IPR000152, EGF-type_Asp/Asn_ ...
Browse our Complement Component C1s Lysate catalog backed by our Guarantee+. ... Complement Component C1s Lysates available through Novus Biologicals. ... Alternate Names for Complement Component C1s Lysates. Complement Component C1s lysate, C1S lysate, basic proline-rich peptide ... We offer Complement Component C1s Lysates for use in common research applications: Western Blot. Each Complement Component C1s ...
Browse our Complement Component C1s Antibody Pair catalog backed by our Guarantee+. ... Complement Component C1s Antibody Pairs available through Novus Biologicals. ... Alternate Names for Complement Component C1s Antibody Pairs. Complement Component C1s antibody pair, C1S antibody pair, basic ... Complement Component C1s Antibody Pairs. We offer Complement Component C1s Antibody Pairs for use in common research ...
c1s (complement C1r) gene expression in Xenopus laevis, NF stage 29 & 30 embryo, in situ hybridization, lateral view, anterior ... c1s.L. X.laevis. Throughout NF stage 29 and 30. notochord. Image source: Large scale screen. Larger Image. Printer Friendly ...
Complement component C1r/C1s deficiency disease page. Quantitative data and detailed annnotation of the targets of licensed and ... Complement component C1r/C1s deficiency. GtoPdb Disease Summaries. This section gives an overview of the disease, and where ...
Genetic analysis of complement C1s deficiency associated with systemic lupus erythernatosus highlights alternative splicing of ... of complement C1s deficiency associated with systemic lupus erythernatosus highlights alternative splicing of normal C1s gene ... autoinimunity, immunodeficiency diseases, complement, human, systemic, lupus erythernatosus. in Molecular Immunology. volume. ... Deficiencies of complement proteins of the classical pathway are strongly associated with the development of autoimmune ...
The structure and enzymic activities of the C1r and C1s subcomponents of C1, the first component of human serum complement. R B ... The structure and enzymic activities of the C1r and C1s subcomponents of C1, the first component of human serum complement ... The structure and enzymic activities of the C1r and C1s subcomponents of C1, the first component of human serum complement ... The structure and enzymic activities of the C1r and C1s subcomponents of C1, the first component of human serum complement ...
complement C1s - S1: Chymotrypsin. Detailed annotation on the structure, function, physiology, pharmacology and clinical ... Complement component C1s deficiency Synonyms:. Immunodeficiency due to an early component of complement deficiency [Orphanet: ... C1sb , complement component C1SB , r-gsp , Gm5077 , predicted gene 5077 , complement component 1 , complement component 1, s ... S1: Chymotrypsin: complement C1s. Last modified on 15/02/2018. Accessed on 17/06/2019. IUPHAR/BPS Guide to PHARMACOLOGY, http ...
THE ANTI C1S COMPLEMENT ANTIBODY TNT009 INDUCES RAPID COMPLETE REMISSIONS OF ANAEMIA IN PATIENTS WITH PRIMARY COLD AGGLUTININ ... an antibody directed against complement component C1s, could stop haemolysis in CAD patients.. Methods. This represents an ... This complement deposition opsonizes erythrocytes which undergo extravascular haemolysis in the liver.. Aims. Based on ... Keyword(s): Clinical trial, Complement, Hemolytic anemia, Monoclonal antibody Code of conduct/disclaimer available in General ...
Complement C1r/C1s components (1 copy). - Complement-activating component of Ra-reactive factor (RARF) (1 copy).. - Complement ...
Complement C1s Homo sapiens 0.718 CHEMBL6007 Transient receptor potential cation channel subfamily A member 1 Homo sapiens ...
... activated complement C1s, complement C overbar 1r, C1s) is a protein involved in the complement system. C1s is part of the C1 ... complement activation, lectin pathway. • complement activation. • regulation of complement activation. Sources:Amigo / QuickGO ... "Selective complement C1s deficiency caused by homozygous four-base deletion in the C1s gene". Human Genetics. 103 (4): 415-8. ... Sim RB (1981). "The human complement system serine proteases C1r and C1s and their proenzymes". Methods in Enzymology. 80 Pt C ...
Complement C1s. Complement C1 Inhibitor Protein. Complement C1 Inactivator Proteins. Immunologic Factors. Physiological Effects ... Complement C4 Serum Levels [ Time Frame: Pre-infusion to 1 hour post-infusion ]. Change in complement C4 serum levels from pre- ...
Complement C1s. Complement C1 Inhibitor Protein. Complement C1 Inactivator Proteins. Immunologic Factors. Physiological Effects ...
The C1s protease from the classical complement pathway propagates the original. Posted on November 19, 2018. by bassresearch ... The C1s protease from the classical complement pathway propagates the original activation of the pathway of the machine by ... Cleavage from the REPLi combinatorial substrate collection by C1s enzymes Crazy type or mutant C1s forms (400?nM) were tested ... The pace of boost of fluorescence in the current presence of 400?nM C1s (crazy type or mutant) was measured on the BMG Systems ...
Home > Protein > C1S rat. human. mouse. New Protein Search:. Complement C1s subcomponent ...
Tumour-cell-derived complement components C1r and C1s promote growth of cutaneous squamous cell carcinoma (2019) British ... Complement System in Cutaneous Squamous Cell Carcinoma (2019) International Journal of Molecular Sciences ...
ABC, ATP-Binding Cassette; Ch, Choline; CUB, Complement C1r/C1s, Uegf, Bmp1 domain; EC Material, Extracellular Material; NHR, ...
Complement C1s subcomponent C1S P09871 DDA 0.02 0.59 Protein Name Gene Acc. UNIPROT MS Type p-value Fold change ... 8], we found decreased levels of Complement C1 protein. Interestingly we also found that the complement activator of the lectin ... The 55 features of the cluster were used for GO analysis and the results are reported in the histogram (B). The complement ... The 55 features of the cluster were used for GO analysis and the results are reported in the histogram (B). The complement ...
... which then cleaves C1s to generate an active serine protease. Once activated, C1s cleaves C4, etc, leading to the complement ... ANTI-COMPLEMENT FACTOR C1S ANTIBODIES AND USES THEREOF. WO2015006507A1. 2015-01-15. METHODS OF TREATMENT FOR ALZHEIMERS ... C1 inhibitor regulates the classical pathway of complement activation by blocking the active site of C1r and C1s and ... Complement. Complement is a system of plasma proteins that interacts with the cell surfaces of pathogens or cells to mark them ...
Rabbit recombinant monoclonal C1s antibody [EPR9066(B)]. Validated in WB, IP, IHC, Flow Cyt and tested in Human. Cited in 1 ... Defects in C1S are the cause of complement component C1s deficiency (C1SD) [MIM:613783]. A rare defect resulting in C1 ... C1s B chain is a serine protease that combines with C1q and C1s to form C1, the first component of the classical pathway of the ... All lanes : Anti-C1s antibody [EPR9066(B)] (ab134943) at 1/1000 dilution. Lane 1 : Human fetal kidney lysate. Lane 2 : A431 ...
Mouse monoclonal C1s antibody [9]. Validated in ELISA and tested in Human. Immunogen corresponding to full length native ... Defects in C1S are the cause of complement component C1s deficiency (C1SD) [MIM:613783]. A rare defect resulting in C1 ... C1s B chain is a serine protease that combines with C1q and C1s to form C1, the first component of the classical pathway of the ... I have a customer who is interested in AB39551 Mouse monoclonal to C1s, and wants to know the domain this protein is targeted ...
This is, as far as we are aware, the first candidate gene in the complement system with an association to a clinically relevant ... Dysregulation of the complement system has been implicated, but molecular mechanisms are incompletely understood. In this study ... The result highlights a potential role for the complement system in the pathogenesis of preeclampsia and may help in defining ... In this study, we determined the potential linkage of severe preeclampsia to the most central complement gene, C3. Three ...
"Molecular cloning of cDNA for human complement component C1s. The complete amino acid sequence". European Journal of ...
The lectin pathway of the complement system is initiated by mannose-binding lectin (MBL)-associated serine proteases, MASP-1, ... The lectin pathway of the complement system is initiated by mannose-binding lectin (MBL)-associated serine proteases, MASP-1, ... Early complement proteases: C1r, C1s and MASPs. A structural insight into activation and functions. Mol Immunol (2009) 46:2745- ... This is a valid assumption as it was shown for C1s and C1s-C1 inhibitor complex in guinea pig (53). Synthesis rates (Table S1 ...
S905 LONG TERM INHIBITION OF COMPLEMENT C1S IN PATIENTS WITH COLD AGGLUTININ DISEASE: RESULTS FROM A NAMED PATIENT PROGRAM. ...
K01331 C1S; complement component 1, s subcomponent [EC:3.4.21.42] K01331 C1S; complement component 1, s subcomponent [EC:3.4. ... 107756066 complement factor B-like 107756181 complement factor B-like 107723846 complement factor B-like 107715263 complement ... complement component 3 K03990 C3; complement component 3 K03990 C3; complement component 3 K03989 C4; complement component 4 ... 107714593 complement C1s subcomponent-like 107724792 mannan-binding lectin serine protease 2-like 107720441 complement C2-like ...
The quaternary structure in solution of human complement subcomponent C1r2C1s2. S J Perkins, A S Nealis ... Neutron scattering analyses on C1s and C1r2 are consistent with a linear structure for C1s, but not for C1r2. An X-shaped ... The quaternary structure in solution of human complement subcomponent C1r2C1s2 ... The quaternary structure in solution of human complement subcomponent C1r2C1s2 ...
C1r activates C1s so that it can, in turn, activat.... Gene Name. C1S. Uniprot ID. P09871. Uniprot Name. Complement C1s ... C1q associates with the proenzymes C1r and C1s to yield C1, the first component of the serum complement system. The collagen- ... C1q associates with the proenzymes C1r and C1s to yield C1, the first component of the serum complement system. The collagen- ... C1q associates with the proenzymes C1r and C1s to yield C1, the first component of the serum complement system. The collagen- ...
complement protein C1r/C1s, Uegf, and Bmp1. EGF. epidermal growth factor. PPP. platelet-poor plasma. PRP. platelet-rich plasma ... signal peptide-CUB (complement protein C1r/C1s, Uegf, and Bmp1)-EGF (epidermal growth factor) domain-containing protein 1. ... SCUBE1 (signal peptide-CUB [complement protein C1r/C1s, Uegf, and Bmp1]-EGF [epidermal growth factor] domain-containing protein ...
  • When tested on synthetic amino acid esters, subcomponent C-1r hydrolysed both lysine and tyrosine ester bonds, but subcomponent C-1r did not hydrolyse any amino acid esters tested nor any protein substrate except subcomponent C1s. (biochemj.org)
  • The lysine esterase activity of subcomponent C1s provides a rapid and sensitive assay of the subcomponent. (biochemj.org)
  • C1r2C1s2 is a subcomponent of first component C1 of the complement cascade. (biochemj.org)
  • Complement component 1 Q subcomponent-binding protein, mitochondrial is a protein that in humans is encoded by the C1QBP gene . (wikipedia.org)
  • The human complement subcomponent C1q associates with C1r and C1s in order to yield the first component of the serum complement system. (wikipedia.org)
  • This gene encodes a serine protease, which is a major constituent of the human complement subcomponent C1. (abnova.com)
  • Deficiencies of complement proteins of the classical pathway are strongly associated with the development of autoimmune diseases. (lu.se)
  • A protease component of the C1-complex of the classical complement pathway. (guidetopharmacology.org)
  • TNT009 blocked the classical pathway of complement as demonstrated by an immediate drop in CH50 and rise in C4. (ehaweb.org)
  • C1s cleaves C4 and C2 , which eventually leads to the production of the classical pathway C3-convertase . (wikipedia.org)
  • The C1s protease from the classical complement pathway propagates the original activation of the pathway of the machine by cleaving and thereby activating the C4 and C2 complement components. (bassresearch.com)
  • Understanding the foundation from the connection between C1s and its own physiological substrates will probably result in insights you can use to design effective inhibitors from the enzyme for make use of in treating illnesses caused by swelling as consequence of over-activity from the traditional go with pathway. (bassresearch.com)
  • C1s B chain is a serine protease that combines with C1q and C1s to form C1, the first component of the classical pathway of the complement system. (abcam.com)
  • A rare defect resulting in C1 deficiency and impaired activation of the complement classical pathway. (abcam.com)
  • The lectin pathway of the complement system is initiated by mannose-binding lectin (MBL)-associated SPs (MASP)-1, and MASP-2, which are known to be present as proenzymes in blood. (frontiersin.org)
  • Complement activation can be triggered via three different, however interconnected routes: the classical, lectin, and alternative pathways, then the three routes converge into the common terminal pathway. (frontiersin.org)
  • Complement alternative pathway in ANCA-associated vasculitis: Two decades from bench to bedside. (nih.gov)
  • Depending on the nature of complement activators, the classic pathway, the alternative pathway, or the more recently discovered lectin pathway is activated predominantly to produce C3 convertase. (medscape.com)
  • Binding of factor H to C3b increases its inactivation by factor I. Properdin stabilizes it, preventing its inactivation by factors H and I. The alternate pathway does not result in a truly nonspecific activation of complement because it requires specific types of compounds for activation. (medscape.com)
  • 11 C4 is the factor activated by C1 esterase during classic pathway activation of the complement cascade. (uspharmacist.com)
  • 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)
  • The Alternative Pathway (AP) is initiated by fragments of the complement component C3. (primaryimmune.org)
  • This time-limitation is another control mechanism for the complement pathway. (primaryimmune.org)
  • The Terminal Pathway (TP) is the final set of steps in the complement activation process that forms a membrane lesion or hole (membrane attack complex or MAC) that kills susceptible bacteria or other cells that activate complement on their surfaces. (primaryimmune.org)
  • Both peptides block the lectin pathway activation completely while leaving the classical and the alternative routes intact and fully functional, demonstrating that of all complement proteases only MASP-1 and/or MASP-2 are inhibited by these peptides. (jimmunol.org)
  • There are three ways through which the complement system can be activated: the classical, the lectin, and the alternative pathway. (jimmunol.org)
  • The biosimilar is a humanized monoclonal antibody that targets complement C1s, a serine protease involved in the activation of the classical complement pathway (CP). (biovision.com)
  • We studied complement 1 inhibitor (C1-INH) as an inhibitor of the alternative complement pathway. (rupress.org)
  • C1-INH prevented lysis, induced by the alternative complement pathway, of paroxysmal nocturnal hemoglobinuria (PNH) erythrocytes in human serum. (rupress.org)
  • Recently, it has been shown to be an inhibitor of the mannan-binding lectin pathway of complement activation, inhibiting mannan-binding lectin-associated serine proteases (MASPs) in that pathway ( 10 ). (rupress.org)
  • Because the alternative complement pathway has many features in common with the classical complement pathway, and because many proteins of that pathway function in a manner analogous to proteins of the classical pathway, we studied the role of the C1-INH in inhibition of the alternative complement pathway. (rupress.org)
  • In the alternate pathway complement C3 undergoes spontaneous cleavage resulting in complement B binding to C3b. (sigmaaldrich.com)
  • The Classical pathway of activation of the complement system is a group of blood proteins that mediate the specific antibody response. (wikipathways.org)
  • The Classical pathway begins with circulating C1Q binding to an antigen on the surface of a pathogen, which goes on to active and recruit 2 copies of each C1R and C1S, forming a C1 complex. (wikipathways.org)
  • There is wide variation of infections associated with complement deficiency depending on which complement protein and activation pathway is affected(Skattum et al 2011). (pediatriconcall.com)
  • C1-inh irreversibly binds to and inactivates C1r and C1s proteinases in the C1 complex of classical pathway of complement. (bionity.com)
  • Mannan-binding lectin activates C3 and the alternative complement pathway without involvement of C2. (lu.se)
  • Their analysis indicated that genetic variation within early classical complement pathway components (C1q, C1r, and C1s) affected survival. (asm.org)
  • Upon binding to carbohydrates such as mannose on pathogens, the MBL-MASP complex activates the lectin complement pathway. (nii.ac.jp)
  • Publications] Matsushita, M.: 'The lectin pathway of the complement system. (nii.ac.jp)
  • Cold agglutinin disease (CAD) is an uncommon autoimmune haemolytic anaemia in which a well‐defined, clonal low‐grade lymphoproliferative disorder of the bone marrow results in erythrocyte destruction mediated by the classical complement pathway. (legeforeningen.no)
  • Therapies targeting the classical complement pathway are promising, and the complement C1s inhibitor, BIVV009, has shown favourable results in preliminary studies. (legeforeningen.no)
  • After binding to microbial surfaces, MBL activates the complement system through a complement activation pathway, the MBL pathway, by using two recently identified serine proteases, MASP-1 and MASP-2, to activate C4 and C2 ( 2 ). (pnas.org)
  • MBP can also associate with serine proteases (MASP-1 and MASP-2) to activate the third complement activation pathway independent of antibody or C1q ( 7 , 14 ). (asm.org)
  • Canonical pathway analysis revealed the identified host proteins are mainly involved in the coagulation cascade, complement pathway or acute phase response signaling pathway. (springer.com)
  • Complement component C1q deficiency (C1QD) [MIM:613652]: A disorder caused by impaired activation of the complement classical pathway. (avivasysbio.com)
  • The levels of Clr observed in the siblings and parents sera were lower than in the control, while the concentrations of other complement proteins (C3, C4, MBL and MASP-2) were normal in all family members. (lu.se)
  • The complement cascade is composed of more than 30 proteins. (frontiersin.org)
  • 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)
  • The complement system consists of more than 30 proteins, present in blood and tissues, as well as other proteins anchored on the surfaces of cells. (primaryimmune.org)
  • Complement proteins in the circulation are not activated until triggered by an encounter with a bacterial cell, a virus, an immune complex, damaged tissue or other substance not usually present in the body. (primaryimmune.org)
  • Kirjavainen V, Jarva H, Biedzka Sarek M, Blom A, Skurnik M, Meri S. Yersinia enterocolitica serum resistance proteins YadA and ail bind the complement regulator C4b-binding protein. (labome.org)
  • 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)
  • But in CAD, the complement proteins attack the body's red blood cells. (pharmalive.com)
  • Fragments resulting from proteolytic cleavage of complement proteins are designated with lower-case-letter suffixes, e.g. (thefreedictionary.com)
  • It was initially believed that MBL-A and MBL-C were found in serum and the liver, respectively, but recently it was shown that both proteins are found in serum in comparable amounts and that both have similar capacities to activate complement ( 12 ). (asm.org)
  • Key components of the complement system are serine proteases (SPs) that are present in the circulation as zymogens ( 2 ). (jimmunol.org)
  • During complement activation, these proteases activate each other in a cascade-like manner. (jimmunol.org)
  • Complement C5a, like C3a is an anaphylatoxin, and is a chemotactic attractant for induction of neutrophilic release of antimicrobial proteases and oxygen radicals. (sigmaaldrich.com)
  • C1INH is the primary inhibitor of the complement proteases C1r and C1s as well as the contact system proteases activated Hageman factor (coagulation factor XIIa and XIIf) and plasma kallikrein. (ingentaconnect.com)
  • Complement component 1s ( EC 3.4.21.42 , C1 esterase , activated complement C1s , complement C overbar 1r , C1s ) is a protein involved in the complement system . (wikipedia.org)
  • Recognition protein C1q of innate immunity agglutinates nanodiamonds without activating complement. (nih.gov)
  • Impact of the surface charge of polydiacetylene micelles on their interaction with human innate immune protein C1q and the complement system. (nih.gov)
  • Patients with complement deficiencies encounter clinical problems that depend on the role of the specific complement protein in normal function. (primaryimmune.org)
  • Meri T, Blom A, Hartmann A, Lenk D, Meri S, Zipfel P. The hyphal and yeast forms of Candida albicans bind the complement regulator C4b-binding protein. (labome.org)
  • Her binder C1q antistoffer eller andre molekyler, for eksempel C-reaktivt protein, som er bundet på en overflate. (tidsskriftet.no)
  • Complement 1 inhibitor (C1-INH) * is a critically important protein that controls activation of multiple plasma mediator pathways ( 1 ). (rupress.org)
  • The sushi domain is also known as the complement controle protein (CCP) module or the short consensus repeat (SCR). (embl-heidelberg.de)
  • Three-dimensional structure of a complement control protein module in solution. (embl-heidelberg.de)
  • The complement control protein (CCP) modules (also known as short consensus repeats) are defined by a consensus sequence within a stretch of about 60 amino acid residues. (embl-heidelberg.de)
  • TNT009 is a monoclonal antibody that blocks the C1s protein, which is part of the complement system. (pharmalive.com)
  • C1-inhibitor ( C1-inh , C1 esterase inhibitor ) is a serine protease inhibitor (serpin) protein, the main function of which is the inhibition of the complement system to prevent spontaneous activation. (bionity.com)
  • Publications] Endo, Y.: 'Exon structure of the gene encoding the human mannose-binding protein-associated serine protease(MASP) light chain : comparison with complement' Int. Immunol.8. (nii.ac.jp)
  • Human C1S full-length ORF ( NP_001725.1, 1 a.a. - 688 a.a.) recombinant protein with GST-tag at N-terminal. (abnova.com)
  • Once bound to microbes, MBL is believed to act as an opsonin for phagocytosis ( 17 ) and to activate the complement cascade via mannose-binding lectin-associated protein 2 (MASP-2) for microbial lysis ( 28 , 34 , 39 ). (asm.org)
  • A gene on chromosome 11q12-q13.1 that encodes a highly glycosylated plasma protein which regulates the complement cascade by inhibiting activated C1r and C1s, thereby preventing complement activation. (thefreedictionary.com)
  • This gene encodes a highly glycosylated plasma protein involved in the regulation of the complement cascade. (genetex.com)
  • Its protein inhibits activated C1r and C1s of the first complement component and thus regulates complement activation. (genetex.com)
  • Defects in C1S are the cause of complement component C1s deficiency (C1SD) [MIM:613783]. (abcam.com)
  • Hereditary angioedema (HAE) is an episodic swelling disease associated with the deficiency or malfunction of complement 1 esterase inhibitor (C1-INH). (arupconsult.com)
  • Defects in this gene are the cause of selective C1s deficiency. (abnova.com)
  • A deficiency of this inhibitor results in a lack of inhibition of C1r and C1s leading to uncontrolled activation of the complement cascade and angioedema. (thefreedictionary.com)
  • This C1 esterase inhibitor complements Shire's FIRAZYR icatibant injection for the treatment of acute HAE attacks. (thefreedictionary.com)
  • Several studies reported that certain glycosaminoglycans prevent the complement cascade by activating C1 inhibitor-mediated inactivation of C1s ( 21 - 23 ). (asnjournals.org)
  • c1s (complement C1r) gene expression in Xenopus. (xenbase.org)
  • c1s (complement C1r) gene expression in Xenopus laevis, NF stage 29 & 30 embryo, in situ hybridization, lateral view, anterior left. (xenbase.org)
  • In this study, we determined the potential linkage of severe PE to the most central complement gene, C3 . (frontiersin.org)
  • This is, as far as we are aware, the first candidate gene in the complement system with an association to a clinically relevant PE subphenotype, severe PE. (frontiersin.org)
  • Low complement C4B gene copy number predicts short-term mortality after acute myocardial infarction. (labome.org)
  • We further confirmed that expression levels of a ten-gene set ( CSF2RB , RHOH , C1S , CCDC69 , CCL22 , CYTIP , POU2AF1 , FGR , CCL21 , and IL7R ) were predictive of tumor purity regardless of tumor type. (biomedcentral.com)
  • MBL is able to bind through multiple sites to various carbohydrate structures ( 29 , 37 ) and, on binding to its ligands, is able to activate complement in an antibody- and C1q-independent manner ( 12 , 24 , 26 ) using MBL-associated serine protease 1 (MASP-1) and MASP-2 ( 23 , 34 ). (asm.org)
  • During complement activation, C4 is cleaved by the serine protease C1s to C4a (9 kDa) and C4b (190 kDa). (leebio.com)
  • The Classical Complement Cascade Mediates CNS Synapse Elimination. (freepatentsonline.com)
  • The complement system functions as an interactive sequence, with one reaction leading to another in the form of a cascade. (medscape.com)
  • The complement system is a complex cascade involving proteolytic cleavage of serum glycoproteins often activated by cell receptors. (sigmaaldrich.com)
  • Ischaemic infarction may also cause initiation of the complement cascade. (sigmaaldrich.com)
  • Bioinformatics analyses indicated that DEPs in both the EOPE and LOPE groups were associated with abnormalities in the activation of the coagulation cascade and complement system as well as with lipid metabolism. (peerj.com)
  • The pathogenesis of EOPE and LOPE appeared to be associated with coagulation cascade activation, lipid metabolism, and complement activation. (peerj.com)
  • The complement system is a biochemical cascade that helps, or complements, the ability of antibodies to clear pathogens from an organism. (wikipathways.org)
  • Unfortunately, reperfusion itself contributes to injury by triggering a cascade of events including neutrophil infiltration, free radical generation, cytokine release and complement activation. (ahajournals.org)
  • The activation of the complement cascade can cause damage to cells, therefore the inhibition of the complement cascade can work as a medicine in certain conditions [1] . (bionity.com)
  • The subcomponents C1r and C1s and their activated forms C-1r and C-1s were each found to have mol.wts. (biochemj.org)
  • The amino acid compositions of each were similar, but there were significant differences in the monosaccharide analyses of subcomponents C1r and C1s, whether activated or not. (biochemj.org)
  • Subcomponents C1r and C1s have only one polypeptide chain, but subcomponents C-1r and C-1s each contain two peptide chains of approx. (biochemj.org)
  • When the classical or lectin pathways are activated, it results in the formation of the C3 convertase, C4bC2a, composed of the cleaved forms of complement factors C4 and C2 ( 4 ). (frontiersin.org)
  • Activation of the complement pathways. (medscape.com)
  • The efficacy of current treatment and the potential held by newer agents that target specific elements in complement or kinin pathways are examined. (springer.com)
  • Complement Systems: Methods and Protocols is composed of 32 individual chapters that describe a variety of protocols to purify and analyze the activity of the individual complement components or pathways. (springer.com)
  • C2 and C4 also participate in the LP. The LP is thought to be the most evolutionarily primitive of the complement pathways and the first to react before the adaptive immune response occurs. (primaryimmune.org)
  • The complement system, an essential part of the innate immune system, can be activated through three distinct routes: the classical, the alternative, and the lectin pathways. (jimmunol.org)
  • All three pathways merge through at common intersection, complement C3. (sigmaaldrich.com)
  • Although named after its complement inhibitory activity, C1-inh also inhibits proteinases of the fibrinolytic, clotting, and kinin pathways. (bionity.com)
  • You need to have more background on the various complement factors, their function, the 3 different pathways of activation etc) NCIPH is a liver disorder of vascular origin defined by a portal venous pressure exceeding 5mm Hg between portal vein and inferior vena cava (sanyal 2008), characterized by occlusion of the 3rd /4th order branches of the hepatic portal vein ( madhu 2008). (ukessays.com)
  • Cold agglutinin disease (CAD) is a difficult to treat autoimmune haemolytic anemia in which IgM antibodies bind to erythrocytes at low temperature and fix complement. (ehaweb.org)
  • Monitoring patients with the C1q assay, which detects antibodies that fix complement, offers a minimally invasive means of identifying patients at risk for transplant glomerulopathy and graft loss. (labome.org)
  • 05). The results of our study suggest that anti-beta2GPI antibodies may play a role in GMT formation, and this process might involve complement activation. (labome.org)
  • Complement Components and Antibodies. (rupress.org)
  • Expression of Complement Regulator Genes in Aβ1-42 Stimulated Human Neuroblastoma Cell. (freepatentsonline.com)
  • We used a custom-made single nucleotide polymorphism (SNP) genotyping assay consisting of 98 SNPs in 18 genes that encode components of the complement system. (frontiersin.org)
  • A recent linkage between the Ehlers-Danlos periodontal syndrome and heterozygous mutations in C1s and C1r genes has been reported recently. (ibs.fr)
  • Most cases of HUS are secondary to enteric infection with certain verotoxin-producing strains of Escherichia coli (particularly O157:H7) ( 14 ), but some cases are associated with genetic defects in complement inhibition on endothelium ( 15 ). (asnjournals.org)
  • C1r activates C1s so that it can, in turn, activate C2 and C4. (abcam.com)
  • In the first phase, a series of specific interactions leads to formation of intrinsic complement proteinase, termed C3 convertase. (medscape.com)
  • The two activated C1s subunits are then able to catalyze the assembly of the C3 convertase (complement C4b2a) from complements C2 and C4. (sigmaaldrich.com)
  • an α 2 -neuraminoglycoprotein that inhibits the enzymatic activity of C1 esterase, the activated first component of complement. (thefreedictionary.com)
  • The large fragment formed when COMPLEMENT C4 is cleaved by COMPLEMENT C1S. (labome.org)
  • This induces a conformational change, leading to autoactivation of C1r that then cleaves C1s to its active state. (medscape.com)
  • Interaction of Complement Defence Collagens C1q and Mannose-Binding Lectin with BMP-1/Tolloid-like Proteinases. (nih.gov)
  • Publications] Endo, M.: 'Glomerular deposition of mannose-binding lectin(MBL) indicates a novel mechanism of complement activation in IgA nephropathy. (nii.ac.jp)
  • This way, C1-inh prevents the proteolytic cleavage of later complement components C4 and C2 by C1 and MBL. (bionity.com)
  • This antibody reacts in ELISA when tested with activated C1s enzyme directly coated onto the microtiter well or in sandwich ELISA in combination with a polyclonal antibody in coat. (abcam.com)
  • The 13 SRCR domains are followed by two C1r/C1s Uegf Bmp1 domains separated by a 14th SRCR domain and a zona pellucida domain. (pnas.org)
  • The complement system, as an essential part of the innate immune response, eliminates invading microorganisms and dangerous host cells ( 1 , 2 ). (frontiersin.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)
  • Thus, the complement system provides the first line of defense before the adaptive immune response builds up. (jimmunol.org)
  • Moreover, the complement system bridges the innate and adaptive immunity, because the activated complement components facilitate the phagocytosis of pathogens by the host's leukocytes and initiate inflammatory reactions by recruiting and stimulating the cellular elements of the immune system. (jimmunol.org)
  • Complement C3b may be an opsonin for antigen-antibody complexes which helps prevent damage from the formation of large, insoluble immune aggregates. (sigmaaldrich.com)
  • Complement inhibitors are being studied as potential therapeutics for immune diseases and Alzheimer's. (sigmaaldrich.com)
  • EDTA/gelatin zymography method to identify C1s versus activated MMP-9 in plasma and immune complexes of patients with systemic lupus erythematosus. (abnova.com)
  • The complement system is a fundamental element of the innate immune system as well as the adaptive immune responses. (ukessays.com)
  • Complement C1r/C1s components (1 copy). (yale.edu)
  • There are deficiencies of each of the individual components of complement. (primaryimmune.org)
  • Despite uncontrolled auto-activation, it is important to note that levels of key complement components are low during an acute attack, because they are being consumed - indeed, low levels of C4 are a key diagnostic test for hereditary angioedema. (bionity.com)
  • These complement components form the final membrane attack complex (MAC). (thefreedictionary.com)
  • Dysregulation of the complement system has been implicated, but molecular mechanisms are incompletely understood. (frontiersin.org)
  • The result highlights a potential role for the complement system in the pathogenesis of PE and may help in defining prognostic and therapeutic subgroups of preeclamptic women. (frontiersin.org)
  • A unique feature of the AP is the presence of the only positive regulator in the complement system, Properdin. (primaryimmune.org)
  • It is important in controlling the C1r and C1s activation in the CP, and the MASPs in the LP along with several enzymes in the coagulation system. (primaryimmune.org)
  • The complement system is an important component of the innate immunity. (jimmunol.org)
  • Also, C4 and C2 cleavage goes unchecked, resulting in auto-activation of the complement system. (bionity.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)
  • Some genetic and molecular defects has been identified to be associated complement deficiencies. (pediatriconcall.com)
  • Complement deficiencies and associated genetic disorders and clinical manifestations is summarized in Table 1. (pediatriconcall.com)
  • Ehrlich's term for the thermolabile substance, normally present in serum, that is destructive to certain bacteria and other cells sensitized by a specific complement-fixing antibody. (thefreedictionary.com)
  • It inhibits complement activation at the yeast surface and, in addition, mediates adhesion of C. albicans to host endothelial cells. (labome.org)
  • It binds stoichiometrically to the active sites on both C1r and C1s to form a complex C1-INH-C1r-C1s-C1-INH and thus inhibits activated C1 ( 3 ). (rupress.org)
  • Complement activation also results in the formation of many biologically active complement fragments that act as anaphylatoxins, opsonins, or chemotactic factors. (thefreedictionary.com)
  • Cleavage from the REPLi combinatorial substrate collection by C1s enzymes Crazy type or mutant C1s forms (400?nM) were tested for his or her capability to cleave a combinatorial Andrographolide peptide substrate collection (REPLi, Mimotopes, Clayton, VIC, Australia) containing 3375 different peptides arranged in 512 swimming pools (17). (bassresearch.com)
  • These are widespread enzymes with triggering and signaling roles (eg, complement C1s and thrombin) and 'effector' roles (eg, elastase, trypsin and kinins). (ahajournals.org)
  • 2014 Blood), we hypothesized that TNT009, an antibody directed against complement component C1s, could stop haemolysis in CAD patients. (ehaweb.org)
  • Subsequent binding of the antibody to complement C1q subunits of C1 result in catalytically active C1s subunits. (sigmaaldrich.com)
  • The data on C1s, C1s2 and C1r are readily represented by straight hydrodynamic cylinders, but not C1r2 or C1r2C1s2. (biochemj.org)
  • It appears to kill CD-3 positive cells by inducing Fc mediated apoptosis, antibody mediated cytotoxicity and complement-dependent cytotoxicity. (drugbank.ca)
  • Some complement deficiencies caries risk of infection, other mainly associated with autoimmune diseases. (pediatriconcall.com)
  • Table 1 - Complement deficiencies and clinical associations. (pediatriconcall.com)
  • Symptoms of the disease can occur annually or several times weekly and are typically self-limiting, generally resolving within 72 hours but potentially lasting up to 5 days until complement C4 is depleted. (uspharmacist.com)
  • Oddly enough, alteration from the K628 residue in C1s got a marked influence on the cleavage of C4, reducing cleavage effectiveness for both mutants about fivefold. (bassresearch.com)
  • Andrographolide Enough time span of cleavage of C4 (1?M) by C1s forms in 1?nM was derived by incubating the elements for 0, 1, 2, 5, 15, 30, 60, and 120?min, following that your response mixtures were treated seeing that described over. (bassresearch.com)
  • Complement C4 (200 kDa) is composed of three polypeptide chains - α (93 kDa), β (78 kDa), and γ (33 kDa). (leebio.com)