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. (1/76)

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)

A novel PCR-based technique using expressed sequence tags and gene homology for murine genetic mapping: localization of the complement genes. (2/76)

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)

Interaction of C1q and mannan-binding lectin (MBL) with C1r, C1s, MBL-associated serine proteases 1 and 2, and the MBL-associated protein MAp19. (3/76)

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. (4/76)

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)

Identification of cDNA encoding a serine protease homologous to human complement C1r precursor from grafted mouse skin. (5/76)

We isolated a cDNA clone from grafted mouse skin that encodes a serine protease homologous to human C1r. The C1r protease is involved in the activation of the first component of the classical pathway in the complement system. In order to identify novel transcripts whose expression is regulated in grafted mouse skin, we first performed differential display reverse transcription polymerase chain reaction analysis and obtained 18 partial cDNA clones whose protein products are likely to play an important role in allograft rejection. One of these showed significant sequence homology with human complement C1r precursor. The other clones displayed no homology to any known sequences, however. Northern blot analysis demonstrated that the level of this transcript was upregulated in day 8 postgrafted skin. The full-length cDNA 2121 nucleotides in length obtained from screening a mouse skin cDNA library contained a single open reading frame encoding 707 amino acid residues with a calculated molecular weight of 80,732 Da. Its deduced amino acid sequence revealed an 81% identity and 89% similarity to the human C1r counterpart. In particular, mouse C1r contained His501, Asp559, and Ser656, which were conserved among this group of serine proteases. This protein was thus designated as mouse C1r. We have expressed a truncated fragment of C1r protein without the N-terminal hydrophobic sequence in Escherichia coli and generated a polyclonal antibody against it. Subsequent immunohistochemical analysis confirmed that mouse C1r was significantly expressed 8 d after the skin graft in both allografted and autografted skins, compared with normal skins. These collective data suggest that a component of the complement system, C1r, might contribute to the graft versus host immune responses in mice.  (+info)

Assembly and enzymatic properties of the catalytic domain of human complement protease C1r. (6/76)

The catalytic properties of C1r, the protease that mediates activation of the C1 complex of complement, are mediated by its C-terminal region, comprising two complement control protein (CCP) modules followed by a serine protease (SP) domain. Baculovirus-mediated expression was used to produce fragments containing the SP domain and either 2 CCP modules (CCP1/2-SP) or only the second CCP module (CCP2-SP). In each case, the wild-type species and two mutants stabilized in the proenzyme form by mutations at the cleavage site (R446Q) or at the active site serine residue (S637A), were produced. Both wild-type fragments were recovered as two-chain, activated proteases, whereas all mutants retained a single-chain, proenzyme structure, providing the first experimental evidence that C1r activation is an autolytic process. As shown by sedimentation velocity analysis, all CCP1/2-SP fragments were dimers (5.5-5.6 S), and all CCP2-SP fragments were monomers (3.2-3.4 S). Thus, CCP1 is essential to the assembly of the dimer, but formation of a stable dimer is not a prerequisite for self-activation. Activation of the R446Q mutants could be achieved by extrinsic cleavage by thermolysin, which cleaved the CCP2-SP species more efficiently than the CCP1/2-SP species and yielded enzymes with C1s-cleaving activities similar to their active wild-type counterparts. C1r and its activated fragments all cleaved C1s, with relative efficiencies in the order C1r < CCP1/2-SP < CCP2-SP, indicating that CCP1 is not involved in C1s recognition.  (+info)

C1 inhibitor: analysis of the role of amino acid residues within the reactive center loop in target protease recognition. (7/76)

Previous analysis of a naturally occurring C1 inhibitor P2 mutant (Ala(443)-->Val) indicated a role for P2 in specificity determination. To define this role and that of other reactive center loop residues, a number of different amino acids were introduced at P2, as well as at P6 (Ala(439)) and P8'/9' (Gln(452)Gln(453)). Ala(439)-->Val is a naturally occurring mutant observed in a patient with hereditary angioedema. Previous data suggested that Gln(452)Gln(453) might be a contact site for C1s. Reactivity of the inhibitors toward target (C1s, C1r, kallikrein, beta factor XIIa, and plasmin) and nontarget proteases (alpha-thrombin and trypsin) were studied. Substitution of P2 with bulky or charged residues resulted in decreased reactivity with all target proteases. Substitution with residues with hydrophobic or polar side chains resulted in decreased reactivity with some proteases, but in unaltered or increased reactivity with others. Second order rate constants for the reaction with C1s were determined for the mutants with activities most similar to the wild-type protein. The three P2 mutants showed reductions in rate from 3.35 x 10(5) M(-1)s(-1) for the wild type to 1.61, 1.29, and 0.63 x 10(5) for the Ser, Thr, and Val mutants, respectively. In contrast, the Ala(439)-->Val and the Gln(452)Gln(453)-->Ala mutants showed little difference in association rates with C1s, in comparison with the wild-type inhibitor. The data confirm the importance of P2 in specificity determination. However, the P6 position appears to be of little, if any, importance. Furthermore, it appears unlikely that Gln(452)Gln(453) comprise a portion of a protease contact site within the inhibitor.  (+info)

The role of the individual domains in the structure and function of the catalytic region of a modular serine protease, C1r. (8/76)

The first enzymatic event in the classical pathway of complement activation is autoactivation of the C1r subcomponent of the C1 complex. Activated C1r then cleaves and activates zymogen C1s. C1r is a multidomain serine protease consisting of N-terminal alpha region interacting with other subcomponents and C-terminal gammaB region mediating proteolytic activity. The gammaB region consists of two complement control protein modules (CCP1, CCP2) and a serine protease domain (SP). To clarify the role of the individual domains in the structural and functional properties of the gammaB region we produced the CCP1-CCP2-SP (gammaB), the CCP2-SP, and the SP fragments in recombinant form in Escherichia coli. We successfully renatured the inclusion body proteins. After renaturation all three fragments were obtained in activated form and showed esterolytic activity on synthetic substrates similar to each other. To study the self-activation process in detail zymogen mutant forms of the three fragments were constructed and expressed. Our major statement is that the ability of autoactivation and C1s cleavage is an inherent property of the SP domain. We observed that the CCP2 module significantly increases proteolytic activity of the SP domain on natural substrate, C1s. Therefore, we propose that CCP2 module provides accessory binding sites. Differential scanning calorimetric measurements demonstrated that CCP2 domain greatly stabilizes the structure of SP domain. Deletion of CCP1 domain from the CCP1-CCP2-SP fragment results in the loss of the dimeric structure. Our experiments also provided evidence that dimerization of C1r is not a prerequisite for autoactivation.  (+info)

1. The a- and b-chains of reduced and alkylated human complement subcomponent C1r were separated by high-pressure gel-permeation chromatography and isolated in good yield and in pure form. 2. CNBr cleavage of C1r b-chain yielded eight major peptides, which were purified by gel filtration and high-pressure reversed-phase chromatography. As determined from the sum of their amino acid compositions, these peptides accounted for a minimum molecular weight of 28 000, close to the value 29 100 calculated from the whole b-chain. 3. N-Terminal sequence determinations of C1r b-chain and its CNBr-cleavage peptides allowed the identification of about two-thirds of the amino acids of C1r b-chain. From our results, and on the basis of homology with other serine proteinases, an alignment of the eight CNBr-cleavage peptides from C1r b-chain is proposed. 4. The residues forming the charge-relay system of the active site of serine proteinases (His-57, Asp-102 and Ser-195 in the chymotrypsinogen numbering) are ...
Complement component 1 Q subcomponent-binding protein, mitochondrial is a protein that in humans is encoded by the C1QBP gene.[5][6][7] The human complement subcomponent C1q associates with C1r and C1s in order to yield the first component of the serum complement system. The protein encoded by this gene is known to bind to the globular heads of C1q molecules and inhibit C1 activation. This protein has also been identified as the p32 subunit of pre-mRNA splicing factor SF2, as well as a hyaluronic acid-binding protein.[7] ...
Recombinant Complement Component 1, R Subcomponent A (C1RA) Protein (His tag). Species: Mouse (Murine). Source: Insect Cells. Order product ABIN3125986.
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 ...
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)].. ...
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Originally Posted by dmatter I took about 10 Years off to Race Dirt Bikes, Separated My shoulders, Broke My Collar Bone, Broke my femur, Bruised about
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 ...
does this sound right to anyone with JAMMIN/PICCO experience...??? tore down my JAMMIN .28 after a successful race season. found that the pistons
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 ...
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 ...
The following information has been self-reported by the entity to which it relates for the purpose of assignment of a unique identifier (CUI). The information has not been verified nor has the entity been authenticated, credentialed, verified, or investigated in any way ...
VueScan, tarayıcınızla gelen yazılımı değiştiren bir uygulamadır. VueScan, Lexmark 5400 ile uyumludur Windows x86, Windows x64 ve Mac OS Xdeki.
The temptation for Kyosho to recreate the same high performance and style of a true AWD (all-wheel-drive) in Mini-Z size was simply too much to resist. The advanced design combines a shaft driven AWD with a precision 4-wheel independent suspension using a
VueScan, tarayıcınızla gelen yazılımı değiştiren bir uygulamadır. VueScan, Plustek EasyScan 400 ile uyumludur Windows x86 ve Windows x64deki.
This is directed mostly to Foodie. I tried to e-mail him but it didnt go through. I really like Arlaud 99 Morey-St.-Denis Les Ruchots (a premier cru) and slightly behind that the Arlaud 99 Morey-St.-Denis Millandes (another premier cru). I also found Arlauds 99 Clos-St.-Denis (grand cru) very appealing and better than the 98 of the same wine. I was surprised that Serena Sutcliffe in a book on Burgundy wines made rather a snotty or snobbish and dismissive comment about Arlaud wines. I know several distributors and retailers who are enthusiastic about this producer and go to extra pains to acquaint people with his wines because Morey-St.-Denis isnt as well known as its neighbors. (I actually prefer the Arlaud M-S-D wines to his Gevrey wines.) Foodie, do you have any experience with Arlauds 99 wines? What is your opinion of them? I may not have enough experience with red burgundies (although I keep trying to increase it)to know if his wines are faithful to terroir or corrupted by modern ...
Clos de la Roche Grand Cru ( Domaine Arlaud Père et Fils ) 2005 - Red wine form France - Visit Winesworlds wine guide with dices and tasting notes
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...
The MUC1 tumor associated antigen is highly expressed on a range of tumors. Its broad distribution on primary tumors and metastases renders it an attractive target for immunotherapy. After synthesis MUC1 is cleaved, yielding a large soluble extracellular alpha subunit containing the tandem repeats array (TRA) domain specifically bound, via non-covalent interaction, to a smaller beta subunit containing the transmembrane and cytoplasmic domains. Thus far, inconclusive efficacy has been reported for anti-MUC1 antibodies directed against the soluble alpha subunit. Targeting the cell bound beta subunit, may bypass limitations posed by circulating TRA domains. MUC1s signal peptide (SP) domain promiscuously binds multiple MHC class II and Class I alleles, which upon vaccination, generated robust T-cell immunity against MUC1-positive tumors. This is a first demonstration of non-MHC associated, MUC1 specific, cell surfaces presence for MUC1 SP domain. Polyclonal and monoclonal antibodies generated ...
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Mellors, R C., (slow) Virus infection, autoimmunity, and and lymphoma, an experimental model of human disease. (1969). Subject Strain Bibliography 1969. 1878 ...
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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
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
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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.
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 ...
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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
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Guzman, R C.; Osborn, R C.; Yang, J; D; and Nandi, S, Transplantation of mouse mammary epithelial cells grown in primary collagen gel cultures. (1982). Subject Strain Bibliography 1982. 2450 ...
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 ...
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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, ...
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Caravan Mirror Monitor Reversing Backup Camera Kit 7 Caravan Mirror Monitor Rear View Monitor Camera Kit K7FV87T1C02 Kit Includes • 1 x R-MFV87 7inch Mirror Monitor With Touch Buttons • 1 x R-C14102 120° or R-C0236 90° or C0260 60° or R-CS212 30° Sharp CCD Camera • 1 x 4m Spring Trailer
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.
Shaft driven AWD with four-wheel independent suspension in Kyoshos micro-sized Mini-Z AWD realizes dynamic performance with an advanced new style. The aggressive design of the front spoiler combines with a mock carbon bonnet (decal finish), side steps, a
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 ...
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This is a bottle of Change Purple Acrylic R/C Racing Finish paint from Pactra! Pactra R/C Acryl is specially formulated for airbrush application and... PACRC5712
3 years ago Disclaimer: We are not responsible for any financial loss, data loss, downgrade in search engine rankings, missed customers, undeliverable email or any other damages that you may suffer upon the expiration of www.donationto.com. For more information please refer to section 17.c.1a of our User Agreement. This is your final notice to renew www.donationto.com: https://www.netcentraldomain.com/?n=www.donationto.com&r=c In the event that www.donationto.com expires, we reserve the right to offer your listing to competing businesses in the same niche and region after 3 business days on an auction basis. This is the final communication that we are required to send out regarding the expiration of www.donationto.com Secure Online Payment: https://www.netcentraldomain.com/?n=www.donationto.com&r=c All services will be automatically restored on www.donationto.com if payment is received in full before expiration. Thank you for your cooperation.. ...
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
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Mace H A , Peterson R C , The Canadian Mineralogist , 33 (1995) p.7-11, The crystal structure of fichtelite, a naturally occuring hydrocarbon ...
EX 28.G Q9 Find the value of m for which the line vector {r} = ( {i}+2 hat{k} ) + lambda ( 2 hat{i}-m hat{j}-3 hat{k} ) is parallel to the plane vector {r} c.
GeneGene-level p-value [a] GS GS p-value MM MM p-valueRank [b] miRNA r [c] p-value FDR GPD1L 2.67E-02 −0.23 1.06E-03 0.90 1.29E-71 1 hsa-miR-210 −0.17 7.37E-03 7.91E-02 CCDC50 2.12E-02 −0.20 4.68E-03 0.85 6.79E-58 2 hsa-miR-501-3p −0.19 3.93E-03 5.05E-02 NAALAD2 8.05E-03 −0.24 5.76E-04 0.85 1.47E-57 3 hsa-miR-140-3p −0.29 1.97E-05 7.98E-04 ALDH1L1 4.94E-03 −0.28 7.13E-05 0.85 3.15E-57 4 hsa-miR-342-5p −0.27 5.13E-05 1. 76E-03 ADH1B 3.14E-02 −0.27 8.82E-05 0.85 4.39E-56 5 - - - - ADH1A 5.00E-02 −0.26 1.83E-04 0.82 2.34E-50 6 hsa-miR-590-3p −0.09 1.06E-01 4.46E-01 PCCA 2.38E-02 −0.21 2.68E-03 0.82 3.87E-49 8 hsa-miR-301b −0.25 2.08E-04 5.42E-03 ORMDL3 3.87E-03 −0.29 3.04E-05 0.80 3.29E-45 9 hsa-miR-362-3p −0.20 2.12E-03 3.23E-02EPB41L4B [d] 7.42E-05 −0.29 3. 47E-05 0.67 2.63E-27 28 has-miR-3613-3p −0.11 6.46E-02 3.4E-01GS, gene significance; MM, module membership. [score:1] ...
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 ...
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.... ...
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
Post 7611401 - HeliFreak.com is the place to learn about Radio Controlled Helicopters and to socialize with others who are also learning and flying R/C Helis. Our Motto is Fun, Learning, Friendship and Mutual Respect. Come join in on the fun and enjoy the free exchange of knowledge and experience.
Post 2783075 - HeliFreak.com is the place to learn about Radio Controlled Helicopters and to socialize with others who are also learning and flying R/C Helis. Our Motto is Fun, Learning, Friendship and Mutual Respect. Come join in on the fun and enjoy the free exchange of knowledge and experience.
who took Tom C o r c o r a n , I s a t between L ord and Lady H a r l e ch. close b y . The McNam a r a s and e s c o r t e d me i n . in front of me. met me a t the f r o n t door And the I was l o s t years For But a f t e r of I d o n ...
Thanks for Bearbound for another great set of shots. This cub is uber sexy anyway, but to have him duct tape gagged and in a strict hogtie...so fucking hot ...
... domain and the complement C1r-like (cEGF) domain, which was first identified in the human complement protease C1r. C1r is a ... Circolo A, Garnier G, Volanakis JE (2003). "A novel murine complement-related gene encoding a C1r-like serum protein". ... like Module of Human Complement Protease C1r, an Atypical Member of the EGF Family". Biochemistry. 37 (5): 1204-14. doi:10.1021 ... highly specific serine protease initiating the classical pathway of complement activation during immune response. Both the hEGF ...
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 ... Complement component 1 Q subcomponent-binding protein, mitochondrial is a protein that in humans is encoded by the C1QBP gene. ... "Entrez Gene: C1QBP complement component 1, q subcomponent binding protein". Jiang, Jianzhong (1999). "rystal structure of human ...
C3 and C1R are involved in the complement system while SH2B3 is a protein which links the T-cell receptor signal to the ... Predicted functional partners of FAM155B include C3, SH2B3, and C1R-- all of which are associated with immune functions. ...
It is the first component of the classical complement pathway and is composed of the subcomponents C1q, C1r and C1s. The C1 ... The C1 complex (complement component 1, C1) is a protein complex involved in the complement system. ... Active C1r cleaves the C1s molecules, activating them. Active C1s splits C4 and then C2, producing C4a, C4b, C2a and C2b. The ... 2001). "The complement system and innate immunity". Immunobiology: The Immune System in Health and Disease. New York: Garland ...
... 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 ...
C1R / C1S C4 - C4a C2 Mannan-binding lectin pathway MASP1 / MASP2 Mannan-binding lectin Alternative complement pathway Factor B ... 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 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 ...
Kusumoto H, Hirosawa S, Salier JP, Hagen FS, Kurachi K (October 1988). "Human genes for complement components C1r and C1s in a ... Complement component 1s (EC 3.4.21.42, C1 esterase, activated complement C1s, complement C overbar 1r, C1s) is a protein ... Tosi M, Duponchel C, Meo T, Couture-Tosi E (August 1989). "Complement genes C1r and C1s feature an intronless serine protease ... Sim RB (1981). "The human complement system serine proteases C1r and C1s and their proenzymes". Methods in Enzymology. 80 Pt C ...
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 together with C1r and C1s form the C1 complex. Antibodies of the adaptive immune system can bind antigen, forming an ...
Complement C1r subcomponent (EC 3.4.21.41, activated complement C1r, C overbar 1r esterase, C1r) is a protein involved in the ... Human C1R genome location and C1R gene details page in the UCSC Genome Browser. Complement+C1r at the US National Library of ... C1r along with C1q and C1s form the C1 complex, which is the first component of the serum complement system. C1r is an enzyme ... "Entrez Gene: C1R complement component 1, r subcomponent". Thielens NM, Enrie K, Lacroix M, Jaquinod M, Hernandez JF, Esser AF, ...
... 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 ...
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 ...
Zipfel P., Hallström T., Riesbeck K. (2013). "Human complement control and complement evasion by pathogenic microbes - Tipping ... More specifically, BBK32 interacts with C1r subunit of C1. C-terminal domain of the BBK32 protein mediates the binding. As a ... This is part of a complement system evasion strategy that leads to downstream blocking of immune response. In addition, ... Borrelia burgdorferi has a strategy to directly inhibit the classical pathway of complement system. A borrelial lipoprotein ...
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 ...
... complement c1 MeSH D12.776.124.486.274.050.270 - complement c1q MeSH D12.776.124.486.274.050.280 - complement c1r MeSH D12.776. ... complement c1r MeSH D12.776.124.486.274.860.290 - complement c1s MeSH D12.776.124.486.274.860.387 - complement c3-c5 ... complement c2 MeSH D12.776.124.486.274.150.500 - complement c2a MeSH D12.776.124.486.274.150.750 - complement c2b MeSH D12.776. ... complement c3c MeSH D12.776.124.486.274.250.260.750 - complement c3d MeSH D12.776.124.486.274.350 - complement c4 MeSH D12.776. ...
C1r is a serine protease. They then cleave C1s (another serine protease). The C1r2s2 component now splits C4 and then C2, ... 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 ...
Such binding leads to conformational changes in the C1q molecule, which leads to the activation of two C1r molecules. C1r is a ... 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 ...
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- ... Cherubism COPA defect Otulipenia/ORAS The complement system is part of the innate as well as the adaptive immune system; it is ... MASP2 deficiency Complement receptor 3 deficiency Membrane cofactor protein (CD46) deficiency Membrane attack complex inhibitor ...
Complement decay-accelerating factor (Antigen CD55) belongs to the Cromer blood group system and is associated with Cr(a), Dr(a ... C1R, C1S, C2, C4BPA, C4BPB, C6, C7, CD46, CD55, CFB, CFH, CFHR1, CFHR2, CFHR3, CFHR4, CFHR5, CR1, CR1L, CR2, CSMD1, CSMD2, ... Complement receptor type 1 (C3b/C4b receptor) (Antigen CD35) belongs to the Knops blood group system and is associated with Kn( ... Complement components may activate B cells through CD21. CD21 is part of a large signal-transduction complex that also involves ...
Cleavage of complement C3 by a free floating convertase, thrombin, plasmin or even a bacterial enzyme leads to formation of C3a ... In the classical pathway, this is by sequential proteolytic activation of proteins within the C1 complex (C1q, C1r, C1s) in ... DAF protects host cells from damage by autologous complement. DAF acts on C2b and Bb and dissociates them rapidly from C4b and ... Hourcade D (2006). "The Role of Properdin in the Assembly of the Alternative Pathway C3 Convertases of Complement". J Biol Chem ...
Zipfel, P. F., Hallström, T., & Riesbeck, K. (2013). Human complement control and complement evasion by pathogenic microbes- ... C1r ja C1s-i aktivatsioonile koos C1s esteraasi formuleerumisega järgneb C4 ja C2 lõhustumine, mis vabastab väikseid peptiide ... 1,0 1,1 1,2 1,3 Rus, H., Cudrici, C., & Niculescu, F. (2005). The role of the complement system in innate immunity. Immunologic ... 7,0 7,1 Lambris, J. D., Ricklin, D., & Geisbrecht, B. V. (2008). Complement evasion by human pathogens. Nature Reviews. ...
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 ...
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 ...
Označujemo jih z veliko črko C (kratica za complement) in številko. Ko signal sproži spremembo enega od začetnih proencimov v ... Z razcepom estrske vezi aktivira dve drugi podenoti, C1r, ti pa nato podenoti C1s (prav tako serinski esterazi). Ti cepita ... ki je prisotna v krvi in se nepovratno veže na C1r ter C1q, s čemer prepreči aktivacijo C2 in C4. Nazadnje pa dejstvo, da je za ...
For two central idempotents e and f the complement ¬e = 1 − e and the join and meet are given by e ∨ f = e + f − ef and e ∧ f ... If a decomposition R = c1R ⊕ c2R ⊕ ... ⊕ cnR exists with each ci a centrally primitive idempotent, then R is a direct sum of ...
Browse our Complement Component C1r Lysate catalog backed by our Guarantee+. ... Complement Component C1r Lysates available through Novus Biologicals. ... Alternate Names for Complement Component C1r Lysates. Complement Component C1r lysate, C1R lysate, complement C1r subcomponent ... We offer Complement Component C1r Lysates for use in common research applications: Western Blot. Each Complement Component C1r ...
Goat Polyclonal Anti-Complement Component C1r Antibody [Unconjugated]. Validated: WB, IP. Tested Reactivity: Human. 100% ... Additional Complement Component C1r Products. Complement Component C1r AF1807 * Complement Component C1r Antibodies ... Home » Complement Component C1r » Complement Component C1r Antibodies » Complement Component C1r Antibody [Unconjugated] ... Blogs on Complement Component C1r. There are no specific blogs for Complement Component C1r, but you can read our latest blog ...
... retention of C1r fragments inside the cell, secretion of aggregates, or a new C1r cleavage site. Overexpression of C1R variants ... In contrast to C1r wild type, and with the exception of a C1R missense variant disabling a C1q binding site, pEDS variants had ... In contrast to C1r wild type, and with the exception of a C1R missense mutation disabling a C1q binding site, pEDS mutations ... The central elements in the pathogenesis of pEDS appear to be the intracellular activation of C1r and/or C1s, and extracellular ...
Anti-C1r-LP, Anti-C1r-like serine protease analog protein, Anti-C1r-like protein, Anti-CLSPa. CAS null. Molecular Weight null. ... Synonyms: Anti-Complement C1r subcomponent-like protein precursor, ... Anti-Complement C1r subcomponent-like protein precursor, Anti-C1r-LP, Anti-C1r-like serine protease analog protein, Anti-C1r- ...
c1s (complement C1r) gene expression in Xenopus laevis, NF stage 29 & 30 embryo, in situ hybridization, lateral view, anterior ...
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 ...
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 subcomponents C1r and C1s and their activated forms C-1r and C-1s were each found to have mol.wts. in dissociating solvents ... The structure and enzymic activities of the C1r and C1s subcomponents of C1, the first component of human serum complement ...
C1r complement component. - Find MSDS or SDS, a COA, data sheets and more information. ... Complement C1r, Human, Activated, Two-Chain Form, CAS 80295-34-7, is a native, activated, ... C1r complement component.. More,, Complement C1r, Human, Activated, Two-Chain Form, CAS 80295-34-7, is a native, activated, C1r ... Native, human, activated, C1r complement component. Generally the non-activated form composed of a single-chain glycoprotein. ...
C1R gene. complement C1r. Enable Javascript to view the expand/collapse boxes.. Open All Close All ... The encoded protein is a proteolytic subunit in the complement system C1 complex. The complement system acts as a mediator in ... C1r B chain is a serine protease that combines with C1q and C1s to form C1, the first component of the classical pathway of the ...
Abcams C1r ELISA Kit suitable for Cell culture supernatant, Saliva, Milk, Urine, Serum, Plasma, Cerebral Spinal Fluid in human ... A Complement C1r specific antibody has been precoated onto 96-well plates and blocked. Standards or test samples are added to ... Abcams Complement C1r Human in vitro ELISA (Enzyme-Linked Immunosorbent Assay) kit is designed for the quantitative ... The density of yellow coloration is directly proportional to the amount of Complement C1r captured in plate. ...
Complement C1r/C1s components (1 copy). - Complement-activating component of Ra-reactive factor (RARF) (1 copy).. - Complement ...
Summary of C1R () expression in human tissue. Mainly cytoplasmic expression and plasma positivity in several different tissue ... Complement C1r subcomponent Complement C1r subcomponent heavy chain Complement C1r subcomponent light chain. ... Complement C1r subcomponent; cDNA FLJ54471, highly similar to Complement C1r subcomponent (EC 3.4.21.41) ... Complement C1r. Protein classi. Protein class the gene product belongs to according to selected gene lists. List of protein ...
... like module of human complement protease C1r, an atypical member of the EGF family. ... COMPLEMENT PROTEASE C1R A 53 Homo sapiens EC#: 3.4.21.41 IUBMB Fragment: EGF-LIKE MODULE Details: CALCIUM-BINDING CONSENSUS ... STRUCTURE OF THE EGF-LIKE MODULE OF HUMAN C1R, NMR, 19 STRUCTURES. *DOI: 10.2210/pdb1apq/pdb ...
Fingerprint Dive into the research topics of Interaction between separated consecutive complement control modules of human C1r ... Interaction between separated consecutive complement control modules of human C1r: Implications for dimerization of the full- ... T1 - Interaction between separated consecutive complement control modules of human C1r. T2 - Implications for dimerization of ... Interaction between separated consecutive complement control modules of human C1r: Implications for dimerization of the full- ...
keywords = "C1r catalytic domain, Complement control protein, Complement system, Mechanism of C1r autoactivation, Modular ... Structure of the active catalytic region of C1r. Together they form a unique fingerprint. * Complement C1r Medicine & Life ... Fingerprint Dive into the research topics of Revisiting the mechanism of the autoactivation of the complement protease C1r in ... Revisiting the mechanism of the autoactivation of the complement protease C1r in the C1 complex: Structure of the active ...
Complement C1 Esterase inhibitor. 15-35. Complement C1r Component. -. Complement C4 Binding Protein. -. ...
Complement C1r [Human].... ... oligos for SYBR Green, TaqMan, LNA spiked ... assays. Highly specific primers are designed by ... Neurobiology of Alzheimers Disease from PromegaHuman Complement C1r from BIOPURBeacon Designer from PREMIER Biosoft ... Mouse Anti-Human Complement factor B Monoclonal Antibody, Unconjugated, Clone 9B6 from AntibodyShop A/S. 11. Mouse Anti-Human ...
Human Complement C1r from BIOPURbiotin-4-fluorescein from Molecular Probes (Invitrogen)LVF Kit: LVF-UV-HL, LVF-CUV-ADP, FHS-LVF ... This complement ... (Date:10/11/2017)... ... October 11, 2017 , ... Proscia Inc ., ... a Webinar titled, "Pathology is going ...
... the first component of the classical pathway of the complement system. ... Complement protein C1r is a serine protease that combines with C1q and C1s to form C1, ... During complement activation, C1r and C1s act to cleave complement proteins C2 and C4. ... Complement protein C1r is a serine protease that combines with C1q and C1s to form C1, the first component of the classical ...
SA = signal/anchor sequence; LDLR = LDL receptorlike domain; Muc = Mucin-domain; Meprin = Meprinlike domain; C1r/s = Complement ... SA = signal/anchor sequence; LDLR = LDL receptorlike domain; Muc = Mucin-domain; Meprin = Meprinlike domain; C1r/s = Complement ...
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 C1r subcomponent C1R P00736 DDA 0.02 0.55 Inter-α-trypsin inhibitor heavy chain 3 ITIH3 C9JX84 DIA 0.007 1.56 ... 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 ...
Complement subcomponents C1r/C1sC1s, uegf, bone morphogenetic protein. CUZD1:. CUB and zona pellucida-like domains-containing ... CUB (complement subcomponents C1r/C1sC1s, Uegf, bone morphogenetic protein) domains [45] are structural motifs frequently ... "Identification of the C1q-binding sites of human C1r and C1s. A refined three-dimensional model of the C1 complex of complement ... CUB-containing proteins are multifunctional and play a role in embryogenenic signalling [48], complement activation [47, 49], ...
Sim RB (1981). "The human complement system serine proteases C1r and C1s and their proenzymes". Methods in Enzymology. 80 Pt C ... Complement component 1s (EC 3.4.21.42, C1 esterase, activated complement C1s, complement C overbar 1r, C1s) is a protein ... complement activation, lectin pathway. • complement activation. • regulation of complement activation. Sources:Amigo / QuickGO ... Tosi M, Duponchel C, Meo T, Couture-Tosi E (August 1989). "Complement genes C1r and C1s feature an intronless serine protease ...
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 ...
Rat Complement from Pel-Freez Biologicals,Mature rat whole blood (Sprague-Dawley, averaging 7-8 weeks of age, either sex) is ... Human Complement C1r from BIOPUR. 11. Goat Anti-Mouse Complement Component C5a Polyclonal Antibody, Unconjugated from R&D ... Mouse Complement from Equitech-Bio. 5. Guinea Pig Complement from Pel-Freez Biologicals. 6. Mouse Complement from Pel-Freez ... Rabbit Complement from Pel-Freez Biologicals. 8. Rabbit Complement from Pel-Freez Biologicals. 9. 3-4 Week Baby Rabbit ...
This gene encodes a major constituent of the human complement subcomponent C1q. C1q associates with C1r and C1s in order to ... C4A (Complement Fragment 4a): C4A Antikörper C4A ELISA Kits C4A Proteine C4 - Complement 4: C4 Antikörper C4 ELISA Kits C4 ... CR1 (Complement Component Receptor 1 (CD35)): CR1 Antikörper CR1 ELISA Kits CFP (Complement Factor P): CFP Antikörper CFP ELISA ... C7 (Complement Component C7): C7 Antikörper C7 ELISA Kits C7 Proteine C8A (Complement Component 8, alpha Polypeptide): C8A ...
C1R , Review 6 of antibodies with 5 images, 2 of protocols, relevant gene trends, publication graphs, tissue expression data, ... the first component of the classical pathway of the complement system. ... Human C1R antibody; Homo sapiens C1R antibody. D3DUT5. 93.0%. ARP60068. Human C1R antibody; Homo sapiens C1R antibody. F5GWL0. ... Human C1R antibody; Homo sapiens C1R antibody. F5GZR1. 93.0%. ARP60068. Human C1R antibody; Homo sapiens C1R antibody. F5H2D0. ...
C1R. Uniprot ID. P00736. Uniprot Name. Complement C1r subcomponent. Molecular Weight. 80118.04 Da. ... 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- ...
  • The classical complement pathway plays a major role in innate immunity against infection. (novusbio.com)
  • This pathway is triggered by C1, a multimolecular complex composed of the recognition protein C1q and two serine proteases, C1r and C1s. (novusbio.com)
  • Ensuing C1q binding to classical complement pathway activators, each C1r proenzyme monomer is activated by cleavage into two disulfide-linked fragments of M.W. 60,000 and 35,000. (merckmillipore.com)
  • Activated C1r continues activation via classical pathway by cleaving, and thus activating C1s. (merckmillipore.com)
  • C1r 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. (nih.gov)
  • C1r is a modular serine protease which is the autoactivating component of the C1 complex of the classical pathway of the complement system. (elsevier.com)
  • This gene encodes a serine protease that functions as a component of the lectin pathway of complement activation. (antikoerper-online.de)
  • The complement pathway plays an essential role in the innate and adaptive immune response. (antikoerper-online.de)
  • This gene encodes complement factor B, a component of the alternative pathway of complement activation. (antikoerper-online.de)
  • C1r is a highly specific serine protease initiating the classical pathway of complement activation during immune response. (wikipedia.org)
  • 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)
  • Thus, blocking the classical complement pathway with a highly specific and potent synthetic inhibitor of the activated C1 complex appears to be an effective mean to preserve ischemic myocardium from injury following reperfusion. (jimmunol.org)
  • The classical complement pathway can be activated by certain sensitizing Abs, cardiac mitochondrial particles, cardiolipin, or the fibrinolytic system. (jimmunol.org)
  • In this regard, blocking of the classical complement pathway with exogenous C1 esterase inhibitor isolated from plasma exerted cardioprotective effects following ischemia and reperfusion ( 6 , 7 ). (jimmunol.org)
  • C1 esterase inhibitor is an endogenous inhibitor of the classical complement pathway which might be insufficiently active in situations like ischemia and reperfusion, since C1 esterase inhibitor can be inactivated by neutrophil released proteases such as elastase ( 12 ). (jimmunol.org)
  • Further, beside its inhibitory effect on the activated C1 complex (C1q, C1r, C1s), C1 esterase inhibitor has blocking potency on the mannose-binding lectin (MBL) pathway, the kallikrein system, the coagulation system, and the fibrinolytic system. (jimmunol.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 sequence of reactions, each being the catalyst for the next, that leads to the terminal complement pathway and cell lysis. (thefreedictionary.com)
  • 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)
  • 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)
  • 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)
  • 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)
  • 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)
  • 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)
  • Component C2 is a serum glycoprotein that functions as part of the classical pathway of the complement system. (genecards.org)
  • Among its related pathways are Complement and coagulation cascades and Immune response Lectin induced complement pathway . (genecards.org)
  • Component C2 which is part of the classical pathway of the complement system is cleaved by activated factor C1 into two fragments: C2b and C2a. (genecards.org)
  • This results in inhibition of the classical and lectin pathway of complement activation, probably due to interference with binding of C2a to C4b such that C3 convertase cannot be formed. (genecards.org)
  • 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)
  • 1 MBL mediates lectin dependent activation of the complement pathway, 1 and has an important role in host defence against micro-organisms. (bmj.com)
  • Three biochemical pathways activate the complement system: the classical complement pathway , the alternative complement pathway , and the mannose-binding lectin pathway . (wikidoc.org)
  • The classical complement pathway typically requires antibodies for activation (specific immune response), while the alternative and mannose-binding lectin pathways can be activated by C3 hydrolysis or antigens without the presence of antibodies (non-specific immune response). (wikidoc.org)
  • The classical pathway is triggered by activation of the C1-complex (which consists of one molecule C1q and two molecules C1r and C1s), either by C1q's binding to antibodies from classes M and G, complexed with antigens , or by its binding C1q to the surface of the pathogen. (wikidoc.org)
  • C1-inh irreversibly binds to and inactivates C1r and C1s proteinases in the C1 complex of classical pathway of complement. (bionity.com)
  • Both C1r and C1s activation involve cleavage of a specific Arg-Ile bond, converting single-chain proenzymes into active proteases of disulfide bond-linked chains (A and B) (2). (novusbio.com)
  • These genes code for complement 1 subunits C1r and C1s, serine proteases that play a key role in the innate immune response. (frontiersin.org)
  • Complement C1s and C1r proteases were purified from human and rabbit plasma according to a procedure described by Lane et al. (jimmunol.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)
  • 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)
  • C1r and C1s have a similar protein domain structure with the N-terminal interaction domain including CUB (complement C1r/C1s, Uegf, Bmp1) and EGF (epidermal growth factor) modules in a CUB1-EGF-CUB2 arrangement, two complement-control-protein modules CCP1 and CCP2, and a serine protease (SP) domain ( Figure 1A , Table 1 ). (frontiersin.org)
  • The encoded protein is a proteolytic subunit in the complement system C1 complex. (nih.gov)
  • Complement control protein modules (CCP) typically mediate protein:protein interaction during immune response in vertebrates. (elsevier.com)
  • This fragment contains the C-terminal serine protease (SP) domain and the preceding two complement control protein (CCP) modules. (elsevier.com)
  • The encoded protein is a component of the alternative complement. (antikoerper-online.de)
  • The protein encoded by this gene is the fifth component of complement, which plays an important role in inflammatory and cell killing processes. (antikoerper-online.de)
  • 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)
  • Complement component 1 Q subcomponent-binding protein, mitochondrial is a protein that in humans is encoded by the C1QBP gene . (wikipedia.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)
  • 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)
  • Description: A sandwich ELISA for quantitative measurement of Rabbit Complement C1r subcomponent like protein(C1RL) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. (applesci.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 1 inhibitor (C1-INH) * is a critically important protein that controls activation of multiple plasma mediator pathways ( 1 ). (rupress.org)
  • In this study, a proHp-cleaving enzyme was isolated from human serum and identified as complement C1r-like protein (C1rLP). (diva-portal.org)
  • Patients with complement deficiencies encounter clinical problems that depend on the role of the specific complement protein in normal function. (primaryimmune.org)
  • 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)
  • Novel substrates identified and biochemically validated include insulin-like growth factor binding protein-4, complement C1r component A, galectin-1, dickkopf-related protein-3, and thrombospondin-2. (mcponline.org)
  • 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)
  • C2 (Complement C2) is a Protein Coding gene. (genecards.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)
  • A complement protein attacking an invader. (wikidoc.org)
  • Over 20 proteins and protein fragments make up the complement system, including serum proteins, serosal proteins, and cell membrane receptors. (wikidoc.org)
  • 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)
  • There are currently no images for Complement Component C1r Antibody (AF1807). (novusbio.com)
  • A Complement C1r specific antibody has been precoated onto 96-well plates and blocked. (abcam.com)
  • Standards or test samples are added to the wells and subsequently a Complement C1r specific biotinylated detection antibody is added and then followed by washing with wash buffer. (abcam.com)
  • If the serum contains antibody, i.e. is positive, an antibody-antigen complex is formed which also binds (fixes) complement. (thefreedictionary.com)
  • If antibody was not present in stage 1, then the free complement lyses the sensitized sheep red blood cells. (thefreedictionary.com)
  • Abcam's Complement C1r Human in vitro ELISA (Enzyme-Linked Immunosorbent Assay) kit is designed for the quantitative measurement of Complement C1r concentrations in plasma, serum, saliva, urine, milk and cell culture supernatants. (abcam.com)
  • The human complement subcomponent C1q associates with C1r and C1s in order to yield the first component of the serum complement system. (wikipedia.org)
  • In the first, also referred to as the test system, antigen is mixed usually with serial dilutions of a test serum in the presence of complement. (thefreedictionary.com)
  • 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)
  • Complement is the term used to describe a group of serum proteins that are critically important in our defense against infection. (primaryimmune.org)
  • The inhibition of plasmin, plasma kallikrein, plasma permeability factor, and C1r subcomponent of the first component of complement by serum C1 esterase inhibitor. (labcorp.com)
  • During inflammation, vascular permeability is increased by various proteolytic events, such as the generation of bradykinin, that augment local tissue responses by enabling tissue penetration of serum proteins, including complement and acute-phase proteins. (sciencemag.org)
  • During complement activation, C1r and C1s act to cleave complement proteins C2 and C4. (quidel.com)
  • These modules have been identified more than 140 times in over 20 proteins, including 12 proteins of the complement system. (embl-heidelberg.de)
  • Complement comprises 25 to 30 discrete proteins, labeled numerically as C1 to C9, and by letters, i.e. (thefreedictionary.com)
  • The complement system consists of more than 30 proteins, present in blood and tissues, as well as other proteins anchored on the surfaces of cells. (primaryimmune.org)
  • 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)
  • The complement system consists of a number of small proteins found in the blood, normally circulating as inactive zymogens . (wikidoc.org)
  • Of the proteins identified in normal skin, about half were cleaved, and phorbol ester-induced inflammation increased the proportion of cleaved proteins, including chemokines and complement proteins, that were processed at previously uncharacterized sites. (sciencemag.org)
  • Hereditary angioedema (HAE) is an episodic swelling disease associated with the deficiency or malfunction of complement 1 esterase inhibitor (C1-INH). (arupconsult.com)
  • See Complement C1 Esterase Inhibitor [004648] . (labcorp.com)
  • This C1 esterase inhibitor complements Shire's FIRAZYR icatibant injection for the treatment of acute HAE attacks. (thefreedictionary.com)
  • Using NMR chemical shift perturbation mapping, we present previously lacking experimental evidence for intermolecular interactions between the CCP1 and CCP2 modules of the human C1r serine protease (SP). (elsevier.com)
  • The two main proposed types of EGF-like domains are the human EGF-like (hEGF) domain and the complement C1r-like (cEGF) domain, which was first identified in the human complement protease C1r. (wikipedia.org)
  • Until now there was no specific inhibitor of the classical complement system available. (jimmunol.org)
  • Therefore, the major purposes of this study were to determine the effects of a novel synthetic small molecule inhibitor of C1s on 1) complement activation, 2) myocardial tissue injury, and 3) neutrophil accumulation in a well-established rabbit model of myocardial ischemia and reperfusion. (jimmunol.org)
  • 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)
  • 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)
  • This gene encodes a major constituent of the human complement subcomponent C1q. (antikoerper-online.de)
  • 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)
  • 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)
  • 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)
  • 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)
  • Activation of the complement pathways. (medscape.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)
  • 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)
  • The classical and alternative complement pathways. (wikidoc.org)
  • Although named after its complement inhibitory activity, C1-inh also inhibits proteinases of the fibrinolytic, clotting, and kinin pathways. (bionity.com)
  • Complement Components and Antibodies. (rupress.org)
  • The complement system is a biochemical cascade that helps, or complements, the ability of antibodies to clear pathogens from an organism. (wikipathways.org)
  • In the early 20th century, this controversy was resolved when it was understood that complement can act in combination with specific antibodies, or on its own in a non-specific way. (wikidoc.org)
  • B-LCL by gamma irradiation followed by selection for Class I monoclonal antibodies and complement. (atcc.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)
  • In direct ELISAs, less than 1% cross-reactivity with recombinant human Complement Component C1s is observed. (novusbio.com)
  • In direct ELISAs and Western blots, approximately 10% cross-reactivity with recombinant mouse C1rLP is observed and less than 5% cross-reactivity with recombinant human (rh) C1R, rhC1S, and rhMASP-3 is observed. (thermofisher.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)
  • Expression of Complement Regulator Genes in Aβ1-42 Stimulated Human Neuroblastoma Cell. (freepatentsonline.com)
  • The central elements in the pathogenesis of pEDS seem to be the intracellular activation of C1r and/or C1s, and extracellular presence of activated C1s that independently of microbial triggers can activate the classical complement cascade. (frontiersin.org)
  • C1r2C1s2 is a subcomponent of first component C1 of the complement cascade. (biochemj.org)
  • 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)
  • 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 which helps clear pathogens from an organism. (wikidoc.org)
  • In contrast to C1r wild type, and with the exception of a C1R missense variant disabling a C1q binding site, pEDS variants had different impact on the cell: retention of C1r fragments inside the cell, secretion of aggregates, or a new C1r cleavage site. (frontiersin.org)
  • Activation of C1r by proteolytic cleavage. (nih.gov)
  • This way, C1-inh prevents the proteolytic cleavage of later complement components C4 and C2 by C1 and MBL. (bionity.com)
  • Also, C4 and C2 cleavage goes unchecked, resulting in auto-activation of the complement system. (bionity.com)
  • Heterozygous missense or in-frame insertion/deletion mutations in complement 1 subunits C1r and C1s cause periodontal Ehlers-Danlos Syndrome (pEDS), a specific EDS subtype characterized by early severe periodontal destruction and connective tissue abnormalities like easy bruising, pretibial haemosiderotic plaques, and joint hypermobility. (frontiersin.org)
  • 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)
  • Immunofluorescence studies on the subcomponents of the first component of complement (C1): detection of C1q and C1s in different cells of biopsy material and on human as well as on guinea pig peritoneal macrophages. (freepatentsonline.com)
  • Periodontal Ehlers-Danlos syndrome is caused by mutations in C1R and C1S, which encode subcomponents C1r and C1s of complement. (expasy.org)
  • 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)
  • The full-length (amino acid residues 1‑705) of human C1r was expressed, which had the Leu152 natural variant (3). (novusbio.com)
  • Structural models of the CCP1-CCP2-SP segments of two C1r molecules built on the basis of shift perturbation data are fully consistent with an extended interaction interface and suggests the possibility of a structural rearrangement as a switch between functional states of human C1r. (elsevier.com)
  • We have determined the first crystal structure of the entire active catalytic region of human C1r. (elsevier.com)
  • We report extensive functional studies of 16 C1R variants associated with pEDS by in-vitro overexpression studies in HEK293T cells followed by western blot, size exclusion chromatography and surface plasmon resonance analyses. (frontiersin.org)
  • All Quidel complement components are tested for functional activity in a standard lytic or applicable functional assay and for biochemical purity by SDS-polyacrylamide gel electrophoresis. (quidel.com)
  • Physicochemical and functional characterization of the C1r subunit of the first complement component. (nih.gov)
  • Initial laboratory assessment involves testing for C1-INH levels, complement 4 (C4) levels, functional C1-INH activity, and possibly C1q levels. (arupconsult.com)
  • We offer Complement Component C1r Lysates for use in common research applications: Western Blot. (novusbio.com)
  • Overexpression of C1R variants in HEK293T as well as western blot analyses of patient fibroblasts showed decreased levels of secreted C1r. (frontiersin.org)
  • A combinatorial, conditional genetic model, involving an interaction between the C5 and C1r/s alleles, accurately predicted survival after disseminated candidiasis. (asm.org)
  • Non-activated C1r is found in circulating blood plasma as a dimer that associates with one C1q molecule and two C1s molecules to form the first component of complement (C1). (merckmillipore.com)
  • Moreover, in the crystal lattice there is an enzyme-product relationship between the C1r molecules of neighboring dimers. (elsevier.com)
  • One, the recognition unit, consists of a trimolecular complex of C1q, 2 molecules of C1r, and 2 molecules of C1s held together by calcium. (medscape.com)
  • The complement system acts as a mediator in the innate immune response by ultimately triggering phagocytosis, inflammation, and rupturing the bacterial cell wall. (nih.gov)
  • The complement system is an important component of the innate immunity. (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)
  • 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)
  • The complement system is a fundamental element of the innate immune system as well as the adaptive immune responses. (ukessays.com)
  • We recently showed that pEDS is caused by heterozygous missense or in-frame insertion/deletion mutations in C1R or C1S ( 4 ). (frontiersin.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)
  • 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)
  • Ehrlich believed that each antigen-specific amboceptor had its own specific complement, while Bordet believed that there is only one type of complement. (wikidoc.org)
  • Based on these novel structural information we propose a new split-and-reassembly model for the autoactivation of the C1r. (elsevier.com)
  • It allows autoactivation of C1r without large-scale, directed movement of C1q arms. (elsevier.com)
  • This induces a conformational change, leading to autoactivation of C1r that then cleaves C1s to its active state. (medscape.com)
  • In the first phase, a series of specific interactions leads to formation of intrinsic complement proteinase, termed C3 convertase. (medscape.com)
  • The serine proteinase C2a then combines with complement factor 4b to create the C3 or C5 convertase. (genecards.org)
  • Molecular cloning of cDNA for human complement component C1s. (wikipedia.org)
  • Dysregulation of the complement system has been implicated, but molecular mechanisms are incompletely understood. (frontiersin.org)
  • This infers resistance to complement-mediated cell lysis, allowing parasite survival and infection. (genecards.org)
  • Kupffer cells and other macrophage cell types help clear complement-coated pathogens. (wikidoc.org)
  • Overexpression of C1R variants in HEK293T cells revealed that none of the pEDS variants was integrated into the C1 complex but cause extracellular presence of catalytic C1r/C1s activities. (frontiersin.org)