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Complement C3b Inactivator ProteinsComplement C3Complement C4Complement Inactivator ProteinsComplement C1 Inactivator ProteinsProperdinComplement C3bComplement System ProteinsComplement ActivationComplement C4aComplement C2Complement C3-C5 ConvertasesComplement Factor BComplement C3aComplement Pathway, AlternativeComplement C1qComplement C5Complement C5aComplement C4bComplement Activating EnzymesBeta-GlobulinsComplement C6Complement C3cComplement C3dSerum GlobulinsComplement C9Receptors, ComplementHemolysisComplement C1sComplement Membrane Attack ComplexComplement C1rMicropore FiltersComplement C7Complement Pathway, ClassicalBlood ProteinsComplement C8Complement C1Complement Factor HAnaphylatoxinsReceptors, Complement 3bComplement C5bComplement C2aReceptor, Anaphylatoxin C5aErythrocytesComplement Inactivating AgentsComplement Hemolytic Activity AssayReceptors, Complement 3dComplement Fixation TestsComplement Factor DAntigen-Antibody ComplexComplement Factor IComplement C4b-Binding ProteinAntigens, CD55Complement C3-C5 Convertases, Classical PathwayComplement C2bAntigens, CD59Lysine CarboxypeptidaseMolecular Sequence DataKineticsImmunoelectrophoresisCytochrome P-450 CYP2B1PhagocytosisCobra VenomsSteroid 21-HydroxylaseChloromercurinitrophenolsComplement C3-C5 Convertases, Alternative PathwayAmino Acid SequenceAngioedemaComplement C1 Inhibitor Protein
Complement C3b Inactivator Proteins
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.
Complement C3
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.
Complement C4
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.
Complement Inactivator Proteins
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.
Complement C1 Inactivator Proteins
Serum proteins that inhibit, antagonize, or inactivate COMPLEMENT C1 or its subunits.
Properdin
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.
Complement C3b
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.
Complement System Proteins
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).
Complement Activation
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.
Complement C4a
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.
Complement C2
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).
Complement C3-C5 Convertases
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.
Complement Factor B
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 C3a
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.
Complement Pathway, Alternative
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 C1q
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.
Complement C5
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.
Complement C5a
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.
Complement C4b
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).
Complement Activating Enzymes
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.
Beta-Globulins
Serum proteins with an electrophoretic mobility that falls between ALPHA-GLOBULINS and GAMMA-GLOBULINS.
Complement C6
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.
Complement C3c
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.
Complement C3d
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).
Serum Globulins
All blood proteins except albumin ( = SERUM ALBUMIN, which is not a globulin) and FIBRINOGEN (which is not in the serum). The serum globulins are subdivided into ALPHA-GLOBULINS; BETA-GLOBULINS; and GAMMA-GLOBULINS on the basis of their electrophoretic mobilities. (From Dorland, 28th ed)
Complement C9
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.
Receptors, Complement
Molecules on the surface of some B-lymphocytes and macrophages, that recognize and combine with the C3b, C3d, C1q, and C4b components of complement.
Hemolysis
The destruction of ERYTHROCYTES by many different causal agents such as antibodies, bacteria, chemicals, temperature, and changes in tonicity.
Complement C1s
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).
Complement Membrane Attack Complex
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.
Complement C1r
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.
Micropore Filters
A membrane or barrier with micrometer sized pores used for separation purification processes.
Complement C7
A 93-kDa serum glycoprotein encoded by C7 gene. It is a polypeptide chain with 28 disulfide bridges. In the formation of MEMBRANE ATTACK COMPLEX; C7 is the next component to bind the C5b-6 complex forming a trimolecular complex C5b-7 which is lipophilic, resembles an integral membrane protein, and serves as an anchor for the late complement components, C8 and C9.
Complement Pathway, Classical
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.
Blood Proteins
Proteins that are present in blood serum, including SERUM ALBUMIN; BLOOD COAGULATION FACTORS; and many other types of proteins.
Complement C8
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.
Complement C1
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.
Complement Factor H
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).
Anaphylatoxins
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.
Receptors, Complement 3b
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.
Complement C5b
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.
Complement C2a
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).
Receptor, Anaphylatoxin C5a
A G-protein-coupled receptor that signals an increase in intracellular calcium in response to the potent ANAPHYLATOXIN peptide COMPLEMENT C5A.
Erythrocytes
Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN.
Complement Inactivating Agents
Compounds that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host.
Complement Hemolytic Activity Assay
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.
Receptors, Complement 3d
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.
Complement Fixation Tests
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.
Complement Factor D
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.
Antigen-Antibody Complex
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.
Complement Factor I
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.
Complement C4b-Binding Protein
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).
Antigens, CD55
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.
Complement C3-C5 Convertases, Classical Pathway
Important enzymes in the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. They cleave COMPLEMENT C3 and COMPLEMENT C5.
Complement C2b
The N-terminal fragment of COMPLEMENT 2, released by the action of activated COMPLEMENT C1S.
Antigens, CD59
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)
Lysine Carboxypeptidase
A metallocarboxypeptidase that removes C-terminal basic amino acid from peptides and proteins, with preference shown for lysine over arginine. It is a plasma zinc enzyme that inactivates bradykinin and anaphylatoxins.
Molecular Sequence Data
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.
Kinetics
The rate dynamics in chemical or physical systems.
Immunoelectrophoresis
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.
Cytochrome P-450 CYP2B1
A major cytochrome P-450 enzyme which is inducible by PHENOBARBITAL in both the LIVER and SMALL INTESTINE. It is active in the metabolism of compounds like pentoxyresorufin, TESTOSTERONE, and ANDROSTENEDIONE. This enzyme, encoded by CYP2B1 gene, also mediates the activation of CYCLOPHOSPHAMIDE and IFOSFAMIDE to MUTAGENS.
Phagocytosis
The engulfing and degradation of microorganisms; other cells that are dead, dying, or pathogenic; and foreign particles by phagocytic cells (PHAGOCYTES).
Cobra Venoms
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.
Steroid 21-Hydroxylase
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).
Chloromercurinitrophenols
Mercuriphenols substituted with one or more chlorine atoms and one or more nitro groups. Some of these are sulfhydryl reagents which act as chromophoric probes in enzymes and other proteins.
Complement C3-C5 Convertases, Alternative Pathway
Important enzymes in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY. They cleave COMPLEMENT C3 and COMPLEMENT C5.
Amino Acid Sequence
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.
Angioedema
Swelling involving the deep DERMIS, subcutaneous, or submucosal tissues, representing localized EDEMA. Angioedema often occurs in the face, lips, tongue, and larynx.
Complement C1 Inhibitor Protein
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.

Regulation of complement activation by C-reactive protein: targeting the complement inhibitory activity of factor H by an interaction with short consensus repeat domains 7 and 8-11. (1/204)

C-reactive protein (CRP) is a major acute phase protein whose functions are not totally clear. In this study, we examined the interaction of CRP with factor H (FH), a key regulator of the alternative pathway (AP) of complement. Using the surface plasmon resonance technique and a panel of recombinantly expressed FH constructs, we observed that CRP binds to two closely located regions on short consensus repeat (SCR) domains 7 and 8-11 of FH. Also FH-like protein 1 (FHL-1), an alternatively spliced product of the FH gene, bound to CRP with its most C-terminal domain (SCR 7). The binding reactions were calcium-dependent and partially inhibited by heparin. In accordance with the finding that CRP binding sites on FH were distinct from the C3b binding sites, CRP preserved the ability of FH to promote factor I-mediated cleavage of C3b. We propose that the function of CRP is to target functionally active FH and FHL-1 to injured self tissues. Thereby, CRP could restrict excessive complement attack in tissues while allowing a temporarily enhanced AP activity against invading microbes in blood.  (+info)

Complement activation in patients with systemic lupus erythematosus without nephritis. (2/204)

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

Different regulation of factor H and FHL-1/reconectin by inflammatory mediators and expression of the two proteins in rheumatoid arthritis (RA). (3/204)

Factor H and the FHL-1/reconectin protein are two human plasma proteins that act as important regulators of the alternative complement pathway. Each protein is encoded by a unique transcript, but both mRNAs are derived from the factor H gene by means of alternative processing. In order to address potential functional differences between the two proteins we analysed their expression in hepatic and non-hepatic cells and studied their regulation by inflammatory mediators. We demonstrate that factor H and FHL-1/reconectin transcripts which are regulated by the same gene promoter and are initiated at the same transcription start site are differently expressed. Expression of the molecules is induced and regulated by the inflammatory mediators interferon-gamma (IFN-gamma) and the anti-inflammatory glucocorticoid dexamethasone. Both factor H and FHL-1/reconectin are expressed and secreted by synovial fibroblasts and are present in synovial fluid derived from patients suffering from rheumatoid or reactive arthritis. The local synthesis in synovial fibroblasts and their induction by IFN-gamma and dexamethasone, but not by tumour necrosis factor-alpha, suggests for each of the two complement regulators a protective role in RA.  (+info)

Structure-guided identification of C3d residues essential for its binding to complement receptor 2 (CD21). (4/204)

A vital role for complement in adaptive humoral immunity is now beyond dispute. The crucial interaction is that between B cell and follicular dendritic cell-resident complement receptor 2 (CR2, CD21) and its Ag-associated ligands iC3b and C3dg, where the latter have been deposited as a result of classical pathway activation. Despite the obvious importance of this interaction, the location of a CR2 binding site within C3d, a proteolytic limit fragment of C3dg retaining CR2 binding activity, has not been firmly established. The recently determined x-ray structure of human C3d suggested a candidate site that was remote from the site of covalent attachment to Ag and consisted of an acidic residue-lined depression, which accordingly displays a significant electronegative surface potential. These attributes were consistent with the known ionic strength dependence of the CR2-C3d interaction and with the fact that a significant electropositive surface was apparent in a modeled structure of the C3d-binding domains of CR2. Therefore, we have performed an alanine scan of all of the residues within and immediately adjacent to the acidic pocket in C3d. By testing the mutant iC3b molecules for their ability to bind CR2, we have identified two separate clusters of residues on opposite sides of the acidic pocket, specifically E37/E39 and E160/D163/I164/E166, as being important CR2-contacting residues in C3d. Within the second cluster even single mutations cause near total loss of CR2 binding activity. Consistent with the proposed oppositely charged nature of the interface, we have also found that removal of a positive charge immediately adjacent to the acidic pocket (mutant K162A) results in a 2-fold enhancement in CR2 binding activity.  (+info)

Cooperation between decay-accelerating factor and membrane cofactor protein in protecting cells from autologous complement attack. (5/204)

Decay-accelerating factor (DAF or CD55) and membrane cofactor protein (MCP or CD46) function intrinsically in the membranes of self cells to prevent activation of autologous complement on their surfaces. How these two regulatory proteins cooperate on self-cell surfaces to inhibit autologous complement attack is unknown. In this study, a GPI-anchored form of MCP was generated. The ability of this recombinant protein and that of naturally GPI-anchored DAF to incorporate into cell membranes then was exploited to examine the combined functions of DAF and MCP in regulating complement intermediates assembled from purified alternative pathway components on rabbit erythrocytes. Quantitative studies with complement-coated rabbit erythrocyte intermediates constituted with each protein individually or the two proteins together demonstrated that DAF and MCP synergize the actions of each other in preventing C3b deposition on the cell surface. Further analyses showed that MCP's ability to catalyze the factor I-mediated cleavage of cell-bound C3b is inhibited in the presence of factors B and D and is restored when DAF is incorporated into the cells. Thus, the activities of DAF and MCP, when present together, are greater than the sum of the two proteins individually, and DAF is required for MCP to catalyze the cleavage of cell-bound C3b in the presence of excess factors B and D. These data are relevant to xenotransplantation, pharmacological inhibition of complement in inflammatory diseases, and evasion of tumor cells from humoral immune responses.  (+info)

The complement regulator factor H binds to the surface protein OspE of Borrelia burgdorferi. (6/204)

Spirochete bacteria of the Borrelia burgdorferi sensu lato complex cause Lyme borreliosis. The three pathogenic subspecies Borrelia garinii, Borrelia afzelii, and Borrelia burgdorferi sensu stricto differ in their disease profiles and susceptibility to complement lysis. We investigated whether complement resistance of Borreliae could be due to acquisition of the main soluble inhibitors of the alternative complement pathway, factor H and the factor H-like protein 1. When exposed to nonimmune EDTA-plasma, the serum-resistant B. afzelii and B. burgdorferi sensu stricto strains bound factor H/factor H-like protein 1 to their surfaces. Assays with radiolabeled proteins showed that factor H bound strongly to the B. burgdorferi sensu stricto strain. To identify factor H ligands on the borrelial surface, we analyzed a panel of outer surface proteins of B. burgdorferi sensu stricto with the surface plasmon resonance technique. The outer surface lipoprotein OspE was identified as a specific ligand for factor H. Using recombinant constructs of factor H, the binding site for OspE was localized to the C-terminal short consensus repeat domains 15-20. Specific binding of factor H to B. burgdorferi sensu stricto OspE may help the pathogen to evade complement attack and phagocytosis.  (+info)

Complement evasion by Borrelia burgdorferi: serum-resistant strains promote C3b inactivation. (7/204)

The most characteristic features of the Lyme disease pathogens, the Borrelia burgdorferi sensu lato (s.l.) group, are their ability to invade tissues and to circumvent the immune defenses of the host for extended periods of time, despite elevated levels of borrelia-specific antibodies in serum and other body fluids. Our aim in the present study was to determine whether B. burgdorferi is able to interfere with complement (C) at the level of C3 by accelerating C3b inactivation and thus to inhibit the amplification of the C cascade. Strains belonging to different genospecies (Borrelia garinii, B. burgdorferi sensu stricto, and Borrelia afzelii) were compared for their sensitivities to normal human serum and abilities to promote factor I-mediated C3b degradation. B. burgdorferi sensu stricto and B. afzelii strains were found to be serum resistant. When the spirochetes were incubated with radiolabeled C3b, factor I-mediated degradation of C3b was observed in the presence of C-resistant B. afzelii (n = 3) and B. burgdorferi sensu stricto (n = 1) strains but not in the presence of C-sensitive B. garinii (n = 7) strains or control bacteria (Escherichia coli, Staphylococcus aureus, and Enterococcus faecalis). Immunoblotting and radioligand binding analyses showed that the C-resistant strains had the capacity to acquire the C inhibitors factor H and factor H-like protein 1 (FHL-1) from growth medium and human serum. A novel surface protein with an apparent molecular mass of 35 kDa was found to preferentially bind to the N terminus region of factor H. Thus, the serum-resistant B. burgdorferi s.l. strains can circumvent C attack by binding the C inhibitors factor H and FHL-1 to their surfaces and promoting factor I-mediated C3b degradation.  (+info)

Relative importance of C3b inactivator and beta 1H globulin in the modulation of the properdin amplification loop in systemic lupus erythematosus. (8/204)

Serum concentrations of C4, C3, factor B (B), properdin (P), C3b inactivator (C3bINA) and beta 1H globulin have been measured by radial immunodiffusion in sixty-two samples from thirteen patients with systemic lupus erythematosus (SLE). Significant reductions in the mean serum concentrations of C4 (classical pathway) B and P (alternative pathway) and C3 were found. In addition, the mean level of the control protein beta 1H, but not C3bINA, was reduced. Sera from thirteen patients taking during disease exacerbation (low C3) showed significantly lower levels of both C3bINA and beta 1H than sera taken from the same thirteen patients during disease remission (high C3). Serum concentrations of C3bINA correlated with B (P less than 0.005) but not C4, C3 or P, whereas levels of beta 1H correlated with C4 (P less than 0.01), B (P less than 0.005) and properdin (P less than 0.01). Serial measurements of the serum concentrations of C3bINA and beta 1H showed that levels of these protein fell during exacerbation, and such falls were more closely associated with diseases in the serum levels of the alternative pathways proteins than C4. It is concluded from these observations that serum concentrations of the control proteins C3bINA and beta 1H, especially the latter, control the extent of turnover of the alternative pathway in SLE. Metabolic studies are required to determine the causes of the decreased serum concentrations of these control proteins.  (+info)

Variants in Complement Factor H and Complement Factor H-Related Protein Genes, CFHR3 and CFHR1, Affect Complement Activation in...Variants in Complement Factor H and Complement Factor H-Related Protein Genes, CFHR3 and CFHR1, Affect Complement Activation in...
To further investigate this hypothesis, we enrolled group 2. In group 2, we found individuals with the rs6677604-A allele had increased CFH levels. Recently, Ansari et al.12 reported that rs6677604 and CFHR3-1∆ were strongly correlated with plasma CFH concentration, which is in accordance with our findings. Moreover, the CFHR3-1∆ resulted in the absence of CFHR1 protein, which was recently shown to function as a competitive antagonist of CFH.33 Therefore, higher CFH levels, together with the absence of antagonist (CFHR1 protein), resulted in the robust complement inhibition, which is represented by the higher circulating C3 and lower C3a that we observed in patients with IgAN with the rs6677604-AA genotype and CFHR3-1Δ. In accordance with our findings, Yang et al.34 reported that rs3753394 in CFH was associated with circulating C3 levels. Because rs3753394 and rs6677604 are in LD, the study by Yang et al.34 provides independent validation for our findings. Meanwhile, by showing that plasma ...
https://jasn.asnjournals.org/content/26/5/1195?ijkey=faf593e3f8c8653a47f4e9e73a43bc24bbea1c1e&keytype2=tf_ipsecsha
Factor H-related protein 1 (FHR-1): A complement regulatory pr...Factor H-related protein 1 (FHR-1): A complement regulatory pr...
Factor H-related protein 1 (FHR-1): A complement regulatory protein and guardian of necrotic type surfaces.: Factor H-related protein 1 (FHR-1) is a member of t
https://www.mysciencework.com/publication/show/factor-hrelated-protein-1-fhr1-complement-regulatory-protein-guardian-necrotic-type-surfaces-b1c09ebb?search=1
Interdomain contact regions and angles between adjacent short consensus repeat domainsInterdomain contact regions and angles between adjacent short consensus repeat domains
The short consensus repeat domain (SCR, complement control protein module, sushi-domain) is a structural unit found in multiple adjacent copies in more than 40 human proteins. Each bead-like domain is composed of approximately 60 residues and the adjacent domains are connected in a head-to-tail fash …
https://pubmed.ncbi.nlm.nih.gov/15561150/
Pesquisa | Portal Regional da BVSPesquisa | Portal Regional da BVS
There is increasing evidence that human complement factor H-related protein 1 (CFHR1) plays a crucial role in the development of malignant diseases. However, few studies have identified the roles of CFHR1 in the occurrence and prognosis of lung adenocarcinoma (LADC). In the present study, comprehensive bioinformatic analyses of data obtained from the Oncomine platform, UALCAN and Gene Expression Profiling Interactive Analysis (GEPIA) demonstrated that CFHR1 expression is significantly reduced in both LADC tissues and cancer cells. The patients presenting with downregulation of CFHR1 had significantly lower overall survival (OS) and post progression survival (PPS) times. Through analysis of the datasets from Gene Expression Omnibus database, we found that the compound actinomycin D promoted CFHR1 expression, further displaying the cytotoxic effect in the LADC cell line A549. In addition, the expression level of CFHR1 in the cisplatin-resistant LADC cell line CDDP-R (derived from H460) was also ...
https://pesquisa.bvsalud.org/portal/?lang=pt&q=au:%22Wu,%20Geting%22
YB1 - NBP1-78116 | acris-antibodies.com
YB1, 0.1 mg. CRASP-1, or Complement Regulator-Acquiring Surface Protein 1, is a multifunctional protein of Lyme disease-causing B.
https://www.acris-antibodies.com/antibodies/primary-antibodies/yb1-nbp1-78116.htm
Modulation of the Alternative Pathway of Complement by Murine Factor H-Related Proteins | The Journal of ImmunologyModulation of the Alternative Pathway of Complement by Murine Factor H-Related Proteins | The Journal of Immunology
The complement system is a major mediator of immune surveillance and homeostasis. At present, there is an increased focus on understanding the regulation of the different pathways, especially the AP, which is the primary pathway by which the system damages cells and tissues. The spontaneous hydrolysis of C3 leading to generation of C3b must be well controlled to prevent inappropriate and potentially rapid AP complement activation. FH is a major soluble complement regulator that is essential for controlling AP activation in plasma and on cell surfaces through the engagement of polyanionic surface markers, such as sialic acid, and C3 fragments, such as C3b and C3d. Mutations or deletions in FH, particularly within the C-terminal region, are associated with a number of different autoimmune and inflammatory illnesses. Moreover, pathogens and altered-host cells, such as cancer cells, exploit the protective function of FH by recruiting FH to their surfaces as part of an immune evasion/modulation ...
https://www.jimmunol.org/content/200/1/316
Narges Bani Asadi, Bina Technologies | FierceBiotechNarges Bani Asadi, Bina Technologies | FierceBiotech
Narges Bani Asadi Analyzing and interpreting whole human -omes Company: Bina Technologies Title: Cofounder and CEO Bina Technologies launched in 2011 planning to attack one of the major...
https://www.fiercebiotech.com/special-report/narges-bani-asadi-bina-technologies
RCSB PDB - 4AYI: Structure of a complex between CCPs 6 and 7 of Human Complement Factor H and Neisseria meningitidis FHbp...RCSB PDB - 4AYI: Structure of a complex between CCPs 6 and 7 of Human Complement Factor H and Neisseria meningitidis FHbp...
4AYI: Structure of a complex between CCPs 6 and 7 of Human Complement Factor H and Neisseria meningitidis FHbp Variant 3 Wild type
https://www.rcsb.org/structure/4AYI
New insights into disease-specific absence of complement factor H related protein C in mouse models of spontaneous autoimmune...New insights into disease-specific absence of complement factor H related protein C in mouse models of spontaneous autoimmune...
Complement factor H (CFH) protein is an inhibitor of the alternative pathway of complement (AP) both in the fluid phase and on the surface of host cells. Mouse and human complement factor H-related (CFHR) proteins also belong to the fH family of plasma glycoproteins. The main goal of the current study was to compare the presence of mRNA for two mCFHR proteins in spontaneously developing autoimmune diseases in mice such as dense deposit disease (DDD), diabetes mellitus (DM), basal laminar deposits (BLD), collagen antibody-induced arthrits (CAIA) and systemic lupus erythematosus (SLE). Here we report for the first time that the CFHR-C mRNA was universally absent in the liver from three strains of lupus-prone mice and in a diabetic-prone mouse strain. The mRNA levels (pg/ng) for CFH and CFHR-B in MRL-lpr/lpr, at 9wks and 23wks were 707.2±44.4, 54.5±5.75 and 729±252.9, 74.04±22.76, respectively. The mRNA levels for CFH and CFHR-B in NZB/NZW mice, at 9wks and 54wks were 579.9±23.8, 58.8±1.41 ...
https://www.leibniz-hki.de/en/ib-publications-details.html?publication=757
Isolation from human serum of an inactivator of bacterial lipopolysaccharide.
By a series of chromatographic procedures involving precipitation by salt, gel filtration, anionic exchange, and hydroxyapatite elution, a protein--termed the lipopolysaccharide inactivator (LPS-I)--has been isolated from normal human serum. As a res
http://www.biomedsearch.com/nih/Isolation-from-human-serum-inactivator/70173.html
Human complement factor H Y402H polymorphism causes an age-related macular degeneration phenotype and lipoprotein dysregulation...Human complement factor H Y402H polymorphism causes an age-related macular degeneration phenotype and lipoprotein dysregulation...
Human complement factor H Y402H polymorphism causes an age-related macular degeneration phenotype and lipoprotein dysregulation in mice.
https://scholars.duke.edu/display/pub1373203
Candida albicans uses the surface protein Gpm1 to attach to human endothelial cells and to keratinocytes via the adhesive...Candida albicans uses the surface protein Gpm1 to attach to human endothelial cells and to keratinocytes via the adhesive...
Candida albicans is a major cause of invasive fungal infections worldwide. Upon infection and when in contact with human plasma as well as body fluids the fungus is challenged by the activated complement system a central part of the human innate immune response. C. albicans controls and evades host complement attack by binding several human complement regulators like Factor H, Factor H-like protein 1 and C4BP to the surface. Gpm1 (Phosphoglycerate mutase 1) is one fungal Factor H/FHL1 -binding protein. As Gpm1 is surface exposed, we asked whether Gpm1 also contributes to host cell attachment. Here, we show by flow cytometry and by laser scanning microscopy that candida Gpm1 binds to human umbilical vein endothelial cells (HUVEC) to keratinocytes (HaCaT), and also to monocytic U937 cells. Wild type candida did bind, but the candida gpm1Δ/Δ knock-out mutant did not bind to these human cells. In addition Gpm1 when attached to latex beads also conferred attachment to human endothelial cells. When ...
https://www.leibniz-hki.de/en/ib-publications-details.html?publication=762
Unraveling the Effect of a Potentiating Anti-Factor H Antibody on Atypical Hemolytic Uremic Syndrome-Associated Factor H...Unraveling the Effect of a Potentiating Anti-Factor H Antibody on Atypical Hemolytic Uremic Syndrome-Associated Factor H...
The complement system plays an important role in our innate immune system. Complement activation results in clearance of pathogens, immune complex, and apoptotic cells. The host is protected from complement-mediated damage by several complement regulators. Factor H (FH) is the most important fluid-phase regulator of the alternative pathway of the complement system. Heterozygous mutations in FH are associated with complement-related diseases such as atypical hemolytic uremic syndrome (aHUS) and age-related macular degeneration. We recently described an agonistic anti-FH mAb that can potentiate the regulatory function of FH. This Ab could serve as a potential new drug for aHUS patients and alternative to C5 blockade by eculizumab. However, it is unclear whether this Ab can potentiate FH mutant variants in addition to wild-type (WT) FH. In this study, the functionality and potential of the agonistic Ab in the context of pathogenic aHUS-related FH mutant proteins was investigated. The binding ...
https://www.jimmunol.org/content/205/7/1778
Innopsys - Complement factor H autoantibodies are associated with early stage NSCLCInnopsys - Complement factor H autoantibodies are associated with early stage NSCLC
Nita Amornsiripanitch1, Shaolin Hong1, Michael J. Campa1, Michael M. Frank2, Elizabeth B. Gottlin1, and Edward F. Patz Jr.1,3 Author Affiliations. Corresponding Author: Edward F. Patz, Jr., Duke University Medical Center, Department of Radiology, Box 3808, Durham, NC 27710. Phone: 919-684-7311; Fax: 919-681-7165; E-mail: [email protected] ...
https://www.innopsys.com/en/publications-microarrays/literature/publications/publication-is710/226-amornsiripanitch
Publication | Luxembourg Institute of HealthPublication | Luxembourg Institute of Health
Directing selective complement activation towards tumour cells is an attractive strategy to promote their elimination. In the present work, we have generated heteromultimeric immunoconjugates that selectively activate the complement alternative pathway (AP) on tumour cells. We used the C4b-binding protein C-terminal-alpha-/beta-chain scaffold for multimerisation to generate heteromultimeric immunoconjugates displaying (a) a multivalent-positive regulator of the AP, the human factor H-related protein 4 (FHR4) with; (b) a multivalent targeting function directed against erbB2 (HER2); and (c) a monovalent enhanced GFP tracking function. Two distinct VH H targeting two different epitopes against HER2 and competing either with trastuzumab or with pertuzumab-recognising epitopes [VH H(T) or VH H(P)], respectively, were used as HER2 anchoring moieties. Optimised high-FHR4 valence heteromultimeric immunoconjugates [FHR4/VH H(T) or FHR4/VH H(P)] were selected by sequential cell cloning and a selective ...
https://www.lih.lu/crp/publication/fhr4-based-immunoconjugates-direct-complement-dependent-cytotoxicity-and-phagocytosis-towards-her2-positive-cancer-cells-14027
Archive | www.bioquicknews.comArchive | www.bioquicknews.com
An international team of scientists has identified a protein that is strongly linked to the commonest cause of blindness in developed countries when its levels are raised in the blood. The discovery is a major step forward in the understanding of age-related macular degeneration (AMD), which affects 1.5 million people in the UK alone. The study, carried out by a team from the Universities of Manchester, Cardiff, London, and Nijmegen, and Manchester Foundation NHS Trust was published online on February 7, 2020 in Nature Communications. The open-access article is titled Increased Circulating Levels of Factor H Related Protein 4 Are Strongly Associated with Age-Related Macular Degeneration. The protein, Factor H-Related Protein 4 (FHR4), was found by the team to be present at higher levels in the blood of patients with AMD compared to individuals of a similar age without the disease. The findings were confirmed in 484 patient and 522 control samples from two independent collections across ...
http://www.bioquicknews.com/archive/all?page=1
SMART: CCP domain annotationSMART: CCP domain annotation
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. These modules have been identified more than 140 times in over 20 proteins, including 12 proteins of the complement system. The solution structure of the 16th CCP module from human complement factor H has been determined by a combination of 2-dimensional nuclear magnetic resonance spectroscopy and restrained simulated annealing. In all, 548 structurally important nuclear Overhauser enhancement cross-peaks were quantified as distance restraints and, together with 41 experimentally measured angle restraints, were incorporated into a simulated annealing protocol to determine a family of closely related structures that satisfied the experimental observations. The CCP structure is shown to be based on a beta-sandwich arrangement; one face made up of three beta-strands hydrogen-bonded to form a triple-stranded region at its centre ...
http://smart.embl.de/smart/do_annotation.pl?DOMAIN=CCP&START=1741&END=1798&E_VALUE=9.46e-12&TYPE=SMART&BLAST=CSSVPEPRYGRRIGSEFSAGSIVRFECNPGYLLQGSTAIRCQSVPNALAQWNDTIPSC
SMART: CCP domain annotationSMART: CCP domain annotation
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. These modules have been identified more than 140 times in over 20 proteins, including 12 proteins of the complement system. The solution structure of the 16th CCP module from human complement factor H has been determined by a combination of 2-dimensional nuclear magnetic resonance spectroscopy and restrained simulated annealing. In all, 548 structurally important nuclear Overhauser enhancement cross-peaks were quantified as distance restraints and, together with 41 experimentally measured angle restraints, were incorporated into a simulated annealing protocol to determine a family of closely related structures that satisfied the experimental observations. The CCP structure is shown to be based on a beta-sandwich arrangement; one face made up of three beta-strands hydrogen-bonded to form a triple-stranded region at its centre ...
http://smart.embl.de/smart/do_annotation.pl?DOMAIN=CCP&START=2772&END=2825&E_VALUE=3.64e-13&TYPE=SMART&BLAST=CSKPNPLWNGSIKGDDYSYLGVLYYECDSGYILNGSKKRTCQENRDWDGHEPMC
JCI - Complement factor H-related hybrid protein deregulates complement in dense deposit diseaseJCI - Complement factor H-related hybrid protein deregulates complement in dense deposit disease
Patients. The index family has 2 affected children. In 1987, at the age of 2, patient no. 635, a girl, suffered from urinary tract infection followed by proteinuria and hematuria. Similar symptoms were also found in her older brother (patient no. 638). No other clinical signs were noted, and serology for antinuclear antibody and antineutrophil cytoplasmic autoantibody was negative. The proteinuria was steroid resistant, increased slowly over the years, and developed into nephrotic syndrome. In 2000, renal biopsies of both siblings revealed prominent C3c staining along the GBM and also partly within the mesangium but no relevant immunoglobulin staining. Electron microscopy in one reprocessed paraffin-embedded biopsy showed intense partly ribbon-like, electron-dense deposits within and along the GBM and in the mesangium. The observed images were interpreted as evidence of C3 glomerulopathy in both cases. In the case analyzed by electron microscopy, some features were reminiscent of DDD. Renal ...
https://www.jci.org/articles/view/71866
Rat CFH(Complement Factor H) ELISA Kit - Rat CFH(Complement Factor H) ELISA Kit Exporter, Importer, Manufacturer, Distributor,...
SR GROUP - Exporter, Importer, Manufacturer, Distributor, Supplier, Trading Company of Rat CFH(Complement Factor H) ELISA Kit based in Delhi, India
https://www.srgroupchemical.com/rat-cfh-complement-factor-h-elisa-kit-4465217.html
Aperçu des produits pour Complement Factor H kit ELISAAperçu des produits pour Complement Factor H kit ELISA
Commander Complement Factor H kit ELISA pour beaucoup de réactivité. Poulet, Boeuf (Vache), Chien et plus. Comparez Complement Factor H kit ELISA et trouvez le bon produit chez anticorps-enligne.fr.
https://www.anticorps-enligne.fr/systeme-du-complement-pathway-11/complement-factor-h-elisa-kit-60/
Successful pre-transplant management of a patient with anti-factor H autoantibodies-associated haemolytic uraemic syndrome :...
Theresa Kwon, Marie-Agnes Dragon-Durey, Marie-Alice Macher, Veronique Baudouin, Anne Maisin, Michel Peuchmaur, Veronique Fremeaux-Bacchi and Chantal Loirat. in Nephrology Dialysis Transplantation Published on behalf of European Renal Association - European Dialysis and Transplant Assoc ...
http://oxfordindex.oup.com/view/10.1093/ndt/gfn063
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CFH健康知識網 - 專欄搜尋結果CFH健康知識網 - 專欄搜尋結果
CFH健康知識網,健康知識庫,健康知識中心.專業健康文章,康活健康知識網,健康百科,健康生活家,疾病,疾病簡介,保健, 預防,慢性病,慢性病預防,營養成分,
http://cfh.com.tw/CFHModule/BlogTagSearchResult.aspx?tagname=autoimmune&groupid=4
First-line therapy in atypical hemolytic uremic syndrome: consideration on infants with a poor prognosis<...
TY - JOUR. T1 - First-line therapy in atypical hemolytic uremic syndrome. T2 - consideration on infants with a poor prognosis. AU - Szarvas, Nóra. AU - Szilágyi, Ágnes. AU - Tasic, Velibor. AU - Nushi-Stavileci, Valbona. AU - Sofijanova, Aspazija. AU - Gucev, Zoran. AU - Szabó, Miklós. AU - Szabó, Attila. AU - Szeifert, Lilla. AU - Reusz, György. AU - Rusai, Krisztina. AU - Arbeiter, Klaus. AU - Müller, Thomas. AU - Prohászka, Zoltán. PY - 2014/12/11. Y1 - 2014/12/11. N2 - BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare and heterogeneous disorder. The first line treatment of aHUS is plasma therapy, but in the past few years, the recommendations have changed greatly with the advent of eculizumab, a humanized monoclonal anti C5-antibody. Although recent recommendations suggest using it as a primary treatment for aHUS, important questions have arisen about the necessity of immediate use of eculizumab in all cases. We aimed to draw attention to a specific subgroup of aHUS ...
https://hungary.pure.elsevier.com/en/publications/first-line-therapy-in-atypical-hemolytic-uremic-syndrome-consider
Atypical hemolytic uremic syndrome: a case reportAtypical hemolytic uremic syndrome: a case report
ConclusionThe atypical hemolytic uremic syndrome is a rare disease entity requiring a high index of suspicion to diagnose. It is a diagnosis of exclusion. Early diagnosis with prompt treatment will render a better outcome. The atypical hemolytic uremic syndrome needs to be considered in all patients with thrombotic microangiopathy....
https://medworm.com/758933819/atypical-hemolytic-uremic-syndrome-a-case-report/
A receptor for the complement regulator factor H increases transmission of trypanosomes to tsetse flies. - Oxford Neuroscience
Persistent pathogens have evolved to avoid elimination by the mammalian immune system including mechanisms to evade complement. Infections with African trypanosomes can persist for years and cause human and animal disease throughout sub-Saharan Africa. It is not known how trypanosomes limit the action of the alternative complement pathway. Here we identify an African trypanosome receptor for mammalian factor H, a negative regulator of the alternative pathway. Structural studies show how the receptor binds ligand, leaving inhibitory domains of factor H free to inactivate complement C3b deposited on the trypanosome surface. Receptor expression is highest in developmental stages transmitted to the tsetse fly vector and those exposed to blood meals in the tsetse gut. Receptor gene deletion reduced tsetse infection, identifying this receptor as a virulence factor for transmission. This demonstrates how a pathogen evolved a molecular mechanism to increase transmission to an insect vector by exploitation of a
https://www.neuroscience.ox.ac.uk/publications/1095214
Safety and Efficacy Study of OMS721 in Patients With Atypical Hemolytic Uremic Syndrome - Full Text View - ClinicalTrials.govSafety and Efficacy Study of OMS721 in Patients With Atypical Hemolytic Uremic Syndrome - Full Text View - ClinicalTrials.gov
This is a Phase 3, multicenter study of OMS721 in adults and adolescents with atypical hemolytic uremic syndrome (aHUS). The uncontrolled, open-label study will evaluate the effect of OMS721 in subjects with plasma therapy-resistant aHUS and plasma therapy-responsive aHUS. This study has four periods: Screening, Treatment Induction, Treatment Maintenance, and Follow-up. Approximate enrollment is 80 subjects. An interim analysis will be performed after 40 subjects have completed 26 weeks of treatment for potential registration ...
https://clinicaltrials.gov/ct2/show/study?term=oms721&rank=1
Eculizumab Is an Effective Treatment for Atypical Hemolytic Uremic Syndrome in Patients with or without Identified Genetic...Eculizumab Is an Effective Treatment for Atypical Hemolytic Uremic Syndrome in Patients with or without Identified Genetic...
Abstract. Abstract 2085Introduction:. Atypical hemolytic uremic syndrome (aHUS) is a rare chronic disorder characterized by persistent uncontrolled complement
https://ashpublications.org/blood/article/120/21/2085/85509/Eculizumab-Is-an-Effective-Treatment-for-Atypical
Eculizumab for the treatment of atypical hemolytic uremic syndrome recurrence after kidney transplantation associated with...Eculizumab for the treatment of atypical hemolytic uremic syndrome recurrence after kidney transplantation associated with...
Zuber J, Le Quintrec M, Krid S, Bertoye C, Gueutin V, Lahoche A et al (2012) Eculizumab for atypical hemolytic uremic syndrome recurrence in renal transplantation. Am J Transplant 12(12):3337-3354. doi:10.1111/j.1600-6143.2012.04252.x CrossRefPubMedGoogle Scholar ...
https://link.springer.com/article/10.1007%2Fs11255-016-1234-y
CFHR2 gene - Genetics Home ReferenceCFHR2 gene - Genetics Home Reference
From NCBI Gene:. This gene belongs to a family of complement factor H-related genes (CFHR), which are clustered together with complement factor H gene on chromosome 1, and are involved in regulation of complement. Mutations in CFHR genes have been associated with dense deposit disease and atypical haemolytic-uraemic syndrome. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Aug 2015]. From UniProt: ...
https://ghr.nlm.nih.gov/gene/CFHR2
Staff Profile - Faculty of Medical Sciences - Newcastle UniversityStaff Profile - Faculty of Medical Sciences - Newcastle University
Brocklebank V, Johnson S, Sheerin TP, Marks SD, Gilbert RD, Tyerman K, Kinoshita M, Awan A, Kaur A, Webb N, Hegde S, Finlay E, Fitzpatrick M, Walsh P, Wong EKS, Booth C, Kerecuk L, Salama A, Almond M, Inward C, Goodship TH, Sheerin N, Marchbank KJ, Kavanagh D. Factor H autoantibody is associated with atypical hemolytic uremic syndrome in children in the United Kingdom and Ireland. Kidney International 2017, 92(5), Pages 1261-1271 ...
https://www.ncl.ac.uk/medical-sciences/people/profile/patrickwalsh.html
Microbial complement inhibitors as vaccines. - NextBio article
Complement inhibiting surface proteins of pathogenic bacteria provide candidates for vaccines because of two reasons. First, an immune response against them would recognize the microbes and secondly, it would neutralize the key bacterial virulence mechanism. Prerequisites for a vaccine protein include the following: (i) it should show limited variability, (ii) it should be immunogenic and the immune response against it should cover a sufficiently broad range of microbial strains, (iii) it should not be hidden beneath a capsule, long LPS O-polysaccharide side chains or a protein coat and (iv) it should not raise unwanted immune responses against host structures. Bacterial complement inhibitors often act by binding the soluble inhibitors factor H or C4 bp, by blocking C3 or C5 activation or by enzymatically cleaving key complement components. Inhibitors have been found from all major types of pathogens and may offer promise as rational vaccine candidates for preventing diseases such as ...
https://accounts.public.ce.basespace.illumina.com/b/search/article.nb?id=19388175
Soliris® (eculizumab) Inhibits TMA and Improves Renal Function in Pediatric and Adult Patients withSoliris® (eculizumab) Inhibits TMA and Improves Renal Function in Pediatric and Adult Patients with
CHESHIRE, CT, Nov. 9, 2013- Soliris® (eculizumab) Inhibits TMA and Improves Renal Function in Pediatric and Adult Patients with atypical Hemolytic Uremic Syndrome (aHUS).
http://www.newswire.ca/news-releases/soliris-eculizumab-inhibits-tma-and-improves-renal-function-in-pediatric-and-adult-patients-with-atypical-hemolytic-uremic-syndrome-ahus-513214471.html
Global Dialysis - Announcing Indias first Atypical HUS Registry!
Atypical Hemolytic Uremic Syndrome is an ultra-rare disease affecting a small number of patients world-wide. This devastating disease affects childre...
http://mail.globaldialysis.com/blogs/kamaldshah/item/577-announcing-indias-first-atypical-hus-registry.html
New insight into common cause of blindnessNew insight into common cause of blindness
Dr Simon Clark, a Medical Research Council Career Development Fellow, led the research: FHL-1 is a smaller version of FH, in fact it is about a third of the size. However, it has all the necessary components to regulate the immune system and is still subject to the genetic alterations that affect AMD risk. Our research has shown that the FHL-1, because it is smaller than FH, can get into structures of the back of the eye which cannot be reached by the larger FH.. He continues: Therefore, this research suggests that it is FHL-1 rather than FH which protects the back of the eye from immune attack and that insufficient FHL-1 in the back of the eye may result in inflammation that eventually results in vision loss from AMD. FHL-1, although very similar to FH in many ways, does have a totally unique tail structure at its end. This tail seems to mediate how FHL-1 binds tissue. As such, this work has identified a new target for therapeutics aimed at readdressing immune imbalance in the eye, thereby ...
http://www.innovations-report.com/html/reports/life-sciences/new-insight-into-common-cause-of-blindness.html
Tissue expression of CFHR2 - Staining in testis - The Human Protein AtlasTissue expression of CFHR2 - Staining in testis - The Human Protein Atlas
Expression of CFHR2 (CFHL2, FHR2, HFL3) in testis tissue. Antibody staining with HPA038915, HPA040726 and HPA049813 in immunohistochemistry.
http://www.proteinatlas.org/ENSG00000080910-CFHR2/tissue/testis
CFHR1 (Human) Matched Antibody Pair - (H00003078-AP61) - Products - Abnova
This antibody pair set comes with matched antibody pair to detect and quantify protein level of human CFHR1. (H00003078-AP61) - Products - Abnova
http://www.abnova.com/products/products_detail.asp?catalog_id=H00003078-AP61
Site | AEDESite | AEDE
the new mark and basic instructions, as well as download the relevant files. Moving forward, by ... and files that combine the Extension mark with the 4-H emblem have been created for your use. ... apparel guidelines, and it will be built into 4-H-related templates on the CFAES brand site .... ...
https://aede.osu.edu/search/site/sites%20aede%20files%20syllabuses%20AEDE%204597%20AU18%20Dadzie%20pdf?f%5B0%5D=hash%3An0w4rm&f%5B1%5D=hash%3A1i6uos&f%5B2%5D=hash%3A2phugs
This show is very much realistic | 1589630 | Saas Bina Sasuraal ForumThis show is very much realistic | 1589630 | Saas Bina Sasuraal Forum
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Work with us
Virtual Internship: We are looking for interns from Pakistan and abroad. Our virtual internship offers the opportunity to work with our team to further the objectives of the CFHR!. Part-time, Unpaid Position. Deadline: 15th September 2020. For more information please email us at [email protected] ...
http://cfhr.ucl.edu.pk/get-involved/work-with-us/
CFH健康知識網 - Dec. 2011CFH健康知識網 - Dec. 2011
CFH健康知識網,健康知識庫,健康知識中心.專業健康文章,康活健康知識網,健康百科,健康生活家,疾病,疾病簡介,保健, 預防,慢性病,慢性病預防,營養成分,
http://cfh.com.tw/HealthTopic/General/%E5%81%A5%E5%BA%B7%E7%9D%A1%E7%9C%A0/Dec--2011.aspx
Atypical hemolytic uremic syndrome and mutation analysis of factor H g | IJNRDAtypical hemolytic uremic syndrome and mutation analysis of factor H g | IJNRD
Atypical hemolytic uremic syndrome and mutation analysis of factor H gene in two Tunisian families Imen Habibi1,Imen Sfar1,Walid Ben Alaya1, Jihen Methlouthi2, Abdelkrim Ayadi2, Mounira Brahim2, Jacques Blouin3, Raoudha Dhagbouj1, Thouraya Ben Rhomdhane1, Mouna Makhlouf1, Houda Aouadi1, Saloua Ayed-Jendoubi1, Véronique Fremeaux-bacchi3, Tahar Sfar2, Taieb Ben Abdallah1, Khaled Ayed1, Yousr Gorgi11Laboratory of Immunology, Charles Nicolle Hospital, Tunis, Tunisia; 2Paediatric Department, Tahar Sfar Hospital, Mahdia, Tunisia; 3Immunology Department, George Pompidou Hospital, Paris, FranceAbstract: We carried out a protein and genetic investigation of the ¬factor H gene mutations within two families presenting with a diagnostic suspicion of atypical hemolytic uremic syndrome (aHUS). The results within the patients of the first family revealed a factor H-deficiency. Direct sequencing allowed the detection of a 4-nucleotide deletion in the factor H gene. This deletion was found as the homozygote
https://www.dovepress.com/atypical-hemolytic-uremic-syndrome-and-mutation-analysis-of-factor-h-g-peer-reviewed-fulltext-article-IJNRD
Atypical hemolytic uremic syndrome - WikipediaAtypical hemolytic uremic syndrome - Wikipedia
Atypical hemolytic uremic syndrome (aHUS) is an extremely rare, life-threatening, progressive disease that frequently has a genetic component. In most cases it is caused by chronic, uncontrolled activation of the complement system, a branch of the bodys immune system that destroys and removes foreign particles. The disease affects both children and adults and is characterized by systemic thrombotic microangiopathy (TMA), the formation of blood clots in small blood vessels throughout the body, which can lead to stroke, heart attack, kidney failure, and death. The complement system activation may be due to mutations in the complement regulatory proteins (factor H, factor I, or membrane cofactor protein), or is occasionally due to acquired neutralizing autoantibody inhibitors of these complement system components, for example anti-factor H antibodies. Despite the use of supportive care, historically an estimated 33-40% of patients died or developed end-stage renal disease (ESRD) with the first ...
https://en.wikipedia.org/wiki/Atypical_hemolytic_uremic_syndrome
A novel CFHR5 mutation associated with C3 glomerulonephritis in a Turkish girl | AVESİSA novel CFHR5 mutation associated with C3 glomerulonephritis in a Turkish girl | AVESİS
C3 glomerulopathy defines a subgroup of membranoproliferative glomerulonephritis (MPGN) characterized by complement 3 (C3)-positive, immunoglobulin-negative deposits in immunofluorescence microscopy. It comprises 3 clinical conditions: dense deposit disease, C3 glomerulonephritis, and complement factor H-related 5 (CFHR5) nephropathy. Mutations in genes encoding regulatory proteins of the alternative complement pathway have been described. A 16-year-old girl was admitted to the hospital due to periorbital edema. Nephrotic syndrome accompanied by low C3 level was diagnosed. Renal biopsy showed MPGN in light microscopy, only C3 deposits in immunofluorescence microscopy, and subendothelial electron dense deposits and capillary basement membrane thickening with double contour formation in electron microscopy. C3 nephritic factor and anti complement factor H antibody were negative. Complement factor H level was normal. Genetic screening showed a novel heterozygous p.Cys269Arg variation in the CFHR5 ...
https://avesis.hacettepe.edu.tr/yayin/7c0c2238-7fe1-48fd-84f7-84ade3591ec0/a-novel-cfhr5-mutation-associated-with-c3-glomerulonephritis-in-a-turkish-girl
Triggering of atypical hemolytic uremic syndrome by influenza A (H1N1)Triggering of atypical hemolytic uremic syndrome by influenza A (H1N1)
We report a case of atypical hemolytic uremic syndrome (aHUS) triggered by influenza A (H1N1) in a 17-year-old boy with a mutation in the gene (CD46) encoding the transmembrane complement regulator membrane cofactor protein. The patient recovered completely following treatment with oseltamivir, plas …
https://pubmed.ncbi.nlm.nih.gov/20540647/?dopt=Abstract
Partial Treatment with Exchange Transfusion in Neonatal Atypical Hemolytic Uremic Syndrome: Case Report | AbstractPartial Treatment with Exchange Transfusion in Neonatal Atypical Hemolytic Uremic Syndrome: Case Report | Abstract
Neonatal atypical hemolytic uremic syndrome is characterized by hemolytic anemia, thrombocytopenia and thrombotic microangiopathy. Disease caused by a..
https://clinical-experimental-nephrology.imedpub.com/abstract/partial-treatment-with-exchange-transfusion-in-neonatal-atypical-hemolytic-uremic-syndrome-case-report-32613.html
CFHR5 gene - Genetics Home ReferenceCFHR5 gene - Genetics Home Reference
Several mutations in the CFHR5 gene have been found to cause a rare form of kidney disease called C3 glomerulopathy. This disorder damages the kidneys and can lead to end-stage renal disease (ESRD), a life-threatening condition that prevents the kidneys from filtering fluids and waste products from the body effectively.. The most common CFHR5 gene mutation has been identified in people from the Mediterranean island of Cyprus. This genetic change abnormally copies (duplicates) regions of the CFHR5 gene known as exons 2 and 3. The duplication alters the structure and function of complement factor H-related 5, preventing it from regulating the complement system effectively. As a result, the complement system becomes overactive, which damages structures called glomeruli in the kidneys. These structures are clusters of tiny blood vessels that help filter waste products from the blood. Damage to glomeruli prevents the kidneys from filtering waste products normally and can lead to ESRD.. Several other ...
https://ghr.nlm.nih.gov/gene/CFHR5
PDB: 2XRC
HEADER IMMUNE SYSTEM 13-SEP-10 2XRC TITLE HUMAN COMPLEMENT FACTOR I CAVEAT 2XRC NAG A 646 HAS WRONG CHIRALITY AT ATOM C1 NAG A 659 HAS WRONG CAVEAT 2 2XRC CHIRALITY AT ATOM C1 NAG A 676 HAS WRONG CHIRALITY AT ATOM CAVEAT 3 2XRC C1 NAG B 646 HAS WRONG CHIRALITY AT ATOM C1 NAG D 646 HAS CAVEAT 4 2XRC WRONG CHIRALITY AT ATOM C1 COMPND MOL_ID: 1; COMPND 2 MOLECULE: HUMAN COMPLEMENT FACTOR I; COMPND 3 CHAIN: A, B, C, D; COMPND 4 SYNONYM: C3B/C4B INACTIVATOR, COMPLEMENT FACTOR I HEAVY CHAIN, COMPND 5 COMPLEMENT FACTOR I LIGHT CHAIN, KAF; COMPND 6 EC: 3.4.21.45 SOURCE MOL_ID: 1; SOURCE 2 ORGANISM_SCIENTIFIC: HOMO SAPIENS; SOURCE 3 ORGANISM_COMMON: HUMAN; SOURCE 4 ORGANISM_TAXID: 9606; SOURCE 5 TISSUE: SERUM KEYWDS IMMUNE SYSTEM, HYDROLASE, CONGLUTINOGEN ACTIVATING FACTOR, SERINE KEYWDS 2 PROTEASE, COMPLEMENT SYSTEM EXPDTA X-RAY DIFFRACTION AUTHOR P.ROVERSI,S.JOHNSON,S.M.LEA REVDAT 3 29-JUL-20 2XRC 1 CAVEAT COMPND REMARK HETNAM REVDAT 3 2 1 LINK SITE REVDAT 2 17-AUG-11 2XRC 1 JRNL REVDAT 1 13-JUL-11 ...
https://www.genome.jp/entry/pdb:2XRC
Open Label Controlled Trial of Eculizumab in Adult Patients With Plasma Therapy-sensitive Atypical Hemolytic Uremic Syndrome ...Open Label Controlled Trial of Eculizumab in Adult Patients With Plasma Therapy-sensitive Atypical Hemolytic Uremic Syndrome ...
The purpose of this study is to determine whether eculizumab is safe and effective in the treatment of adult patients with plasma therapy-sensitive Atypical Hemolytic-Uremic Syndrome (aHUS).
https://ichgcp.net/clinical-trials-registry/NCT00838513
aHUS and Complement Dysregulation | aHUS SourceaHUS and Complement Dysregulation | aHUS Source
Information for healthcare professionals for diagnosing and treating Atypical Hemolytic Uremic Syndrome. Soliris is the only therapy approved for the treatment of aHUS.
https://ahussource.com/Physician/ComplementAHUS
抗Complement Factor H抗体产品概述抗Complement Factor H抗体产品概述
购买单克隆、多克隆可使用于多种应用范围的Complement Factor H抗体。精选反Complement Factor H抗体生产商。
https://www.antibodies-online.cn/complement-system-pathway-11/complement-factor-h-antibody-59/
aHUS May be Associated With Heart Disease, Case Report SaysaHUS May be Associated With Heart Disease, Case Report Says
Read about a case report study describing the clinical case of a patient with atypical hemolytic uremic syndrome (aHUS) associated with heart disease.
https://ahusnews.com/2019/06/05/ahus-may-be-associated-with-heart-disease-case-report-says/
Human complement factor 3 polymorphism determination by capillary electrophoresis of serum - De Witte - 2012 - ELECTROPHORESIS ...Human complement factor 3 polymorphism determination by capillary electrophoresis of serum - De Witte - 2012 - ELECTROPHORESIS ...
If you are a society or association member and require assistance with obtaining online access instructions please contact our Journal Customer Services team ...
http://onlinelibrary.wiley.com/doi/10.1002/elps.201100451/pdf
Global Dialysis - FDA approves Soliris (Eculizumab) for atypical HUS
In a significant development the FDA in the USA approved Soliris (Eculizumab) for use in atypical Hemolytic Uremic Syndrome. To those saying,
http://www.globaldialysis.com/blogs/big-d-and-me/item/340-fda-approves-soliris-eculizumab-for-atypical-hus.html
Most recent papers with the keyword Lambris | Read by QxMDMost recent papers with the keyword Lambris | Read by QxMD
The complement system labels microbes and host debris for clearance. Degradation of surface-bound C3b is pivotal to direct immune responses and protect host cells. How the serine protease factor I (FI), assisted by regulators, cleaves either two or three distant peptide bonds in the CUB domain of C3b remains unclear. We present a crystal structure of C3b in complex with FI and regulator factor H (FH; domains 1-4 with 19-20). FI binds C3b-FH between FH domains 2 and 3 and a reoriented C3b C-terminal domain and docks onto the first scissile bond, while stabilizing its catalytic domain for proteolytic activity ...
https://www.readbyqxmd.com/keyword/21491
Tissue expression of CFHR2 - Staining in esophagus - The Human Protein AtlasTissue expression of CFHR2 - Staining in esophagus - The Human Protein Atlas
Expression of CFHR2 (CFHL2, FHR2, HFL3) in esophagus tissue. Antibody staining with HPA038915, HPA040726 and HPA049813 in immunohistochemistry.
http://www.proteinatlas.org/ENSG00000080910-CFHR2/tissue/esophagus
AT2G02650 expression profile
Description : Ribonuclease H-like superfamily protein. Condition specificity: Seedlings, etiolated (SPM: 0.72, entropy: 0.47, tau: 0.97) ...
https://conekt.sbs.ntu.edu.sg/profile/view/10009
Rusia bina peluru berpandu terpantas - Suara MerdekaRusia bina peluru berpandu terpantas - Suara Merdeka
SEBUAH peluru berpandu rentas benua baharu dibangunkan Rusia didakwa mampu terbang 27 kali ganda kelajuan bunyi. Senjata berkenaan dikatakan mampu men
https://suaramerdeka.com.my/rusia-bina-peluru-berpandu-terpantas/
FBA倉庫地址清單
我們可以幫您構建一個更高穩定性和安全性的物流體系，降低運輸的難度和運營成本，讓您可以減少花在貨運上的精力和時間，更專注于您的業務創新。 ...
http://www.rcnclw.icu/Manage/WebManage/OrderCommonPage.aspx?ParentID=510&StyleID=511
Complement factor IComplement factor I
... , also known as C3b/C4b inactivator, is a protein that in humans is encoded by the CFI gene. Complement ... either C3b or C4b) and a cofactor protein (Factor H, C4b-binding protein, complement receptor 1, and membrane cofactor protein ... Whaley K (March 1980). "Biosynthesis of the complement components and the regulatory proteins of the alternative complement ... is a protein of the complement system, first isolated in 1966 in guinea pig serum, that regulates complement activation by ...
https://en.wikipedia.org/wiki/Complement_factor_I
Alternative complement pathwayAlternative complement pathway
Factor I requires a C3b-binding protein cofactor such as complement factor H, CR1, or Membrane Cofactor of Proteolysis (MCP or ... Pangburn MK, Schreiber RD, Müller-Eberhard HJ (July 1977). "Human complement C3b inactivator: isolation, characterization, and ... cells from complement-mediated damage. CFHR5 (Complement Factor H-Related protein 5) is able to bind to act as a cofactor for ... Since C3b is free and abundant in the plasma, it can bind to either a host cell or a pathogen surface. To prevent complement ...
https://en.wikipedia.org/wiki/Alternative_complement_pathway
C3-convertaseC3-convertase
C4b-binding protein inhibits the haemolytic function of cell-bound C4b. C4b-binding protein and C3b inactivator control the C3 ... complement receptor 1 (CR1), C4b-binding protein and Factor H. Convertase assembly is suppressed by the proteolytic cleavage of ... "Modulation of the Classical Pathway C3 Convertase by Plasma Proteins C4 Binding Protein and C3b Inactivator". Proc Natl Acad ... in addition to enhancing proteolytic inactivation of C3b by C3b inactivator (C3bINA - endopeptidase). Membrane-associated ...
https://en.wikipedia.org/wiki/C3-convertase
List of MeSH codes (D12.776.124)List of MeSH codes (D12.776.124)
... complement c3b inactivator proteins MeSH D12.776.124.486.274.920.325.200 - complement factor h MeSH D12.776.124.486.274.920. ... complement c1 inactivator proteins MeSH D12.776.124.486.274.920.250.500 - complement c1 inhibitor protein MeSH D12.776.124.486. ... complement factor b MeSH D12.776.124.486.274.920 - complement inactivator proteins MeSH D12.776.124.486.274.920.124 - antigens ... complement c3 MeSH D12.776.124.486.274.250.250 - complement c3a MeSH D12.776.124.486.274.250.260 - complement c3b MeSH D12.776. ...
https://en.wikipedia.org/wiki/List_of_MeSH_codes_(D12.776.124)
Complement control proteinComplement control protein
Complement C3b/C4b Receptor 1, CR1 (CD35) Complement Regulator of the Immunoglobulin Superfamily, CRIg Soluble complement ... Complement+Inactivator+Proteins at the US National Library of Medicine Medical Subject Headings (MeSH) (CS1: long volume value ... Complement control protein are proteins that interact with components of the complement system. The complement system is ... Complement control proteins also play a role in malignancy. Complement proteins protect against malignant cells- both by direct ...
https://en.wikipedia.org/wiki/Complement_control_protein
CFI gene: MedlinePlus GeneticsCFI gene: MedlinePlus Genetics
The CFI gene provides instructions for making a protein called complement factor I. Learn about this gene and related health ... C3B/C4B inactivator. *C3BINA. *CFAI_HUMAN. *complement component I. *complement control protein factor I ... The CFI gene provides instructions for making a protein called complement factor I. This protein helps regulate a part of the ... Complement factor I and several related proteins protect healthy cells by preventing activation of the complement system when ...
https://medlineplus.gov/genetics/gene/cfi/
DeCSDeCS
C4b-C3b Inactivator Complement 4b Binding Protein Complement C3b C4b Inactivator Cofactor Complement C3b-C4b Inactivator ... C4b-C3b Inactivator. Complement 4b Binding Protein. Complement C3b C4b Inactivator Cofactor. Complement C3b-C4b Inactivator ... C4b Binding Protein C4b C3b Inactivator Cofactor C4b-Binding Protein C4b-Binding Protein, Complement C4b-C3b Inactivator ... C4b Binding Protein. C4b C3b Inactivator Cofactor. C4b-Binding Protein. C4b-Binding Protein, Complement. C4b-C3b Inactivator ...
https://decs.bvsalud.org/en/ths/resource/?id=50837
Complement cascade and its inhibitors | AbcamComplement cascade and its inhibitors | Abcam
Learn about the three pathways lead to complement activation and some of their key inhibitors. ... Complement inhibitors include the plasma serine proteinase inhibitor serpin (C1 inactivator). The plasma proteins, Factor I and ... Note, C3b generated in the classical pathway feeds into the alternative pathway to amplify the activation of complement. ... Most of the C3b generated by the convertase is hydrolysed. However, if C3b comes into contact with an invading micro-organism ...
https://www.abcam.com/pathways/complement-cascade-and-its-inhibitors
DeCS 2017 - December 21, 2017 versionDeCS 2017 - December 21, 2017 version
Factors, Conglutinogen Activating use Complement C3b Inactivator Proteins Factors, Cord use Cord Factors ... FADD Protein, Fas Associating use Fas-Associated Death Domain Protein FADD Protein, Fas-Associating use Fas-Associated Death ... FADD, Fas-Associating Protein use Fas-Associated Death Domain Protein Fade, Neuromuscular use Refractory Period, ... F Box and WD 40 Domain Containing Protein 7 use F-Box-WD Repeat-Containing Protein 7 ...
https://decs.bvs.br/I/DeCS2017_Alfab-F.htm
MeSH BrowserMeSH Browser
Complement 4b Binding Protein Complement C3b-C4b Inactivator Cofactor Complement Component 4b-Binding Protein Pharm Action. ... Complement C3b Inactivator Proteins [D12.776.124.486.274.920.325] * Complement C4b-Binding Protein [D12.776.124.486.274.920.662 ... Complement System Proteins [D12.776.124.486.274] * Complement Inactivator Proteins [D12.776.124.486.274.920] * Membrane ... Complement C1 Inactivator Proteins [D12.776.124.486.274.920.250] * Complement C3 Nephritic Factor [D12.776.124.486.274.920.287] ...
https://meshb-prev.nlm.nih.gov/record/ui?ui=D050716
DeCS 2017 - December 21, 2017 version
Factors, Conglutinogen Activating use Complement C3b Inactivator Proteins Factors, Cord use Cord Factors ... FADD Protein, Fas Associating use Fas-Associated Death Domain Protein FADD Protein, Fas-Associating use Fas-Associated Death ... FADD, Fas-Associating Protein use Fas-Associated Death Domain Protein Fade, Neuromuscular use Refractory Period, ... F Box and WD 40 Domain Containing Protein 7 use F-Box-WD Repeat-Containing Protein 7 ...
https://decs2019.bvsalud.org/I/DeCS2017_Alfab-F.htm
DeCS 2018 - July 31, 2018 version
Factors, Conglutinogen Activating use Complement C3b Inactivator Proteins Factors, Cord use Cord Factors ... FADD Protein, Fas Associating use Fas-Associated Death Domain Protein FADD Protein, Fas-Associating use Fas-Associated Death ... FADD, Fas-Associating Protein use Fas-Associated Death Domain Protein Fade, Neuromuscular use Refractory Period, ... F Box and WD 40 Domain Containing Protein 7 use F-Box-WD Repeat-Containing Protein 7 ...
https://decs2018.bvsalud.org/I/DeCS2018_Alfab-F.htm
DeCS 2017 - July 04, 2017 version
Factors, Conglutinogen Activating use Complement C3b Inactivator Proteins Factors, Cord use Cord Factors ... FADD Protein, Fas Associating use Fas-Associated Death Domain Protein FADD Protein, Fas-Associating use Fas-Associated Death ... FADD, Fas-Associating Protein use Fas-Associated Death Domain Protein Fade, Neuromuscular use Refractory Period, ... Factor-1, Myeloid Cell use Myeloid Cell Leukemia Sequence 1 Protein Factor-4A1, Eukaryotic Initiation use Eukaryotic Initiation ...
https://decs2017.bvsalud.org/I/DeCS2017_Alfab-F.htm
DeCS 2020 - June 23, 2020 version
Factors, Conglutinogen Activating use Complement C3b Inactivator Proteins Factors, Cord use Cord Factors ... FADD Protein, Fas Associating use Fas-Associated Death Domain Protein FADD Protein, Fas-Associating use Fas-Associated Death ... FADD, Fas-Associating Protein use Fas-Associated Death Domain Protein Fade, Neuromuscular use Refractory Period, ... F Box and WD 40 Domain Containing Protein 7 use F-Box-WD Repeat-Containing Protein 7 ...
https://decs2020.bvsalud.org/I/DeCS2020_Alfab-F.htm
Complement - pediagenosisComplement - pediagenosis
C3b inactivator enzymes rapidly inactivate C3b, releasing the fragment C3c and leaving membrane bound C3d.. ... MBL Mannose-binding lectin (also variously referred to as mannose binding protein or mannan-binding protein), a C1q-like ... Complement. Fifteen or more serum components constitute the complement system, the sequential activation and assembly into ... Complement inhibitors. In order to prevent over-activation of the complement cascade, there are numerous inhibitory mechanisms ...
https://www.pediagenosis.com/2018/07/complement.html
DeCS 2006 - Changed terms
Complement 3a. Complement C3a. Complement 3b. Complement C3b. Complement 3b Inactivators. Complement C3b Inactivator Proteins. ... Complement Inactivators. Complement Inactivator Proteins. DNA-Binding Protein, Cyclic AMP-Responsive. Cyclic AMP Response ... Proto-Oncogene Protein c-met. Proto-Oncogene Proteins c-met. Proto-Oncogene Protein p21(ras). Proto-Oncogene Proteins p21(ras) ... Complement. Complement System Proteins. Complement 1. Complement C1. Complement 1 Inactivators. Complement C1 Inactivator ...
https://decs2012.bvsalud.org/I/rep_i2006.htm
DeCS 2006 - Changed terms
Complement 3a. Complement C3a. Complement 3b. Complement C3b. Complement 3b Inactivators. Complement C3b Inactivator Proteins. ... Complement Inactivators. Complement Inactivator Proteins. DNA-Binding Protein, Cyclic AMP-Responsive. Cyclic AMP Response ... Proto-Oncogene Protein c-met. Proto-Oncogene Proteins c-met. Proto-Oncogene Protein p21(ras). Proto-Oncogene Proteins p21(ras) ... Complement. Complement System Proteins. Complement 1. Complement C1. Complement 1 Inactivators. Complement C1 Inactivator ...
https://decs2008.bvsalud.org/I/rep_i2006.htm
DeCS 2006 - Changed terms
Complement 3a. Complement C3a. Complement 3b. Complement C3b. Complement 3b Inactivators. Complement C3b Inactivator Proteins. ... Complement Inactivators. Complement Inactivator Proteins. DNA-Binding Protein, Cyclic AMP-Responsive. Cyclic AMP Response ... Proto-Oncogene Protein c-met. Proto-Oncogene Proteins c-met. Proto-Oncogene Protein p21(ras). Proto-Oncogene Proteins p21(ras) ... Complement. Complement System Proteins. Complement 1. Complement C1. Complement 1 Inactivators. Complement C1 Inactivator ...
https://decs2010.bvsalud.org/I/rep_i2006.htm
DeCS 2006 - Changed terms
Complement 3a. Complement C3a. Complement 3b. Complement C3b. Complement 3b Inactivators. Complement C3b Inactivator Proteins. ... Complement Inactivators. Complement Inactivator Proteins. DNA-Binding Protein, Cyclic AMP-Responsive. Cyclic AMP Response ... Proto-Oncogene Protein c-met. Proto-Oncogene Proteins c-met. Proto-Oncogene Protein p21(ras). Proto-Oncogene Proteins p21(ras) ... Complement. Complement System Proteins. Complement 1. Complement C1. Complement 1 Inactivators. Complement C1 Inactivator ...
https://decs2014.bvsalud.org/I/rep_i2006.htm
DeCS 2006 - Changed terms
Complement 3a. Complement C3a. Complement 3b. Complement C3b. Complement 3b Inactivators. Complement C3b Inactivator Proteins. ... Complement Inactivators. Complement Inactivator Proteins. DNA-Binding Protein, Cyclic AMP-Responsive. Cyclic AMP Response ... Proto-Oncogene Protein c-met. Proto-Oncogene Proteins c-met. Proto-Oncogene Protein p21(ras). Proto-Oncogene Proteins p21(ras) ... Complement. Complement System Proteins. Complement 1. Complement C1. Complement 1 Inactivators. Complement C1 Inactivator ...
https://decs2013.bvsalud.org/I/rep_i2006.htm
DeCS 2006 - Changed terms
Complement 3a. Complement C3a. Complement 3b. Complement C3b. Complement 3b Inactivators. Complement C3b Inactivator Proteins. ... Complement Inactivators. Complement Inactivator Proteins. DNA-Binding Protein, Cyclic AMP-Responsive. Cyclic AMP Response ... Proto-Oncogene Protein c-met. Proto-Oncogene Proteins c-met. Proto-Oncogene Protein p21(ras). Proto-Oncogene Proteins p21(ras) ... Complement. Complement System Proteins. Complement 1. Complement C1. Complement 1 Inactivators. Complement C1 Inactivator ...
https://decs2011.bvsalud.org/I/rep_i2006.htm
DeCS 2006 - Changed terms
Complement 3a. Complement C3a. Complement 3b. Complement C3b. Complement 3b Inactivators. Complement C3b Inactivator Proteins. ... Complement Inactivators. Complement Inactivator Proteins. DNA-Binding Protein, Cyclic AMP-Responsive. Cyclic AMP Response ... Proto-Oncogene Protein c-met. Proto-Oncogene Proteins c-met. Proto-Oncogene Protein p21(ras). Proto-Oncogene Proteins p21(ras) ... Complement. Complement System Proteins. Complement 1. Complement C1. Complement 1 Inactivators. Complement C1 Inactivator ...
https://decs2016.bvsalud.org/I/rep_i2006.htm
DeCS 2006 - Changed terms
Complement 3a. Complement C3a. Complement 3b. Complement C3b. Complement 3b Inactivators. Complement C3b Inactivator Proteins. ... Complement Inactivators. Complement Inactivator Proteins. DNA-Binding Protein, Cyclic AMP-Responsive. Cyclic AMP Response ... Proto-Oncogene Protein c-met. Proto-Oncogene Proteins c-met. Proto-Oncogene Protein p21(ras). Proto-Oncogene Proteins p21(ras) ... Complement. Complement System Proteins. Complement 1. Complement C1. Complement 1 Inactivators. Complement C1 Inactivator ...
https://decs2015.bvsalud.org/I/rep_i2006.htm
Complement Factor I (C3B/C4B Inactivator, CFI, IF), IgG, Mouse, Polyclonal | LabstoreComplement Factor I (C3B/C4B Inactivator, CFI, IF), IgG, Mouse, Polyclonal | Labstore
Factor I is an 88kD protein consisting of one polypeptide chain of 35.4kD and one of 27.6kD. Factor I is a serine protease ... Complement Factor I is a member of the peptidase S1 family present in the blood plasma. ... ZIK1 (Zinc Finger Protein Interacting with Ribonucleoprotein K, Zinc Finger Protein Interacting with K Protein 1), rabbit, ... MAP1LC3B (Microtubule-associated Proteins 1A/1B Light Chain 3B, Autophagy-related Protein LC3 B, Autophagy-related Ubiquitin- ...
https://labstore.ru/en/catalog/antitela-3/complement-factor-i-c3bc4b-inactivator-cfi-if-igg-mouse-polyclonal/50-mkg/
The failure to show a necessary role for C3 in the in vitro antibody response. - Immunology
2) Similarly, normal antibody responses are obtained if the cultures are grown in human serum depleted of C3b-inactivator, ... This shows that no exogenous source of complement is necessary for in vitro antibody formation by spleen cells. ( ... which contains high concentrations of C3b. (3) In the presence of antibody to mouse C3 the response to the T-independent ... and T-independent carriers as well as to sheep erythrocytes has been studied to investigate the possible role of complement ...
https://www.immunology.ox.ac.uk/publications/12306
Factor H autoantibodies and deletion of Complement Factor H-Related protein-1 in rheumatic diseases in comparison to atypical...Factor H autoantibodies and deletion of Complement Factor H-Related protein-1 in rheumatic diseases in comparison to atypical...
Complement C3b Inactivator Proteins / genetics Actions. * Search in PubMed * Search in MeSH ... Complement factor H related proteins (CFHRs). Skerka C, Chen Q, Fremeaux-Bacchi V, Roumenina LT. Skerka C, et al. Mol Immunol. ... Complement factor H-related protein 1 deficiency and factor H antibodies in pediatric patients with atypical hemolytic uremic ... Blom AM, Villoutreix BO, Dahlbäck B. Complement inhibitor C4b-binding protein - friend or foe in the innate immune system? Mol ...
https://pubmed.ncbi.nlm.nih.gov/22894814
CFI - Early...CFI - Early...
C3b-Inactivator. *CFI. *Complement Component I. *Complement Control Protein Factor I. *Complement Factor I ... This heterodimer can cleave and inactivate the complement components C4b and C3b, and it prevents the assembly of the C3 and C5 ... From NCBI Gene: This gene encodes a serine proteinase that is essential for regulating the complement cascade. The encoded ... Defects in this gene cause complement factor I deficiency, an autosomal recessive disease associated with a susceptibility to ...
https://edrn.nci.nih.gov/data-and-resources/biomarkers/cfi/
MeSH BrowserMeSH Browser
Complement 3b Inactivators Complement 3b Inhibitors Complement C3b Inactivators Complement C3b Inhibitor Proteins Conglutinogen ... Endogenous proteins that inhibit or inactivate COMPLEMENT C3B. They include COMPLEMENT FACTOR H and COMPLEMENT FACTOR I (C3b/ ... Endogenous proteins that inhibit or inactivate COMPLEMENT C3B. They include COMPLEMENT FACTOR H and COMPLEMENT FACTOR I (C3b/ ... Complement C3b Inactivator Proteins [D12.776.124.486.274.920.325] * Complement Factor H [D12.776.124.486.274.920.325.200] ...
https://meshb.nlm.nih.gov/record/ui?ui=D003180
MeSH BrowserMeSH Browser
Complement 3b Inactivators Complement 3b Inhibitors Complement C3b Inactivators Complement C3b Inhibitor Proteins Conglutinogen ... Endogenous proteins that inhibit or inactivate COMPLEMENT C3B. They include COMPLEMENT FACTOR H and COMPLEMENT FACTOR I (C3b/ ... Endogenous proteins that inhibit or inactivate COMPLEMENT C3B. They include COMPLEMENT FACTOR H and COMPLEMENT FACTOR I (C3b/ ... Complement C3b Inactivator Proteins [D12.776.124.486.274.920.325] * Complement Factor H [D12.776.124.486.274.920.325.200] ...
https://meshb-prev.nlm.nih.gov/record/ui?ui=D003180
DeCS Ingl s
D12.776.124.486.274.920 Complement Inactivator Proteins .. D12.776.124.486.274.920.325 Complement C3b Inactivator Proteins .. ... Complement .. Complement 3b Complement 4b Inactivator .. Complement C4b C3b Inactivator .. Inactivator, C3b .. Inactivator, C3b ... Complement 3b-Complement 4b Inactivator .. Complement C4b-C3b Inactivator .. C3b C4b Inactivator .. C4b C3b INA .. C4b-C3b ... Complement, Hemolytic .. Protein, Complement .. Proteins, Complement .. Proteins, Complement System .. Complement Proteins .. ...
http://trigramas.bireme.br/cgi-bin/mx/[email protected]?collection=DeCSi&lang=p&minsim=0.30&maxrel=10&text=Factor%20P,%20Complement
GSTT1
complement c3b inactivator proteins*genetic loci*ethacrynic acid*genetic variation*penetrance*vindesine*coal tar*cytogenetics* ... xeroderma pigmentosum group d protein*trihalomethanes*areca*small cell lung carcinoma*radiation exposure*hair color* ...
http://www.labome.org/gene/human/gstt1-2952.html
Complement-Related Disorders: Background, Pathophysiology, ActivationComplement-Related Disorders: Background, Pathophysiology, Activation
Complement receptor 1 (CR1) has factor H-like activity, permitting factor I to cleave C3b. Membrane cofactor protein also has ... Table 3. Proteins of the Human Complement (C) System, Lectin Pathway *Table 4. Proteins of the Human Complement (C) System, C3 ... Table 1. Proteins of the Human Complement (C) System, Classical Pathway* *Table 2. Proteins of the Human Complement (C) System ... Table 6. Proteins of the Human Complement (C) System, Membrane Receptor and Control Proteins (Open Table in a new window) ...
https://emedicine.medscape.com/article/136368-overview
NDF-RT Code NDF-RT NameNDF-RT Code NDF-RT Name
N0000169287 Complement C3a N0000169279 Complement C3b N0000169306 Complement C3b Inactivator Proteins N0000169280 Complement ... Complement C1 N0000170005 Complement C1 Inactivator Proteins N0000169305 Complement C1 Inhibitor Protein N0000169277 Complement ... Complement Factor D N0000169401 Complement Factor H N0000169307 Complement Factor I N0000169304 Complement Inactivator Proteins ... C3d N0000169282 Complement C4 N0000169290 Complement C4a N0000169283 Complement C4b N0000169308 Complement C4b-Binding Protein ...
https://evs.nci.nih.gov/ftp1/FDA/ndfrt/Archive/StructuralClass%202007-10-09.txt
Complement Factor I | Profiles RNS
Complement Inactivator Proteins [D12.776.124.486.274.920]. *Complement C3b Inactivator Proteins [D12.776.124.486.274.920.325] ... that cleaves the alpha-chains of C3b and C4b in the presence of the cofactors COMPLEMENT FACTOR H and C4-binding protein, ... It is a 66-kDa glycoprotein that converts C3b to inactivated C3b (iC3b) followed by the release of two fragments, C3c (150-kDa ... C3b C4b Inactivator. *Inactivator, C3b-C4b. *Complement 3b-Complement 4b Inactivator. *Complement 3b Complement 4b Inactivator ...
https://profiles.rush.edu/display/15179
Complement factor h. Medical search. DefinitionsComplement factor h. Medical search. Definitions
Complement Factor HComplement Factor BComplement C3b Inactivator ProteinsComplement Factor IComplement C3bComplement C3 ... Complement Factor HComplement Factor BComplement C3b Inactivator ProteinsMacular DegenerationComplement Factor IComplement C3b ... Complement C3b Inactivator Proteins. Endogenous proteins that inhibit or inactivate COMPLEMENT C3B. They include COMPLEMENT ... Complement Factor DComplement System ProteinsComplement C2Complement C3dComplement C4Complement C5Complement C5aComplement C3- ...
https://lookformedical.com/en/definitions/complement-factor-h
DeCS 2020 - June 23, 2020 versionDeCS 2020 - June 23, 2020 version
Factors, Conglutinogen Activating use Complement C3b Inactivator Proteins Factors, Cord use Cord Factors ... FADD Protein, Fas Associating use Fas-Associated Death Domain Protein FADD Protein, Fas-Associating use Fas-Associated Death ... FADD, Fas-Associating Protein use Fas-Associated Death Domain Protein Fade, Neuromuscular use Refractory Period, ... F Box and WD 40 Domain Containing Protein 7 use F-Box-WD Repeat-Containing Protein 7 ...
https://decs.bvsalud.org/I/DeCS2020_Alfab-F.htm
MESH TREE NUMBER CHANGES - 2006 MeSH. August 19, 2005
Complement 3b Inactivators D24.611.333.920.325 (Replaced for 2006 by Complement C3b Inactivator Proteins) Complement 3c D12.776 ... Replaced for 2006 by Complement C1 Inactivator Proteins) Complement 1q D24.611.333.50.270 (Replaced for 2006 by Complement C1q ... Replaced for 2006 by Complement System Proteins) Complement 1 D24.611.333.50 (Replaced for 2006 by Complement C1) Complement 1 ... Replaced for 2006 by Complement Inactivator Proteins) Complement Membrane Attack Complex D24.611.333.930 Complementarity ...
https://nlmpubs.nlm.nih.gov/projects/mesh/1999-2010/newterms/mnchg2006.txt
µµ
... complement inhibitors C1 inhibitor C1 inhibitors C1q C3 C3 activator C3 activators C3bi C3b INA C3b inactivator C3b inactivator ... protein Bence Jones protein den Bence Jones protein Den Bence Jones proteins Bence-Jones proteins Bence Jones protein SUT Bence ... accelerator C3b inactivators C3b INAs C3b INAs c3b inhibitor c3b inhibitors C3/C5 convertase C3 convertase C3H C3H mice C3H ... blood-pressures blood protein blood protein disorder blood protein disorders blood protein electrophoreses blood protein ...
https://lexsrv3.nlm.nih.gov/LexSysGroup/Projects/gSpell/current/data/2006LexiconTerms.txt
µµ
... complement inhibitors C1 inhibitor C1 inhibitors C1q C3 C3 activator C3 activators C3bi C3b INA C3b inactivator C3b inactivator ... protein Bence Jones protein den Bence Jones protein Den Bence Jones proteins Bence-Jones proteins Bence Jones protein SUT Bence ... accelerator C3b inactivators C3b INAs C3b INAs c3b inhibitor c3b inhibitors C3/C5 convertase C3 convertase C3H C3H mice C3H ... blood-pressures blood protein blood protein disorder blood protein disorders blood protein electrophoreses blood protein ...
https://lsg3.nlm.nih.gov/LexSysGroup/Projects/gSpell/current/data/2006LexiconTerms.txt
Antigens, CD46 | Profiles RNS
Complement C1 Inactivator Proteins. *Complement C3 Nephritic Factor. *Complement C3b Inactivator Proteins ... A ubiquitously expressed complement receptor that binds COMPLEMENT C3B and COMPLEMENT C4B and serves as a cofactor for their ... Complement System Proteins [D12.776.124.486.274]. *Complement Inactivator Proteins [D12.776.124.486.274.920] ...
https://researchers.childrensmercy.org/display/26302
Code System Concept
Complement factor H Current Synonym true false 55070010 C3b inactivator accelerator Current Synonym true false ... Complement regulatory factor (substance) {259788001 , SNOMED-CT } Complement regulatory protein, plasma (substance) {106194009 ...
https://phinvads.cdc.gov/vads/ViewCodeSystemConcept.action?oid=2.16.840.1.113883.6.96&code=32990003
TERM
Complement C3a Complement C3b Complement C3b Inactivator Proteins Complement C3c Complement C3d Complement C4 Complement C4a ... Complement C1 Complement C1 Inactivator Proteins Complement C1 Inhibitor Protein Complement C1q Complement C1r Complement C1s ... Complement C6 Complement C7 Complement C8 Complement C9 Complement Factor B Complement Factor D Complement Factor H Complement ... ADAMTS Proteins ADAMTS1 Protein ADAMTS13 Protein ADAMTS4 Protein ADAMTS5 Protein ADAMTS7 Protein ADAMTS9 Protein Adansonia ...
https://nlmpubs.nlm.nih.gov/projects/mesh/.newterms/mshd2017.txt
JCI - Volume 74, Issue 4JCI - Volume 74, Issue 4
There was relatively little binding of IC to leukocytes in both the complement-depleted and complement-repleted condition. Thus ... Binding of immune complexes (IC) to erythrocytes in vitro is the result of interaction between C3b sites on the IC, and ... Human multiple organ-reactive monoclonal autoantibody recognizes growth hormone and a 35,000-molecular weight protein.. ... do not appear to be due to an unstable Factor V molecule or to the presence of a Factor V or Factor Va inhibitor or inactivator ...
https://www.jci.org/74/4
ConvertaseSerumAntibodiesMembrane boundExogenousCleavageReceptorFactorLectinPromoterMoleculeUrineActivationComponentAntibodyImmuneSystem
Convertase4
  • C2a in the convertase complex cleaves C3 releasing C3a and C3b. (abcam.com)
  • It binds as a cofactor to COMPLEMENT FACTOR I which then hydrolyzes the COMPLEMENT C4B in the CLASSICAL PATHWAY C3 CONVERTASE (C4bC2a). (bvsalud.org)
  • C3 (MW 180 000), the central component of all complement reac- tions, split by its convertase into a small (C3a) and a large (C3b) fragment. (pediagenosis.com)
  • 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). (bvsalud.org)
Serum3
  • A serum protein that regulates the CLASSICAL COMPLEMENT ACTIVATION PATHWAY . (bvsalud.org)
  • 2) Similarly, normal antibody responses are obtained if the cultures are grown in human serum depleted of C3b-inactivator, which contains high concentrations of C3b. (ox.ac.uk)
  • We previously constructed an in vitro model comprising cancer cell lines expressing α-gal and serum containing natural antibodies against α-gal and complement. (bvsalud.org)
Antibodies1
  • the classical pathway initiated by antibodies bound to the surface of foreign bodies and the alternative and lectin pathways that provide an antibody-independent mechanism for complement activation, induced by the presence of bacteria and other micro-organisms. (abcam.com)
Membrane bound3
  • Two 'C3b inactivator' enzymes rapidly inactivate C3b, releasing the fragment C3c and leaving membrane bound C3d. (pediagenosis.com)
  • The overexpression of membrane-bound complement regulatory proteins (mCRPs) on tumour cells helps them survive complement attacks by suppressing antibody-mediated complement-dependent cytotoxicity (CDC). (bvsalud.org)
  • Normal primary human alveolar type II epithelial cells (AECs) exposed to exogenous complement 3a or 5a, and primary AECs purified from IPF lungs demonstrated decreased membrane-bound DAF expression with concurrent increase in the endoplasmic reticulum (ER) stress protein, ATF6. (bvsalud.org)
Exogenous1
  • This shows that no exogenous source of complement is necessary for in vitro antibody formation by spleen cells. (ox.ac.uk)
Cleavage1
  • Following these cleavage events, complement pathway activation continues as in the classical pathway. (abcam.com)
Receptor1
Factor13
  • The CFI gene provides instructions for making a protein called complement factor I. This protein helps regulate a part of the body's immune response known as the complement system. (medlineplus.gov)
  • Complement factor I and several related proteins protect healthy cells by preventing activation of the complement system when it is not needed. (medlineplus.gov)
  • At least 10 mutations in the CFI gene have been identified in people with complement factor I deficiency, a disorder characterized by immune system dysfunction. (medlineplus.gov)
  • The mutations result in abnormal, nonfunctional, or absent complement factor I. (medlineplus.gov)
  • The lack (deficiency) of functional complement factor I protein allows uncontrolled activation of the complement system. (medlineplus.gov)
  • This condition, which may also occur in people with complement factor I deficiency, is characterized by kidney malfunction that can be serious or life-threatening. (medlineplus.gov)
  • The CFI gene mutations identified in this disorder result in an abnormal or nonfunctional version of complement factor I. The defective protein allows uncontrolled activation of the complement system. (medlineplus.gov)
  • Variation near complement factor I is associated with risk of advanced AMD. (medlineplus.gov)
  • Characterization of mutations in complement factor I (CFI) associated with hemolytic uremic syndrome. (medlineplus.gov)
  • Ponce-Castro IM, Gonzalez-Rubio C, Delgado-Cervino EM, Abarrategui-Garrido C, Fontan G, Sanchez-Corral P, Lopez-Trascasa M. Molecular characterization of Complement Factor I deficiency in two Spanish families. (medlineplus.gov)
  • Se une al COMPLEMENTO C3B y hace que iC3b (complemento 3b inactivado) sea susceptible al desdoblamiento por el FACTOR I DEL COMPLEMENTO. (bvsalud.org)
  • El factor H del complemento inhibe también la asociación de C3b con el FACTOR B DEL COMPLEMENTO para formar la proenzima C3bB, y promueve la disociación de Bb del complejo C3bBb (CONVERTASA C3 DE LA VÍA ALTERNATIVA DEL COMPLEMENTO). (bvsalud.org)
  • CD55 or decay accelerating factor (DAF), a ubiquitously expressed glycosylphosphatidylinositol (GPI)-anchored protein, confers a protective threshold against complement dysregulation which is linked to the pathogenesis of idiopathic pulmonary fibrosis (IPF). (bvsalud.org)
Lectin1
  • Mannan-binding lectin (MBL) and MBL-associated serine proteases (MASPs) are involved in the initial step of the lectin pathway of complement activation. (abcam.com)
Promoter1
  • CD55/CD59-neutralizing antibody treatment or mutation of the LINC00973 promoter activates the complement and CD8+ T cells, inhibiting tumor growth. (bvsalud.org)
Molecule1
  • C1 is the first molecule in the classical complement cascade and comprises C1q and two molecules of C1r and C1s respectively. (abcam.com)
Urine1
  • The overactive complement system attacks certain kidney cells, which damages the kidneys and leads to a loss of protein in the urine (proteinuria). (medlineplus.gov)
Activation10
  • Learn about the three pathways lead to complement activation and some of their key inhibitors. (abcam.com)
  • Activation of complement can be started either via adaptive or innate immune recognition. (pediagenosis.com)
  • a single activation event (whether by antibody or via innate pathways) leads to the production of many downstream events, such as deposition of C3b. (pediagenosis.com)
  • Activation is usually limited to the immediate vicinity by the very short life of the active products, and in some cases there are special inactivators (represented here by scissors). (pediagenosis.com)
  • Nevertheless, excessive complement activation can cause unpleasant side-effects (see Fig. 36). (pediagenosis.com)
  • The in vitro antibody response of mouse spleen cells to TNP coupled to both T-dependent and T-independent carriers as well as to sheep erythrocytes has been studied to investigate the possible role of complement activation in the induction of antibody formation. (ox.ac.uk)
  • An important soluble regulator of the alternative pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). (bvsalud.org)
  • The complement system is a critical immune component, yet its role in tumor immune evasion and CD8+ T cell activation is not clearly defined. (bvsalud.org)
  • The consequently upregulated CD55/CD59 expression suppresses the complement system and cytokine secretion required for CD8+ T cell activation. (bvsalud.org)
  • These findings underscore the critical role of EGFR/Wnt/ß-catenin-upregulated CD55/CD59 expression in inhibiting the complement and CD8+ T cell activation for tumor immune evasion and immune checkpoint blockade resistance and identify a potential combination therapy to overcome these effects. (bvsalud.org)
Component1
  • The complement system is a heat-labile component of blood that confers bactericidal properties. (abcam.com)
Antibody1
  • Note that, in the absence of antibody, many of the molecules that activate the complement system are carbohydrate or lipid in nature (e.g. lipopolysaccharides, mannose), suggesting that the system evolved mainly to recognize bacterial surfaces via their non-protein features. (pediagenosis.com)
Immune2
  • The functions of complement include the attraction of inflammatory cells, opsonization to promote phagocytosis, immune complex clearance and direct microbial killing through the formation of the membrane attack complex (MAC). (abcam.com)
  • The unregulated activity of the complement system decreases blood levels of another complement protein called C3, reducing the immune system's ability to fight infections. (medlineplus.gov)
System1
  • The complement system is a group of proteins that work together to destroy foreign invaders (such as bacteria and viruses), trigger inflammation, and remove debris from cells and tissues. (medlineplus.gov)