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.
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).
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).
A serum protein which is important in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY. This enzyme cleaves the COMPLEMENT C3B-bound COMPLEMENT FACTOR B to form C3bBb which is ALTERNATIVE PATHWAY C3 CONVERTASE.
The 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.
A serine protease that is the complex of COMPLEMENT C3B and COMPLEMENT FACTOR BB. It cleaves multiple COMPLEMENT C3 into COMPLEMENT C3A (anaphylatoxin) and COMPLEMENT C3B in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY.
A 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 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.
The sequential activation of serum COMPLEMENT PROTEINS to create the COMPLEMENT MEMBRANE ATTACK COMPLEX. Factors initiating complement activation include ANTIGEN-ANTIBODY COMPLEXES, microbial ANTIGENS, or cell surface POLYSACCHARIDES.
A 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.
Degenerative changes in the RETINA usually of older adults which results in a loss of vision in the center of the visual field (the MACULA LUTEA) because of damage to the retina. It occurs in dry and wet forms.
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.
Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY).
A 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.
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.
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.
Physiologically inactive substances that can be converted to active enzymes.
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.
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.
Molecules on the surface of some B-lymphocytes and macrophages, that recognize and combine with the C3b, C3d, C1q, and C4b components of complement.
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 activation initiated by the binding of COMPLEMENT C1 to ANTIGEN-ANTIBODY COMPLEXES at the COMPLEMENT C1Q subunit. This leads to the sequential activation of COMPLEMENT C1R and COMPLEMENT C1S subunits. Activated C1s cleaves COMPLEMENT C4 and COMPLEMENT C2 forming the membrane-bound classical C3 CONVERTASE (C4B2A) and the subsequent C5 CONVERTASE (C4B2A3B) leading to cleavage of COMPLEMENT C5 and the assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.
A 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.
Serum proteins that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host. The complement system is tightly regulated by inactivators that accelerate the decay of intermediates and certain cell surface receptors.
A 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.
The smaller fragment generated from the cleavage of complement C3 by C3 CONVERTASE. C3a, a 77-amino acid peptide, is a mediator of local inflammatory process. It induces smooth MUSCLE CONTRACTION, and HISTAMINE RELEASE from MAST CELLS and LEUKOCYTES. C3a is considered an anaphylatoxin along with COMPLEMENT C4A; COMPLEMENT C5A; and COMPLEMENT C5A, DES-ARGININE.
The 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.
Enzymes that activate one or more COMPLEMENT PROTEINS in the complement system leading to the formation of the COMPLEMENT MEMBRANE ATTACK COMPLEX, an important response in host defense. They are enzymes in the various COMPLEMENT ACTIVATION pathways.
A 53-kDa protein that is a positive regulator of the alternate pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It stabilizes the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb) and protects it from rapid inactivation, thus facilitating the cascade of COMPLEMENT ACTIVATION and the formation of MEMBRANE ATTACK COMPLEX. Individuals with mutation in the PFC gene exhibit properdin deficiency and have a high susceptibility to infections.
Partial proteins formed by partial hydrolysis of complete proteins or generated through PROTEIN ENGINEERING techniques.
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).
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.
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.
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.
Compounds that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host.
A syndrome that is associated with microvascular diseases of the KIDNEY, such as RENAL CORTICAL NECROSIS. It is characterized by hemolytic anemia (ANEMIA, HEMOLYTIC); THROMBOCYTOPENIA; and ACUTE RENAL FAILURE.
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.
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).
A transcriptional elongation factor complex that is comprised of a heterodimer of CYCLIN-DEPENDENT KINASE 9 and one of several CYCLINS including TYPE T CYCLINS and cyclin K. It functions by phosphorylating the carboxy-terminal domain of RNA POLYMERASE II.
A G-protein-coupled receptor that signals an increase in intracellular calcium in response to the potent ANAPHYLATOXIN peptide COMPLEMENT C5A.
A 105-kDa serum glycoprotein with significant homology to the other late complement components, C7-C9. It is a polypeptide chain cross-linked by 32 disulfide bonds. C6 is the next complement component to bind to the membrane-bound COMPLEMENT C5B in the assembly of MEMBRANE ATTACK COMPLEX. It is encoded by gene C6.
A 206-amino-acid fragment in the alpha chain (672-1663) of C3b. It is generated when C3b is inactivated (iC3b) and its alpha chain is cleaved by COMPLEMENT FACTOR I into C3c (749-954), and C3dg (955-1303) in the presence COMPLEMENT FACTOR H.
A 93-kDa serum glycoprotein encoded by C7 gene. It is a polypeptide chain with 28 disulfide bridges. In the formation of MEMBRANE ATTACK COMPLEX; C7 is the next component to bind the C5b-6 complex forming a trimolecular complex C5b-7 which is lipophilic, resembles an integral membrane protein, and serves as an anchor for the late complement components, C8 and C9.
Complement activation triggered by the interaction of microbial POLYSACCHARIDES with serum MANNOSE-BINDING LECTIN resulting in the activation of MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES. As in the classical pathway, MASPs cleave COMPLEMENT C4 and COMPLEMENT C2 to form C3 CONVERTASE (C4B2A) and the subsequent C5 CONVERTASE (C4B2A3B) leading to cleavage of COMPLEMENT C5 and assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.
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.
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.
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.
Databases containing information about PROTEINS such as AMINO ACID SEQUENCE; PROTEIN CONFORMATION; and other properties.
A loose confederation of computer communication networks around the world. The networks that make up the Internet are connected through several backbone networks. The Internet grew out of the US Government ARPAnet project and was designed to facilitate information exchange.
The portion of an interactive computer program that issues messages to and receives commands from a user.
Sequential operating programs and data which instruct the functioning of a digital computer.
Linear POLYPEPTIDES that are synthesized on RIBOSOMES and may be further modified, crosslinked, cleaved, or assembled into complex proteins with several subunits. The specific sequence of AMINO ACIDS determines the shape the polypeptide will take, during PROTEIN FOLDING, and the function of the protein.
A process that includes the determination of AMINO ACID SEQUENCE of a protein (or peptide, oligopeptide or peptide fragment) and the information analysis of the sequence.

Mutations of the type A domain of complement factor B that promote high-affinity C3b-binding. (1/379)

Factor B is a zymogen that carries the catalytic site of the complement alternative pathway convertases. During C3 convertase assembly, factor B associates with C3b and is cleaved at a single site by factor D. The Ba fragment is released, leaving the active complex, C3bBb. During the course of this process, the protease domain becomes activated. The type A domain of factor B, also part of Bb, is similar in structure to the type A domain of the complement receptor and integrin, CR3. Previously, mutations in the factor B type A domain were described that impair C3b-binding. This report describes "gain of function" mutations obtained by substituting factor B type A domain amino acids with homologous ones derived from the type A domain of CR3. Replacement of the betaA-alpha1 Mg2+ binding loop residue D254 with smaller amino acids, especially glycine, increased hemolytic activity and C3bBb stability. The removal of the oligosaccharide at position 260, near the Mg2+ binding cleft, when combined with the D254G substitution, resulted in increased affinity for C3b and iC3b, a C3b derivative. These findings offer strong evidence for the direct involvement of the type A domain in C3b binding, and are suggestive that steric effects of the D254 sidechain and the N260-linked oligosaccharide may contribute to the regulation of ligand binding.  (+info)

Complement activity in middle ear effusions. (2/379)

Evidence for complement utilization in middle ear fluids (MEF) from patients with otitis media with effusion was sought. It was found that cleavage products of C3, C4 and Factor B could be demonstrated immunochemically in MEF, and that native C3 was present in much lower concentrations than other proteins, relative to their serum concentrations. Haemolytic assays for C1-C5 showed that early complement components are inactivated in MEF. Potential mechanisms for complement utilization in MEF are discussed.  (+info)

Alterations in C3 activation and binding caused by phosphorylation by a casein kinase released from activated human platelets. (3/379)

A casein kinase released from activated human platelets phosphorylates a number of plasma proteins extracellularly, and that activation of platelets in systemic lupus erythematosus patients parallels an increase in the phosphate content of plasma proteins, including C3. The present study was undertaken to characterize this platelet protein kinase and to further elucidate the effect(s) on C3 function of phosphorylation by platelet casein kinase. The phosphate content of human plasma C3 was increased from 0.15 to 0.60 mol phosphate/mol of C3 after platelet activation in whole blood or platelet-rich plasma. The platelet casein kinase was distinct from other casein kinases in terms of its dependence on cations, inhibition by specific protein kinase inhibitors, and immunological reactivity. C3 that had been phosphorylated with platelet casein kinase was tested for its susceptibility to cleavage by trypsin or the classical and alternative pathway convertases and its binding to EAC and IgG. Phosphorylation did not affect the cleavage of C3 into C3a and C3b, but the binding of fragments from phosphorylated C3 to EAC14oxy2 cells and to IgG in purified systems and in serum was increased by 1.6-4.5 times over that of unphosphorylated C3. A covariation was seen between the enhanced binding of C3 fragments to IgG after phosphorylation and an increased ratio of glycerol/glycine binding, from 2.0 for unphosphorylated C3 to 4.9 for phosphorylated C3. The present study suggests that an overall effect of phosphorylation of C3 by platelet casein kinase is to enhance the opsonization of immune complexes.  (+info)

Pneumococcal surface protein A inhibits complement activation by Streptococcus pneumoniae. (4/379)

Pneumococcal surface protein A (PspA) is a surface-exposed protein virulence factor for Streptococcus pneumoniae. In this study, no significant depletion of serum complement was observed for the serum of mice infected with pneumococci that express PspA. In contrast, in mice infected with an isogenic strain of pneumococci lacking PspA, significant activation of serum complement was detected within 30 min after infection. Also, the PspA-deficient strain but not the PspA-expressing strain was cleared from the blood within 6 h. The contribution of PspA to pneumococcal virulence was further investigated by using mice deficient for C5, C3, or factor B. In mice deficient for C3 or factor B, PspA-negative pneumococci became fully virulent. In contrast, in C5-deficient mice as in wild-type mice, PspA-deficient pneumococci were avirulent. These in vivo data suggest that, in nonimmune mice infected with pneumococci, PspA interferes with complement-dependent host defense mechanisms mediated by factor B. Immunoblots of pneumococci opsonized in vitro suggested that more C3b was deposited on PspA-negative than on PspA-positive pneumococci. This was observed with and without anticapsular antibody. Furthermore, processing of the alpha chain of C3b was reduced in the presence of PspA. We propose that PspA exerts its virulence function by interfering with deposition of C3b onto pneumococci and/or by inhibiting formation of a fully functional alternative pathway C3 convertase. By blocking recruitment of the alternative pathway, PspA reduces the amount of C3b deposited onto pneumococci, thereby reducing the effectiveness of complement receptor-mediated pathways of clearance.  (+info)

Production and functional activity of a recombinant von Willebrand factor-A domain from human complement factor B. (5/379)

Factor B is a five-domain 90 kDa serine protease proenzyme which is part of the human serum complement system. It binds to other complement proteins C3b and properdin, and is activated by the protease factor D. The fourth domain of factor B is homologous to the type A domain of von Willebrand Factor (vWF-A). A full-length human factor B cDNA clone was used to amplify the region encoding the vWF-A domain (amino acids 229-444 of factor B). A fusion protein expression system was then used to generate it in high yield in Escherichia coli, where thrombin cleavage was used to separate the vWF-A domain from its fusion protein partner. A second vWF-A domain with improved stability and solubility was created using a Cys(267)-->Ser mutation and a four-residue C-terminal extension of the first vWF-A domain. The recombinant domains were investigated by analytical gel filtration, sucrose density centrifugation and analytical ultracentrifugation, in order to show that both domains were monomeric and possessed compact structures that were consistent with known vWF-A crystal structures. This expression system and its characterization permitted the first investigation of the function of the isolated vWF-A domain. It was able to inhibit substantially the binding of (125)I-labelled factor B to immobilized C3b. This demonstrated both the presence of a C3b binding site in this portion of factor B and a ligand-binding property of the vWF-A domain. The site at which factor D cleaves factor B is close to the N-terminus of both recombinant vWF-A domains. Factor D was shown to cleave the vWF-A domain in the presence or absence of C3b, whereas the cleavage of intact factor B under the same conditions occurs only in the presence of C3b.  (+info)

Sodium butyrate blocks interferon-gamma (IFN-gamma)-induced biosynthesis of MHC class III gene products (complement C4 and factor B) in human fetal intestinal epithelial cells. (6/379)

Human intestinal epithelial cells have been established as local sites for complement biosynthesis. In this study, we investigated the effects of IFN-gamma and sodium butyrate on biosynthesis of MHC class III gene products (complement C4 and factor B) in the human fetal intestinal epithelial cell line INT-407. IFN-gamma induced a dose- and time-dependent increase in C4 and factor B secretion. However, sodium butyrate dose-dependently inhibited IFN-gamma-induced C4 and factor B secretion. These effects were also observed at the mRNA level. Immunoblotting indicated that IFN-gamma induced a rapid activation of Stat1alpha, and fluorescence immunohistochemistry detected a translocation of Stat1alpha into the nucleus within 1 h. However, the translocation of Stat1alpha was not affected by the addition of sodium butyrate. Nuclear run-on assay indicated that IFN-gamma induced a weak increase in the transcription rate of factor B gene, and sodium butyrate did not affect this response. IFN-gamma and sodium butyrate induced a counter-regulatory effect on C4 and factor B secretion: IFN-gamma acted as a potent inducer, but sodium butyrate potently abrogated these responses. These are mainly regulated through the post-transcriptional mechanism.  (+info)

Counter-regulatory effect of sodium butyrate on tumour necrosis factor-alpha (TNF-alpha)-induced complement C3 and factor B biosynthesis in human intestinal epithelial cells. (7/379)

The various biological activities of butyrate have been well documented. In this study, we tested the effects of butyrate on TNF-alpha-induced complement C3 and factor B biosynthesis in human intestinal epithelial cells. The biosynthesis of C3, factor B and IL-8 was evaluated at the protein and mRNA levels. To evaluate transcriptional activation, the nuclear run-on assay was performed. The transcription factor-DNA binding activity was assessed by an electrophoretic gel mobility shift assay (EMSA). In the intestinal epithelial cell lines HT-29, T84 and Caco-2, sodium butyrate enhanced TNF-alpha-induced C3 secretion, but suppressed TNF-alpha-induced factor B and IL-8 secretion. Nuclear run-on assay revealed that transcriptional regulatory mechanisms are involved in the effects of sodium butyrate. The EMSAs indicated that sodium butyrate suppressed TNF-alpha-induced nuclear factor (NF)-kappaB- and activation protein (AP)-1-DNA binding activity, but enhanced TNF-alpha-induced activation of CCAAT/enhancer-binding protein (C/EBP)beta (NF-IL-6)-DNA binding activity. Sodium butyrate induced a counter-regulatory effect on TNF-alpha-induced C3 and factor B biosynthesis in human intestinal epithelial cells. Butyrate action has been discussed with its activity to induce histone hyperacetylation, but its counter-regulatory effect on complement biosynthesis may be closely associated with the modulation of transcription factor activation.  (+info)

Mutational analysis of the primary substrate specificity pocket of complement factor B. Asp(226) is a major structural determinant for p(1)-Arg binding. (8/379)

Factor B is a serine protease, which despite its trypsin-like specificity has Asn instead of the typical Asp at the bottom of the S(1) pocket (position 189, chymotrypsinogen numbering). Asp residues are present at positions 187 and 226 and either one could conceivably provide the negative charge for binding the P(1)-Arg of the substrate. Determination of the crystal structure of the factor B serine protease domain has revealed that the side chain of Asp(226) is within the S(1) pocket, whereas Asp(187) is located outside the pocket. To investigate the possible role of these atypical structural features in substrate binding and catalysis, we constructed a panel of mutants of these residues. Replacement of Asp(187) caused moderate (50-60%) decrease in hemolytic activity, compared with wild type factor B, whereas replacement of Asn(189) resulted in more profound reductions (71-95%). Substitutions at these two positions did not significantly affect assembly of the alternative pathway C3 convertase. In contrast, elimination of the negative charge from Asp(226) completely abrogated hemolytic activity and also affected formation of the C3 convertase. Kinetic analyses of the hydrolysis of a P(1)-Arg containing thioester by selected mutants confirmed that residue Asp(226) is a primary structural determinant for P(1)-Arg binding and catalysis.  (+info)

AMD is strongly associated with the over-activation of complement pathways [16]. Supporting evidence comes from the detection of various complement proteins in the drusen of patients with AMD [9] and the close association between AMD and the alternative pathway. Furthermore, the association of genetic variants in complement genes, including the human factor B gene, is a risk factor for all forms of AMD [5,9,22,24]. In addition, it has been shown that polymorphisms of genes encoding the alternative pathway proteins CFH, factor B, and C3, as well as the classical pathway protein C2, are also associated with AMD [23,46]. Studies in animal models of choroidal neovascularization (CNV), a major pathologic association with wet AMD, also supports a role for complements in AMD. Furthermore, a targeted inhibitor specific for the AP of complement significantly reduces CNV and the physiologic consequences of CNV on retinal function [11,47]. Additional evidence for a role of the AP pathway in AMD exists. For ...
The IUPHAR/BPS Guide to Pharmacology. complement factor B - S1: Chymotrypsin. Detailed annotation on the structure, function, physiology, pharmacology and clinical relevance of drug targets.
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A growing body of evidence has recently accumulated about the pathogenic role of the complement system in AAV (1). In 2007, Xiao et al. (4) demonstrated how infusion of ANCA antibodies in wild-type mice could induce glomerular lesions typical of pauci-immune NCGN; interestingly, no lesion was observed when ANCA were injected in mice knocked out for C5 or complement factor B, whereas RPGN fully developed in C4-knockout mice; because C5 belongs to the terminal part of the complement cascade and complement factor B belongs to the cAP, while C4 is a key molecule of the classic pathway, these findings clearly showed that cAP plays a central role in experimental AAV (4). In keeping with this, Gou et al. showed that patients with active AAV have serologic signs of activation of the cAP (augmented serum levels of activated complement proteins, such as C3a, C5a, sC5b9, and Bb), which correlated with acute-phase reactants, number of crescents, and severity of kidney histology (9). The same group ...
Factor B antibody, Internal (complement factor B) for FACS, IHC-P, WB. Anti-Factor B pAb (GTX80605) is tested in Human samples. 100% Ab-Assurance.
Objective: Activation of the alternative pathway of the complement system, in which factor H (fH; CFH) is a key regulatory component, has been suggested as a link between obesity and metabolic disorders. To study the associations between circulating and adipose tissue gene expressions of CFH and complement factor B (fB; CFB) with obesity and insulin resistance.. Research Design and Methods: Circulating fH and fB were determined by ELISA in 398 subjects. CFH and CFB gene expressions were evaluated in 76 adipose tissue samples, in isolated adipocytes and stromo-vascular cells (SVC) (n=13). The effects of weight loss and rosiglitazone were investigated in independent cohorts.. Results: Both circulating fH and fB were positively associated with BMI, waist diameter, triglycerides and inflammatory parameters; and negatively with insulin sensitivity and HDL-cholesterol. For the first time, CFH gene expression was detected in human adipose tissue (significantly increased in subcutaneous compared with ...
Serum levels of complement components Clq, C4, C3, and Properdin factor B, from the classical and alternative pathways of complement activation, have been estimated in patients with ulcerative colitis and Crohns disease. C3, factor B, and to some extent C4 concentrations all increased when the disease was active. In remission the levels of these components did not differ from hospital control patients. There was no evidence for the preferential consumption of the proteins of either pathway of activation, even in those patients with evidence of circulating immune complexes.
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We have reported recently that C1qa−/− mice develop autoimmunity characterized by the production of ANA and immune complex-mediated GN associated with the presence of increased numbers of apoptotic bodies (4). IgG and C3 were present in the glomeruli of the diseased kidneys, which suggested that complement was being activated, most likely by the alternative pathway. This possibility led to the question of whether the development of glomerular injury in this model was dependent upon the activation of C3 in glomeruli by the alternative pathway. To test this hypothesis, we crossed mice deficient in C1q with mice deficient in factor B and C2.. Mice deficient in complement activation by disruption of the C2 and factor B genes did not develop spontaneous autoimmunity. When deficiency of C1q was added, renal damage and autoantibody production developed, suggesting a discrete role for the first component of the classical pathway, and possibly C4, in protection from autoimmunity. A striking feature ...
Looking for online definition of Complement factor b in the Medical Dictionary? Complement factor b explanation free. What is Complement factor b? Meaning of Complement factor b medical term. What does Complement factor b mean?
Looking for Alternative complement pathway? Find out information about Alternative complement pathway. 1. Education courses taken by a student to gain entry to a higher course or towards a final qualification 2. Biochem a chain of reactions associated with a... Explanation of Alternative complement pathway
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 ...
Italy - 7 June, 2021 - Due to the immunological pathogenesis of IgA nephropathy (IgAN), patients used to be given immunosuppressive therapy; however, this was shown to have no long-term benefit over optimal supportive therapy. Promising data from the interim analysis of a Phase II study with iptacopan [1] now show that the targeted inhibition of a specific factor of the immune system (complement factor B) allows a specific approach to therapy for IgAN without burdening patients with the severe side effects of immunosuppression.. IgA nephropathy (IgAN) is a chronic kidney disease occurring in young adults and is one of the most common reasons for kidney transplantation in this age group. IgAN is the most common form of glomerulonephritis (GN), i.e., immunologically induced inflammation of the renal glomeruli. It is characterized by glomerular deposition of immune complexes containing immunoglobulin A (IgA), and by a complex inflammatory response and progressive loss of kidney function. For many ...
SR GROUP - Exporter, Importer, Manufacturer, Distributor, Supplier, Trading Company of Rat CFH(Complement Factor H) ELISA Kit based in Delhi, India
The complement system is readily triggered by the presence of damage-associated molecular patterns on the surface of tumor cells. The complement alternative pathway provides rapid amplification of the molecular stress signal, leading to complement cascade activation to deal with pathogens or maligna …
Dr. Mohamed R. Daha, Department of Nephrology, University Hospital Leiden, Leiden University Medical Center, Building 1 C3-P, PO Box 9600, 2300 RC Leiden, South Holland, The Netherlands. Email: m.r.daha{at}lumc.nl ...
CFB - Cfb - Mouse, 4 unique 29mer shRNA constructs in retroviral untagged vector shRNA available for purchase from OriGene - Your Gene Company.
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Prior absorption of normal human serum (NHS) or C2-deficient human serum (C2D) with zymosan at 0 degrees C results in diminished consumption of C3 and factor B during subsequent incubation of the sera in Mg-EGTA buffer with zymosan at 37 degrees C for 30 min. An acid eluate from the zymosan restores the defect of absorbed NHS and C2D, and also enhances C3 and factor B utilization in hypogammaglobulinemic serum (H gamma S) in a dose-dependent fashion. The activity is specific in that the eluate from zymosan fails to enhance C3 and B depletion in H gamma S or absorbed NHS by lipopolysaccharide or Sepharose. The active component of th zymosan eluate emerges from both Sepharose 4B and Sephacryl S-200 in the region of molecules with m.w. of 150,000. Absorption with protein A-Sepharose removes the activity, demonstrating that it is IgG. Digestion of the IgG with pepsin fails to diminish activity, indicating that the Fc region is not required for activity; reduction to monovalent Fab fragments, ...
Human umbilical vein endothelial cells grown in vitro under standard conditions contain a high level of mRNA specific for the complement regulatory factors H and I. An additional 1.8-kb mRNA encoding a truncated form of factor H is also present. IFN-gamma stimulation of the cells causes a 6-7 fold increase in both factor H mRNA species, and a greater than 10-fold increase in factor I mRNA. IL-1 and LPS slightly suppressed factor H mRNA, while TNF had no effect. mRNA for factor B is also detectable in IFN-gamma-stimulated cells, but messengers for C1q, C4bp, and CR3 beta chain were not found. Secretion of factor H protein was also stimulated by IFN-gamma. The presence of mRNA for factors H, B, and I, together with C3 secretion, demonstrated by others, suggests that endothelial cells can assemble the complete alternative complement pathway. Endothelial cell complement may be involved in leukocyte-endothelium interactions mediated by leukocyte C3 receptors. ...
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 ...
A family of growth regulators (originally called cef10, connective tissue growth factor, fisp-12, cyr61, or, alternatively, beta IG-M1 and beta IG-M2), all belong to immediate-early genes expressed after induction by growth factors or certain oncogenes. Sequence analysis of this family revealed the presence of four distinct modules. Each module has homologues in other extracellular mosaic proteins such as Von Willebrand factor, slit, thrombospondins, fibrillar collagens, IGF-binding proteins and mucins. Classification and analysis of these modules suggests the location of binding regions and, by analogy to better characterised modules in other proteins, sheds some light onto the structure of this new family [(PUBMED:7687569)].. The vWF domain is found in various plasma proteins: complement factors B, C2, CR3 and CR4; the integrins (I-domains); collagen types VI, VII, XII and XIV; and other extracellular proteins [(PUBMED:8412987), (PUBMED:8145250), (PUBMED:1864378)]. Although the majority of ...
A family of growth regulators (originally called cef10, connective tissue growth factor, fisp-12, cyr61, or, alternatively, beta IG-M1 and beta IG-M2), all belong to immediate-early genes expressed after induction by growth factors or certain oncogenes. Sequence analysis of this family revealed the presence of four distinct modules. Each module has homologues in other extracellular mosaic proteins such as Von Willebrand factor, slit, thrombospondins, fibrillar collagens, IGF-binding proteins and mucins. Classification and analysis of these modules suggests the location of binding regions and, by analogy to better characterised modules in other proteins, sheds some light onto the structure of this new family [(PUBMED:7687569)].. The vWF domain is found in various plasma proteins: complement factors B, C2, CR3 and CR4; the integrins (I-domains); collagen types VI, VII, XII and XIV; and other extracellular proteins [(PUBMED:8412987), (PUBMED:8145250), (PUBMED:1864378)]. Although the majority of ...
Hearing Loss, Noise-Induced;NF-kappa B;Vestibulocochlear Nerve Diseases;Nerve Degeneration;Cochlear Nerve;Mice;Spiral Ganglion;Hair Cells, Auditory, Inner;Complement Factor B;Mice, Knockout;Transcription Factors;Hair Cells, Auditory; ...
TY - JOUR. T1 - Complement gene variants in relation to autoantibodies to beta cell specific antigens and type 1 diabetes in the TEDDY Study. AU - Törn, Carina. AU - Liu, Xiang. AU - Hagopian, William. AU - Lernmark, Åke. AU - Simell, Olli. AU - Rewers, Marian. AU - Ziegler, Anette G.. AU - Schatz, Desmond. AU - Akolkar, Beena. AU - Onengut-Gumuscu, Suna. AU - Chen, Wei Min. AU - Toppari, Jorma. AU - Mykkänen, Juha. AU - Ilonen, Jorma. AU - Rich, Stephen S.. AU - She, Jin-Xiong. AU - Sharma, Ashok Kumar. AU - Steck, Andrea. AU - Krischer, Jeffrey. PY - 2016/6/16. Y1 - 2016/6/16. N2 - A total of 15 SNPs within complement genes and present on the ImmunoChip were analyzed in The Environmental Determinants of Diabetes in the Young (TEDDY) study. A total of 5474 subjects were followed from three months of age until islet autoimmunity (IA: n = 413) and the subsequent onset of type 1 diabetes (n = 115) for a median of 73 months (IQR 54-91). Three SNPs within ITGAM were nominally associated (p , ...
I.K. Zarkadis; I. Havvas; A. Deli; D. Marioli; N. Pharmakakis, 2011: Association study of complement C2, C3 and factor B, and age-related macular degeneration in a Greek population
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There are two concepts behind the alternative pathway of complement: what occurs when a non-self cell is absent; and what occurs when a non-self cell is present. When a non-self cell is absent (meaning the tissue is healthy) then there is fluid-phase activation. Fluid-phase activation occurs continuously, spontaneously and very slowly. In fluid-phase activation, C3 spontaneously activates via hydrolysis to form C3H2O -- since it is unstable, C3H2O usually reverts to C3. However, if C3H2O encounters Factor B, then the two molecules bind to form a more stable C3H2OB molecule. Factor D then cleaves C3H2OB molecule to yield the enzyme C3H2OBb (aka fluid-phase C3 convertase). C3H2OBb has an active site on Bb; to culminate fluid-phase activation, this active site cleaves C3 into C3a and C3b. Fluid-phase activation is depicted in the figure to the left.. When a non-self cell is present, then a much faster process occurs. C3b binds to the surface of the non-self cell, then Factor B binds to the C3b. ...
Factor D兔多克隆抗体(ab111204)可与人样本反应并经WB实验严格验证。中国75%以上现货,所有产品均提供质保服务,可通过电话、电邮或微信获得本地专属技术支持。
Looking for online definition of subunit of positive elongation transcription factor b in the Medical Dictionary? subunit of positive elongation transcription factor b explanation free. What is subunit of positive elongation transcription factor b? Meaning of subunit of positive elongation transcription factor b medical term. What does subunit of positive elongation transcription factor b mean?
4AYI: Structure of a complex between CCPs 6 and 7 of Human Complement Factor H and Neisseria meningitidis FHbp Variant 3 Wild type
The optimal reaction conditions for hemolytic assay of alternative complement pathway activity in mouse serum were investigated. A microtiter system was used, in which a number of 7.5×106 rabbit erythrocytes per test well appeared to be optimal. Rabbit erythrocytes were superior as target cells over erythtocytes from a number of ... read more other animal species. The optimal conditions were as follows: an incubation temperature of 39°C, an ionic strength of about 200 mM, and a magnesium concentration of 2.5 mM. Incubation during 60 min was not sufficient for an end-point titration. Addition of 1 mg of zymosan A per test well, however, enhanced and accelerated the hemolytic activity of mouse serum via the alternative pathway resulting in a maximum value after 45 min. This, most probably, proceeded by a mechanism involving the formation of a zymosan-C5-convertase and bystander lysis of the target cells. In contrast to the normal alternative pathway assay the zymosan-potentiated test did, most ...
TY - JOUR. T1 - Coelomocytes express SpBf, a homologue of factor B, the second component in the sea urchin complement system. AU - Smith, L. Courtney. AU - Shih, Chi Schin. AU - Dachenhausen, Sebastian G.. PY - 1998/12/15. Y1 - 1998/12/15. N2 - A homologue of factor B, SpBf, has been cloned and sequenced from an LPS-activated coelomocyte cDNA library from the purple sea urchin, Strongylocentrotus purpuratus. The deduced amino acid sequence and domain structure show significant similarity to the vertebrate Bf/C2 family proteins. SpBf is a mosaic protein, composed of five short consensus repeats, a von Willebrand Factor domain, and a serine protease domain. It has a deduced molecular mass of 91 kDa, with a conserved cleavage site for a putative factor D protease. It has ten consensus recognition sites for N- linked glycosylation. Amino acids involved in both Mg2+ binding and in serine protease activity in the vertebrate C2/Bf proteins are conserved in SpBf. Phylogenetic analysis of SpBf indicates ...
does MHC class III genes encode Interleukin Beta microglobulinc Tumor necro MHC class III genes encodes proteins of classic and alternate complement pathways (C2 and C4, properdin factor B), soluble proteins, tumor necrosis factors (TNF alpha, beta), HSP 70 and the 21 hydroxy
Scaffold attachment factor B2兔多克隆抗体(ab104220)可与人样本反应并经WB, IHC, ICC/IF实验严格验证。所有产品均提供质保服务,中国75%以上现货。
Complementary DNA (cDNA) probes were used to investigate the extrahepatic production of the major histocompatibility complex (MHC)-linked complement components C4, factor B and C2 in various normal human tissues. The presence of the corresponding messenger RNA (mRNA) was tested by Northern blot analysis. Complement C4 mRNA was found in liver, and with high intensity also in normal kidneys. In contrast, no C2 mRNA and only very low amounts of factor B mRNA could be detected in the kidney. Slot blot hybridization was performed to quantitate the amount of C4 mRNA, and the intensity of C4 mRNA hybridization in the kidney samples was about 25% compared with liver RNA. C4-specific transcripts were not present in isolated glomeruli but in the renal interstitium. Other human tissues, such as tonsil, spleen, thymus, brain, lung and peripheral mononuclear cells, contained no C4 mRNA. Low amounts of C4 mRNA were found in colon, thyroid gland, lymph node and breast carcinoma. The results obtained with lung, where
From the hemocyte granules of the horseshoe crabs Limulus and Tachypleus. Factor B is activated by limulus clotting factor C. In peptidase family S1 (trypsin family)
Rabbit polyclonal Scaffold attachment factor B2 antibody validated for WB, IHC, ICC/IF and tested in Human. Immunogen corresponding to recombinant fragment
Mouse Monoclonal Anti-Scaffold attachment factor B2 Antibody (5A11). Validated: WB, ELISA, IHC-P, IF. Tested Reactivity: Human. 100% Guaranteed.
casSAR Dugability of Q2FUY2 | clfB | Clumping factor B - Also known as CLFB_STAA8, clfB. Cell surface-associated protein implicated in virulence by promoting bacterial attachment to both alpha- and beta-chains of human fibrinogen and inducing the formation of bacterial clumps. Partly responsible for mediating bacterial attachment to the highly keratinized squamous epithelial cells from the nasal cavity via an interaction with cytokeratin K10 (K10). Also promotes bacterial attachment to cultured keratinocytes, possibly through an interaction with cytokeratin K10. Binds mouse cytokeratin K10. Activates human platelet aggregation.
Background C3 plays a central role in the activation of the complement system. Its processing by C3 convertase is the central reaction in both classical and alternative complement pathways. After activation C3b can bind...
SEA635Mu, CF-H; FH; FHL1; ARMD4; ARMS1; CFHL3; HF1; HF2; HUS; H Factor 2; Age-Related Maculopathy Susceptibility 1; Adrenomedullin binding protein | Products for research use only!
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Protein delivery is an interesting technique that complements gene transfection and RNA interference for studying protein function and cellular pathways.
Space time is made up of an inactive energy field in a grid structure, it only becomes active when energy acts on it. A second component of space time are...
METHODS AND RESULTS: Screening of the glycoproteome of urine samples from ADPKD patients revealed that levels of complement factor B (CFB), serpin peptidase inhibitor, complement component 1 inhibitor (SERPING1) and complement component 9 (C9) increased, whereas complement component 1, r subcomponent-like (C1RL), CD55 and CD59 levels decreased with disease progression. Immunostaining and Western blot analysis confirmed the enhanced expression of CFB and C9 in cystic kidneys from ADPKD patients. Immunostaining also showed that the expressions of CFB and C9 in renal biopsy tissues from patients with other types of chronic kidney disease were lower than in tissues from ADPKD patients. The effect of the complement inhibitor rosmarinic acid (RMA) was evaluated in Pkd1(-/-) mice and Han:SPRD Cy/+ rats. Compared with vehicle-treated Pkd1(-/-) animals, RMA-treated mice had significantly lower serum creatinine (-50%) and blood urea nitrogen (-78%) levels, two kidneys/body weight ratio (-60%) and renal ...
Human complement factor H Y402H polymorphism causes an age-related macular degeneration phenotype and lipoprotein dysregulation in mice.
Properdin factor d definition at Dictionary.com, a free online dictionary with pronunciation, synonyms and translation. Look it up now!
Purpose: To identify differences in the protein composition of the aqueous humor (AH) of patients with diabetic retinopathy (DR) compared to patients without history of diabetes undergoing cataract surgery (control).. Methods: AH samples (n=6 for control and n=7 for DR) were analyzed by Liquid Chromatography/Tandem Mass Spectrometry (LC-MS/MS) and relative protein abundances were determined by spectral counting. G test followed by post hoc Holm Sidak was used for statistical analyses to determine significance in the differential expression of proteins between DR and control groups. Proteins were classified into functional groups using Scaffold 3.0.. Results: Using stringent filtering criteria, seven proteins were significantly altered in the DR AH (p,0.05). SERPINF1 (PEDF), complement factor B (CFB), lumican and complement C9 (C9) were detected at higher levels (1.5,1.6, 2 and 3 fold respectively), whereas serotransferrin (TF), SERPING1 and prostaglandin-H2 D-isomerase (PTGDS) were detected at ...
TY - JOUR. T1 - Risk of non-Hodgkin lymphoma in association with germline variation in complement genes. AU - Cerhan, James R. AU - Novak, Anne J. AU - Fredericksen, Zachary S.. AU - Wang, Alice H.. AU - Liebow, Mark. AU - Call, Timothy G.. AU - Dogan, Ahmet. AU - Witzig, Thomas Elmer. AU - Ansell, Stephen Maxted. AU - Habermann, Thomas Matthew. AU - Kay, Neil Elliot. AU - Slager, Susan L. PY - 2009/6. Y1 - 2009/6. N2 - Germline mutations in complement genes have been associated with susceptibility to infections and autoimmune diseases, conditions that are associated with non-Hodgkin lymphoma (NHL) risk. To test the hypothesis that common genetic variation in complement genes affect risk of NHL, we genotyped 167 single nucleotide polymorphisms (SNPs) from 31 genes in 441 NHL cases and 475 controls. Principal components (PC) and haplotype analyses were used for gene-level tests of NHL risk, while individual SNPs were modelled as having a log-additive effect. In gene level PC analyses, C2 (P = ...
Objectives: Fatigue is a frequent and often disabling phenomenon that occurs in patients with chronic inflammatory and immunological diseases, and the underlying biological mechanisms are largely unknown. Because fatigue is generated in the brain, we aimed to investigate cerebrospinal fluid and search for molecules that participate in the pathophysiology of fatigue processes. Methods: A label-free shotgun proteomics approach was applied to analyze the cerebrospinal fluid proteome of 20 patients with primary Sjögrens syndrome. Fatigue was measured with the fatigue visual analog scale. Results: A total of 828 proteins were identified and the 15 top discriminatory proteins between patients with high and low fatigue were selected. Among these were apolipoprotein A4, hemopexin, pigment epithelium-derived factor, secretogranin-1, secretogranin-3, selenium-binding protein 1, and complement factor B. Conclusion: Most of the discriminatory proteins have important roles in regulation of innate immunity, ...
C1-INH is a heavily glycosylated, single chain, plasma glycoprotein with an apparent molecular weight of 105 kd on sodium dodecyl sulfate-PAGE. It consists of 478 amino acids comprising a backbone molecular weight of 52,880 (1). The protein acts as a serine protease inhibitor (serpin) binding to and forming covalent bonds with a variety of plasma proteases and thus inhibiting their activity (2, 3). The protein is known to inhibit C1s and C1r, two subcomponents of the complement protein C1. It is for these properties that it received its name. However, it is a known inhibitor of factor FXIIa and FXIIf, kallikrein, FXIa, plasmin, MASP1, and MASP2. Thus it inhibits proteins of the intrinsic coagulation, kinin generating, and fibrinolytic pathways, as well as the mannan binding lectin pathway of complement activation (5-9). As such, it is a potent down regulator of inflammation. C1-INH has been administered to animals in a variety of animal models of disease and shown to have profound inhibitory ...
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 ...
I conduct research into genetic kidney disease and perform a weekly nephrology clinic specializing in the care of patients and families with hereditary kidney problems, including polycystic kidney disease, unexplained familial kidney failure, inherited microscopic haematuria syndromes and renal cancer syndromes.. Using linkage mapping, next generation sequencing and other techniques I have described and identified the molecular defects responsible for the genetic diseases HIF2α erythrocytosis, which results from a defect in cellular oxygen sensing, and CFHR5 nephropathy, which results from a defect of complement alternative pathway regulation and which is endemic in people of Cypriot ancestry.. Ongoing projects aim firstly to improve understanding of the pathophysiology of these diseases; secondly to develop rational approaches to their treatment; and thirdly to investigate other families with inherited kidney disease in order to uncover the genetic change responsible in each one. ...
1HFD: Structures of native and complexed complement factor D: implications of the atypical His57 conformation and self-inhibitory loop in the regulation of specific serine protease activity.
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A new cluster of complement component genes, including C4BP, C3bR, and FH, is described. Family segregation data indicate that FH is linked to the genes for C4-bp and C4bR, previously reported to be linked and to maintain linkage disequilibrium. This cluster is not linked to the major histocompatibility complex, which contains the genes for the complement components, C4, C2, and factor B, or to the C3 locus. These data further suggest that the organization of genes for functionally related proteins in clusters may be a rule for the complement system. ...
In the case-control dataset of all AMD individuals we used the haplo.glm module of haplo.stats to build a regression model to study haplotype-smoking interactions. 40 According to the recommendation of Lake et al., 40 we modeled only haplotypes with a frequency greater than 5%. Four haplotypes in our dataset met this criterion, and one was selected as the baseline haplotype. Though the selection of the baseline neutral haplotype is somewhat arbitrary, we chose the TGGTTTGT haplotype (Fig. 1)because it was the most frequent haplotype occurring in the same percentage of cases and controls (13.5% in controls and 13.8% in cases), and its association score statistic comparing all AMD individuals (grades 3, 4, and 5) to all controls (grades 1 and 2) indicated neither a protective nor a risk effect (haplotype score = −0.997, P = 0.311). Smoking status was obtained from patient questionnaires and dichotomized by classifying individuals who had smoked ,100 cigarettes in their lifetime as smokers. The ...
Complement, C3 Convertase, Regulation, Cells, Disease, Complement Factor H, Inhibition, Therapeutic, Transplant, Allograft, Donor, Donors, and Graft
C3b is the larger of two elements formed by the cleavage of complement component 3, and is considered an important part of the innate immune system. C3b is potent in opsonization: tagging pathogens, immune complexes (antigen-antibody), and apoptotic cells for phagocytosis. Additionally, C3b plays a role in forming a C3 convertase when bound to Factor B (C3bBb complex), or a C5 convertase when bound to C4b and C2b (C4b2b3b complex) or when an additional C3b molecule binds to the C3bBb complex (C3bBb3b complex). C3bs ability to perform these important functions derives from its ability to covalently bind to the surface of invading pathogens within an organisms body. The cleavage of C3 leaves C3b with an exposed thioester bond, allowing C3b to effectively coat and tag foreign cells by covalently binding to hydroxyl (-OH) and amine (-NH2) groups on foreign cell surfaces. This cleavage can occur via three mechanisms (classical pathway, alternative pathway and lectin pathway) that ultimately lead to ...
The gene encoding for the MHC class I H-2 molecule can be activated by TNF-α and IFN-γ (19). Although NF-κB sites have been found within transcriptional control regions of this gene (20, 21), the role of NF-κB proteins in its activation is not known. Furthermore, transcription factors that do not belong to the NF-κB family have also been shown to bind specifically to the MHC κB site (22). Therefore, we wished to determine whether RelA participates in activation of this gene. To this end, primary MEFs derived from RelA+/− or RelA−/− mice were first treated with TNF-α or LPS. We have previously shown that LPS is a potent inducer of NF-κB in MEFs, although the effect of such treatment on gene induction was not determined (23). Treatments were carried out for 6 h, since this time period is sufficient for maximal activation of the genes we have studied and does not result in significant death of RelA−/− MEFs by TNF-α. Northern blot analysis of RNA obtained from TNF-α- or ...
1: AMD and complement factor H (CFH). Common sequence variants of CFH have major roles in determining susceptibility to age-related macular degeneration (AMD)....
Hypothetical LOC387715 is a second major susceptibility gene for age-related macular degeneration, contributing independently of complement factor H to disease risk. ...
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... factor H, factor HR1 or HR3, membrane cofactor protein, factor I, factor B, complement C3, and thrombomodulin). This results in ... The complement system activation may be due to mutations in the complement regulatory proteins (factor H, factor I, or membrane ... of complement can result from production of anti-factor H autoantibodies or from genetic mutations in any of several complement ... "Platelet-associated complement factor H in healthy persons and patients with atypical HUS". Blood. 114 (20): 4538-4545. doi: ...
Some HLA alleles have been suspected along with complement phenotypes as being genetic factors. Non-aggressive Berger's disease ... Other blood tests done to aid in the diagnosis include CRP or ESR, complement levels, ANA, and LDH. Protein electrophoresis and ... Hence the decision on which patients to treat should be based on the prognostic factors and the risk of progression. Also, IgA ... In cases where tonsillitis is the precipitating factor for episodic hematuria, a tonsillectomy has been claimed to reduce the ...
Porins are also recognized by TLR2, they bind complement factors (C3b, C4b, factor H, and C4bp (complement factor 4b-binding ... Complement inhibition[edit]. Factor H binding protein (fHbp) that is exhibited in N. meningitidis and some commensal species is ... Close contact with a carrier is the predominant risk factor. Other risk factors include a weakened general or local immune ... Porins are also an important factor for complement inhibition for both pathogenic and commensal species. Porins are important ...
"Onchocerca volvulus microfilariae avoid complement attack by direct binding of factor H". The Journal of Infectious Diseases. ... The complement system is used to enhance the effect of antibodies and phagocytic cells, which engulf and destroy other cells. ...
"Evid Based Complement Alternat Med. 7 (1): 11-28. doi:10.1093/ecam/nen023. PMC 2816378. PMID 18955327.. ... Multiple factors such as gender, age, ethnicity, education and social class are also shown to have association with prevalence ... "Who Uses CAM? A Narrative Review of Demographic Characteristics and Health Factors Associated with CAM Use" ...
In this analysis, it is usually a reduced complement factor C4, rather than the C1-INH deficiency itself, that is detected. The ... especially depletion of complement factors 2 and 4, may indicate deficiency of C1-inhibitor. HAE type III is a diagnosis of ... which encodes the coagulation protein factor XII. All forms of HAE lead to abnormal activation of the complement system, and ... In hereditary angioedema, bradykinin formation is caused by continuous activation of the complement system due to a deficiency ...
Such proteins are referred to as virokines if they resemble cytokines, growth factors, or complement regulators; the term ... The first identified virokine was an epidermal growth factor-like protein found in myxoma viruses. Much of the early work on ... Kotwal, GJ; Moss, B (8 September 1988). "Vaccinia virus encodes a secretory polypeptide structurally related to complement ... which was discovered to secrete proteins that promote proliferation of neighboring cells and block complement immune activity ...
Able to break down fibrin clots, cleave complement protein C3, and activate Factor XII. ... Faktor XII (Hageman Factor) liver A protein that circulates inactively, until activated by collagen, platelets, or exposed ... The combination and activation of this range of complement proteins forms themembrane attack complex, which is able to insert ... A complex of the complement proteins C5b, C6, C7, C8, and multiple units of C9. ...
Overall this protein shows similarity to the complement 1Q factors (C1Q). However, when the 3-dimensional structure of the ... "The crystal structure of a complement-1q family protein suggests an evolutionary link to tumor necrosis factor". Current ... response to tumor necrosis factor. • positive regulation of blood pressure. • response to nutrient levels. • positive ... negative regulation of tumor necrosis factor production. • protein localization to plasma membrane. • negative regulation of ...
2003) Complement factor H mutations and gene polymorphisms in haemolytic uraemic syndrome: the C-257T, the A2089G and the ... 2008) Factor H dysfunction in patients with atypical hemolytic uremic syndrome contributes to complement deposition on ... 2005) Complement factor H mutation in familial thrombotic thrombocytopenic purpura with ADAMTS13 deficiency and renal ... a b c Meri, S. (2007) Loss of self-control in the complement system and innate autoreactivity. From Ann N Y Acad Sci 1109, 93- ...
Decay accelerating factor (DAF/CD55), a regulatory protein of the complement cascade, interacts with the first and second EGF- ... Several treatments reduce CD97 expression in tumor cells such as cytokine tumor growth factor (TGF)β as well as the compounds ... "The epidermal growth factor-seven transmembrane (EGF-TM7) receptor CD97 is required for neutrophil migration and host defense ... In the case of CD97 the N-terminal domains consist of alternatively spliced epidermal growth factor (EGF)-like domains. ...
Maller JB, Fagerness JA, Reynolds RC, Neale BM, Daly MJ, Seddon JM (2007). "Variation in Complement Factor 3 is Associated with ... Ipak, novi lijekovi, nazvani anti-angiogeni ili anti-VEGF (engl. anti-Vascular Endotheilal Growth Factor) agensi, mogu ... "Risk factors associated with age-related macular degeneration. A case-control study in the age-related eye disease study: Age- ... "Risk factors for the incidence of Advanced Age-Related Macular Degeneration in the Age-Related Eye Disease Study (AREDS) AREDS ...
The C3 Nephritic Factor autoantibody stabilizes C3-convertase, which may lead to an excessive activation of complement.[4]:553 ... Type 2 MPGN, also known as Dense Deposit Disease, is characterised by an excessive activation of the complement system. ... Circulating immune complexes may activate the complement system, leading to inflammation and an influx of inflammatory cells.[4 ...
... complement proteins, and regulatory factors such as interleukin-1. At the same time, they carry receptors for lymphokines that ... tumor necrosis factor-alpha (TNF-alpha), Macrophage colony-stimulating factor (M-CSF/CSF1) and IL-1 and IL-6[57] contributing ... Macrophages also secrete a number of factors such as growth factors and other cytokines, especially during the third and fourth ... macrophages serve as a source for many pro-angiogenic factors including vascular endothelial factor (VEGF), ...
Several virulence factors have been identified, but predominantly by screening S.dysgalactiae isolates for homologues of well- ... The M-protein aids in immune evasion by inhibiting phagocytosis and inactivating the complement system. Furthermore, ... The emm-gene encodes the M-protein, a major virulence factor in both S.pyogenes and Streptococcus dysgalactiae. It is ... The M-protein, the most extensively studied SDSE virulence factor, has been documented to facilitate both adherence to and ...
Abrams, BM (2013). "Factors that cause concern". Med Clin N Am. 97 (2): 225-242. doi:10.1016/j.mcna.2012.11.002. PMID 23419623. ... J Altern Complement Med. 19 (4): 285-97. doi:10.1089/act.2013.19608. PMID 23075410.. ... Hao XA, Xue CC, Dong L, Zheng Z (April 2013). "Factors associated with conflicting findings on acupuncture for tension-type ... Another study found the following factors independently each increase the chance of migraine over tension type headache: nausea ...
... significant success factors were identified, complementing the earlier "just-in-time", lean manufacturing, and agile ... Khairul Anuar Rusli, Azmawani Abd Rahman and Ho, J.A. Green Supply Chain Management in Developing Countries: A Study of Factors ... Supply-chain event management (SCEM) considers all possible events and factors that can disrupt a supply chain. With SCEM, ... As logistics competency becomes a critical factor in creating and maintaining competitive advantage, measuring logistics ...
PSAP Complement component 4, partial deficiency of; 120790; C1NH Complement factor H deficiency; 609814; HF1 Complement factor ... GLA Factor V and factor VIII, combined deficiency of; 227300; MCFD2 Factor V deficiency; 227400; F5 Factor XI deficiency, ... F11 Factor XII deficiency; 234000; F12 Factor XIIIA deficiency; 613225; F13A1 Factor XIIIB deficiency; 613235; F13B Failure of ... LCAT Fletcher factor deficiency; 612423; KLKB1 Focal cortical dysplasia, Taylor balloon cell type; 607341; TSC1 Focal dermal ...
... activates the alternative complement pathway and also interferes with complement regulation by binding to complement factor H, ... 2008). "Factor H dysfunction in patients with atypical hemolytic uremic syndrome contributes to complement deposition on ... a first-in-class terminal complement inhibitor, has been shown in clinical studies to block terminal complement activity in ... an inhibitor of the complement cascade. Shiga-toxin causes complement-mediated platelet, leukocyte, and endothelial cell ...
Bersch B, Hernandez JF, Marion D, Arlaud GJ (February 1998). "Solution structure of the epidermal growth factor (EGF)-like ... Complement component 1s (EC, C1 esterase, activated complement C1s, complement C overbar 1r, C1s) is a protein ... complement activation, lectin pathway. • complement activation. • regulation of complement activation. Sources:Amigo / QuickGO ... complement activation, classical pathway. • immune system process. • innate immune response. • ...
It is not another methodology to deal with risk management, but complements existing ones. It is in direct competition with the ... Factor analysis of information risk (FAIR) is a taxonomy of the factors that contribute to risk and how they affect each other ... These components have characteristics (factors) that, in combination with one another, drive risk. Risk Factoring begins to ... This probabilistic approach is applied to every factor that is analysed. The risk is the probability of a loss tied to an asset ...
Media related to Complement membrane attack complex at Wikimedia Commons. *Complement+Membrane+Attack+Complex at the US ... The membrane attack complex (MAC) or terminal complement complex (TCC) is a structure typically formed on the surface of ... Reid K. B. M., The complement system, in: B. D. Hames and D. M. Glover (eds.), Molecular Immunology, Oxford: IRL Press, 1988, ... MAC is composed of a complex of four complement proteins (C5b, C6, C7, and C8) that bind to the outer surface of the plasma ...
Like complement factor H, CFHR5 is able to bind to complement C3. A mutation in CHFR5 was found in patients with the disease ... "Entrez Gene: CFHR5 complement factor H-related 5". McRae JL, Duthy TG, Griggs KM, et al. (2005). "Human factor H-related ... 2006). "Variations in the complement regulatory genes factor H (CFH) and factor H related 5 (CFHR5) are associated with ... Complement factor H-related protein 5 is a protein that in humans is encoded by the CFHR5 gene. CFHR5 is structurally related ...
A common haplotype in the complement regulatory gene factor H (HF1/CFH) predisposes individuals to age-related macular ... insulin-like growth factor-1 (IGF-1)[23], ciliary neurotrophic factor (CNTF)[24], pigment epithelium-derived factor (PEDF)[25] ... vascular endothelial growth factor (VEGF)[26], 등 각자 다양한 역할을 수행한다. . 면역조절 작용[편집]. 앞에서 말했듯이, 망막 안쪽 부분은 신체 내부의 면역 반응으로부터 분리되었으며, ... 망막색소상피세포는 다양한 신호물질을 분비해서 광수용세포 유지 및 면역 시스템 조절 역할을 수행한다.[19] 그 종류도 fibroblast growth factors[20],[21], transforming growth ...
"Cellular adhesion mediated by factor J, a complement inhibitor. Evidence for nucleolin involvement". The Journal of Biological ... Nucleolin is also able to act as a transcriptional coactivator with Chicken Ovalbumin Upstream Promoter Transcription Factor II ...
... is produced when complement factor I cleaves C3b. Complement receptors on white blood cells are able to bind iC3b, so iC3b ... thus preventing amplification of the complement cascade through the alternative pathway. Complement factor I can further cleave ... iC3b is a protein fragment that is part of the complement system, a component of the vertebrate immune system. ... Unlike intact C3b, iC3b cannot associate with factor B, ...
The recipient's complement cascade can be inhibited through the use of cobra venom factor (which depletes C3), soluble ... The binding of XNAs initiate complement activation through the classical complement pathway. Complement activation causes a ... Expression of human complement regulators (CD55, CD46, and CD59) to inhibit the complement cascade.[27] ... The safety of public health is a factor to be considered.[50] If there is any risk to the public at all for an outbreak from ...
The influence of sex chromosomes and environmental factors are also noteworthy. Usually, these factors contribute to lupus by ... When a single gene deficiency does cause lupus, it is usually attributed to the complement protein genes C1, C2, or C4. ... tumor necrosis factor alpha. Genetics[edit]. It is typically believed that lupus is influenced by multiple genes. Lupus is ... and Traditional Risk Factors". Scientific Reports. 6: 20303. Bibcode:2016NatSR...620303A. doi:10.1038/srep20303. PMC 4740809. ...
The Factor H-related protein 1 (FHR1) has been identified as a novel inhibitor of the complement pathway. FHR1 blocks C5 ... Apparently Factor H and FHR1 control complement activation in a sequential manner. In hemolytic uremic syndrome (HUS), the ... 2009). "Factor H-related protein 1 (FHR-1) inhibits complement C5 convertase activity and terminal complex formation". Blood. ... Factor B then binds to C3b and is cleaved by a plasma serine protease Factor D. The C3bBb complex (= alternative pathway C3 ...
This is a type of safeguard to the system, almost like a two-factor authentication method. First, the B cells have to encounter ... Differentiation of mature B cells into plasma cells is dependent upon the transcription factors Blimp-1/PRDM1 and IRF4. ... Immunology: lymphocytic adaptive immune system and complement. Lymphoid. Antigens. *Antigen *Superantigen. *Allergen ...
Neonatal and adult recent thymic emigrants produce IL-8 and express complement receptors CR1 and CR2. JCI insight. 2017-08-17, ... Cell-intrinsic transforming growth factor-beta signaling mediates virus-specific CD8+ T cell deletion and viral persistence in ...
The size of an animal is also a factor in determining diet type (Allen's rule). Since small mammals have a high ratio of heat- ... and herbivores when they are just obtaining nutrients from materials originating from sources that do not seemingly complement ...
"Evid Based Complement Alternat Med. 2012: 1-19. doi:10.1155/2012/837939. PMC 3443820. PMID 22991573.. ... Other factors. There are also reasons why a placebo treatment group may outperform a "no-treatment" group in a test which are ... "J Altern Complement Med. 12 (3): 323-28. doi:10.1089/acm.2006.12.323. PMID 16646733.. ... Social factors. Authors have speculated on the socio-cultural and psychological reasons for the appeal of alternative medicines ...
... between the then-Australian Prime Minister Tony Abbott and Japanese Prime Minister Shinzō Abe had also been cited as a factor ... Complement:. 60[3]. Sensors and processing systems:. AN/BYG-1 combat system[2]. ...
Indeed, automation threatens repetitive jobs but higher-end jobs are still necessary because they complement technology and ... Superpower § Possible factors. Theories and concepts in technology. *Appropriate technology. *Diffusion of innovations ...
... a k-factor is a factor that is k-regular. In particular, a 1-factor is the same thing as a perfect matching. A factor-critical ... complement. The complement graph G. ¯. {\displaystyle {\bar {G}}}. of a simple graph G is another graph on the same vertex set ... factor. A factor of a graph is a spanning subgraph: a subgraph that includes all of the vertices of the graph. The term is ... A graph factorization is a partition of the edges of the graph into factors; a k-factorization is a partition into k-factors. ...
Stated another way, the correlation is the difference between the common language effect size and its complement: r. =. f. −. ( ...
From 1 June 2014 the list will be complemented with restaurants and bars, dormitories, hotels, long-haul trains, and the ... It has been claimed that the smoke-free law was a significant contributing factor to the closure of hundreds of small rural ...
Urbanization and other anthropogenic factors can be implicated in the spread of Lyme disease to humans. In many areas, ... The resistance of a genospecies of Lyme disease spirochetes to the bacteriolytic activities of the alternative complement ... inactivating key immune components such as complement, and hiding in the extracellular matrix, which may interfere with the ... The authors argued that the factors influencing tick density and human risk between sites are still poorly understood, and that ...
"Evid Based Complement Alternat Med. 3 (4): 425-32. doi:10.1093/ecam/nel044. PMC 1697737 . PMID 17173105. Archived from the ... Gaumer G (2006). "Factors associated with patient satisfaction with chiropractic care: survey and review of the literature". J ... J Altern Complement Med. 14 (4): 361-68. doi:10.1089/acm.2007.0766. PMID 18435599.. ... J Altern Complement Med. 13 (5): 491-512. doi:10.1089/acm.2007.7088. PMID 17604553.. ...
"Non-dietary risk factors for gastric dilatation-volvulus in large and giant breed dogs" (PDF). Archived (PDF) from the original ... the trade in dyeing and affixing their fur to unusual proportions began with the need to complement the Victorian and Georgian ...
Outside of DR3-DQ2 with known associations to autoimmune disease, other factors within A1::DQ2 are believed to also contribute ... July 2008). "Autoimmune-associated HLA-B8-DR3 haplotypes in Asian Indians are unique in C4 complement gene copy numbers and HSP ... DR3-DQ2 is either a known or highly suspect factor in most autoimmune diseases that link to the A1::DQ2 haplotype. ... Most important of which is the TNF (tumor necrosis factors) with 3 loci in the region. Starting from B8, immediately followed ...
Full-time employees have an important role in the temple's active approach of spreading Buddhism: they complement monastics who ...
However, killing another male's cubs, upon the takeover, allows the new selected gene complement of the incoming alpha male to ... These studies show that inbreeding depression and ecological factors have an influence on survival.[20] ... breeding or natural environmental factors, the deleterious inherited traits are culled.[6][7][32] ... "Does morality have a biological basis? An empirical test of the factors governing moral sentiments relating to incest" ...
J Altern Complement Med. 4 (3): 289-303. doi:10.1089/acm.1998.4.3-289. PMID 9764768.. ... Temperature requirements and growth rates are crucial factors that identify O. sinensis from other similar fungi.[6] Climate ...
Their bodies needed additional support, because buoyancy was no longer a factor. Water retention was now important, since it ... other legless reptiles are considered tetrapods because they are sufficiently like other reptiles that have a full complement ...
Complement. deficiency. *C1-inhibitor (Angioedema/Hereditary angioedema). *Complement 2 deficiency/Complement 4 deficiency ... 4) β-Scaffold factors. with minor groove contacts. 4.2. *Hyperimmunoglobulin E syndrome ...
It is widely accepted that there is a factor within the tissues of the first branchial arch that is necessary for the ... The location of the canines reflect their dual function as they complement both the premolars and incisors during chewing. ...
When estimating static lung compliance, volume measurements by the spirometer needs to be complemented by pressure transducers ... and so a correction factor is needed to adjust for standard pressure. Online calculators are available to correct for ...
The N terminus interacts with other cellular transcription factors in a ligand-independent manner; and, depending on these ... complement receptors, Fc receptors, B cell receptors and T cell receptors.[12] ...
Factor Xa inhibitors. (with some II inhibition). Heparin group/. glycosaminoglycans/. (bind antithrombin). *Low molecular ... Plasmin, in addition to lysing fibrin clots, also cleaves the complement system component C3, and fibrin degradation products ... Many trauma patients suffer from an overwhelming activation of tissue factor and thus massive hyperfibrinolysis.[6] Also in ... The ELT measures fibrinolysis by clotting the euglobulin fraction (primarily the important fibrinolytic factors fibrinogen, PAI ...
... complement - complement cascade - complementary and alternative therapy - complete blood count (CBC) - computed tomography scan ... granulocyte macrophage-colony stimulating factor (GM-CSF) - granulocyte-colony stimulating factor (G-CSF) - granulocytopenia ... host factors - HPTN - HPV - HRSA - HTLV-I - HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP) - HTLV-II - ...
This is complemented by less respectable amusements such as striptease, which, since the 1950s has become a fixture of certain ... These and other factors meant that industries relating to construction, repair, and victualling of naval and merchant ships ...
B cells make antibodies that can bind to pathogens, block pathogen invasion, activate the complement system, and enhance ... Cancer - either by growth factors secreted by the tumor or invasion of bone marrow by the cancer ...
Factor I deficiency (pyogenic infections) Factor H deficiency (haemolytic-uraemic syndrome, membranoproliferative ... Complement deficiencies are the result of a lack of any of these proteins. They may predispose to infections but also to ... The complement system is part of the innate as well as the adaptive immune system; it is a group of circulating proteins that ... MASP2 deficiency Complement receptor 3 (CR3) deficiency Membrane cofactor protein (CD46) deficiency Membrane attack complex ...
The lupus anticoagulant will inhibit all the contact activation pathway factors (factor VIII, factor IX, factor XI and factor ... as well as activation of coagulation and complement pathways.[3] ... Levels of protein C, free and total protein S, factor VIII, ... Risk factors[edit]. Risk factors for developing antiphospholipid syndrome include:[citation needed] ... Further studies for factor V Leiden variant and the prothrombin G20210A mutation, factor VIII levels, MTHFR mutation. ...
Steven J. Seiler, Jordana N. Navarro, Bullying on the pixel playground: Investigating risk factors of cyberbullying at the ... J Altern Complement Med. veebruar 2000 ;6(1):7-17. ...
Complement system[change , change source]. The complement system is a biochemical cascade of the immune system that helps ... Chemical factors produced during inflammation attract phagocytes, especially neutrophils.[5] Neutrophils then trigger other ... Elements of the complement cascade can be found in many non-mammalian species including plants, birds, fish and some species of ... Starts the complement cascade to identify bacteria, activate cells and clear out dead cells. ...
Other factors that indicate giftedness will also be considered for eligibility.[31] ... a significant complement of teachers, as well as, many full and part-time staff members. The Administrative infrastructure ... depending on a number of factors, such as property values and the personal income of district residents. Act 1 included 10 ...
Complement factor I deficiency is a disorder that affects the immune system. Explore symptoms, inheritance, genetics of this ... or absent complement factor I. The lack (deficiency) of functional complement factor I protein allows uncontrolled activation ... medlineplus.gov/genetics/condition/complement-factor-i-deficiency/ Complement factor I deficiency. ... Complement factor I and several related proteins protect healthy cells by preventing activation of the complement system when ...
... Jean Charchaflieh,1 Julie Rushbrook,2 Samrat Worah,2 and Ming Zhang ...
Factor H functions as a cofactor in the inactivation of C3b by factor I and also increases the rate of dissociation of the ... C3bBb complex (C3 convertase) and the (C3b)NBB complex (C5 convertase) in the alternative complement pathway. UniProt ...
CFHR5 complement factor H related 5 [Homo sapiens] CFHR5 complement factor H related 5 [Homo sapiens]. Gene ID:81494 ... complement factor H-related protein 5. Names. factor H-related protein 5. ... Studies indicate that complement factor H-related proteins (FHR1-5) may enhance complement activation, with important ... Title: Complement factor H-related hybrid protein deregulates complement in dense deposit disease. ...
Schulz, T. F.; Schwaeble, W.; Stanley, K. K.; Weiss, Elisabeth H.; Dierich, Manfred P. (1986): Human complement factor H. ... kDa fragment of human complement control protein factor H using polyclonal and monoclonal antibodies to screen a human liver ...
Factor I deficiency in turn leads to low levels of complement component 3 (C3), factor B, factor H and properdin. In blood, due ... Complement factor I (factor I) is a protein of the complement system, first isolated in 1966 in guinea pig serum, that ... Loss of function mutations in the Complement Factor I gene lead to low levels of factor I which results in increased complement ... Complement factor I, also known as C3b/C4b inactivator, is a protein that in humans is encoded by the CFI gene. ...
Complement factor B is a protein that in humans is encoded by the CFB gene. This gene encodes complement factor B, a component ... CFB complement factor B". Ambrus JL, Peters MG, Fauci AS, Brown EJ (March 1990). "The Ba fragment of complement factor B ... Christie DL, Gagnon J (January 1983). "Amino acid sequence of the Bb fragment from complement Factor B. Sequence of the major ... Upon activation of the alternative pathway, it is cleaved by complement factor D yielding the noncatalytic chain Ba and the ...
Factor H-like 1 (FHL-1) is a splice variant of FH that also possesses complement-inhibiting function. Additional five factor H- ... In addition to its canonical role in complement regulation, several other functions of FH have been discovered and a large ... While other fluid-phase and cell membrane-bound regulators of complement have been identified, FH is essential for controlling ... Moreover, numerous pathogenic microbes and some tumor cells have developed the ability to exploit FH for complement evasion. ...
Buy our Recombinant Human Complement factor B protein (denatured). Ab174401 is a protein fragment produced in Escherichia coli ... Recombinant Human Complement factor B protein (denatured). See all Complement factor B proteins and peptides. ... Factor B which is part of the alternate pathway of the complement system is cleaved by factor D into 2 fragments: Ba and Bb. Bb ... a serine protease, then combines with complement factor 3b to generate the C3 or C5 convertase. It has also been implicated in ...
Complement factor H gene associations with end-stage kidney disease in African Americans.. Bonomo JA1, Palmer ND2, Hicks PJ3, ... Mutations in the complement factor H gene (CFH) region associate with renal-limited mesangial proliferative forms of ...
Antiphagocytic activity of streptococcal M protein: selective binding of complement control protein factor H. R D Horstmann, H ... Isolated complement components were used to study the regulation of the alternative complement pathway C3 convertase (EC 3.4. ... Antiphagocytic activity of streptococcal M protein: selective binding of complement control protein factor H ... Antiphagocytic activity of streptococcal M protein: selective binding of complement control protein factor H ...
Staphylococcus aureusClumping Factor A Binds to Complement Regulator Factor I and Increases Factor I Cleavage of C3b ... Myeloperoxidase influences the complement regulatory activity of complement factor H. Su-Fang Chen, Feng-Mei Wang, Zhi-Ying Li ... Factor H and Factor H-Related Protein 1 Bind to Human Neutrophils via Complement Receptor 3, Mediate Attachment to Candida ... Acquisition of Complement Inhibitor Serine Protease Factor I and Its Cofactors C4b-Binding Protein and Factor H by Prevotella ...
Complement factor H-related hybrid protein deregulates complement in dense deposit disease. Qian Chen,1 Michael Wiesener,2 ... Variations in the complement regulatory genes factor H (CFH) and factor H related 5 (CFHR5) are associated with ... Then, factor B (2.5 μg/ml, CompTech), factor D (0.5 μg/ml, CompTech), and factor P (2 μg/ml, CompTech), together with the CFHR2 ... Two additional human serum proteins structurally related to complement factor H. Evidence for a family of factor H-related ...
Browse our Complement Factor H Lysate catalog backed by our Guarantee+. ... Complement Factor H Lysates available through Novus Biologicals. ... Complement Factor H Lysates. We offer Complement Factor H ... factor H lysate, factor H-like 1 lysate, FH lysate, FHL1 lysate, H factor 1 (complement) lysate, H factor 1 lysate, H factor 2 ... Alternate Names for Complement Factor H Lysates. Complement Factor H lysate, CFH lysate, adrenomedullin binding protein lysate ...
Browse our Complement Factor H Antibodies all backed by our Guarantee+. ... Complement Factor H Antibodies available through Novus Biologicals. ... anti-H factor 1 (complement) antibody, anti-H factor 1 antibody, anti-H factor 2 (complement) antibody, anti-HF antibody, anti- ... Complement Factor H Antibodies. We offer Complement Factor H Antibodies for use in common research applications: ELISA, Flow ...
J:277202 Song D, et al., Complement Factor H Mutation W1206R Causes Retinal Thrombosis and Ischemic Retinopathy in Mice. Am J ...
CRP-Induced Expression of Complement- Inhibitory Factors Protects HSVECs From Complement-Mediated Injury. To assess whether the ... 13 The effect of CRP on the expression of complement-inhibitory factors is unknown. Because complement-inhibitory proteins ... C-Reactive Protein Upregulates Complement-Inhibitory Factors in Endothelial Cells. Shu-Hong Li, Paul E. Szmitko, Richard D. ... C-Reactive Protein Upregulates Complement-Inhibitory Factors in Endothelial Cells. Shu-Hong Li, Paul E. Szmitko, Richard D. ...
A growing number of pathogenic microorganisms have acquired the ability to bind the complement inhibitor factor H (FH) from ... A growing number of pathogenic microorganisms have acquired the ability to bind the complement inhibitor factor H from body ... Initial studies assumed that these proteins are complement inhibitors similar to factor H. However, recent evidence suggest ... In addition to factor H, binding of factor H-related proteins was also demonstrated for several microbes. ...
Essential cofactors for these reactions include factor H and C4BP in the fluid phase and membrane cofactor protein/CD46 and CR1 ... the absence of such cofactors leads to C3b-mediated complement activation and subsequent opsonization. ... of these cofactors on healthy cells allows degradation of deposited C3b by CFI in order to prevent undesired complement ... Trypsin-like serine protease that plays an essential role in regulating the immune response by controlling all complement ...
Factor D cleaves factor B when the latter is complexed with factor C3b, activating the C3bbb complex, which then becomes the C3 ... "Deficient alternative complement pathway activation due to factor D deficiency by 2 novel mutations in the complement factor D ... "Deficient alternative complement pathway activation due to factor D deficiency by 2 novel mutations in the complement factor D ... "Deficient alternative complement pathway activation due to factor D deficiency by 2 novel mutations in the complement factor D ...
The serum concentration of complement factor C9 (C9) was 260 +/- 47 micrograms/ml (+/- SE) in 14 mothers and less than 42 ... Complement Factor 9 Deficiency in Serum of Human Neonates J Infect Dis. 1992 Jul;166(1):53-7. doi: 10.1093/infdis/166.1.53. ... The serum concentration of complement factor C9 (C9) was 260 +/- 47 micrograms/ml (+/- SE) in 14 mothers and less than 42 ...
... factor VIII deficiency (ortholog); FOUND IN extracellular space (inferred); secretory granule (inferred) ... INVOLVED IN complement activation, alternative pathway (inferred); ASSOCIATED WITH autistic disorder (ortholog); Experimental ... Homo sapiens (human) : CFP (complement factor properdin) HGNC Alliance Mus musculus (house mouse) : Cfp (complement factor ... Sus scrofa (pig) : CFP (complement factor properdin) Chlorocebus sabaeus (African green monkey) : CFP (complement factor ...
... products and learn more about anti-Complement Factor D/Adipsin, Polyclonal, Novus Biologicals 0.1mg; 0.1mg; Unlabeled. ... Adipsin, ADNcomplement factor D, C3 convertase activator, complement factor D (adipsin), complement factor D preproprotein, D ... component of complement (adipsin), DF, EC 3.4.21, EC, PFD, Properdin factor DADIPSIN. ...
Since there is complement activation in all areas of tissue injury, and both C3a and C5a activate MSC, it was asked whether ... The signaling cascade responsible for the production of angiogenic factors by C3a or C5a could be defined as activation of the ... C3a caused significant up-regulation of various angiogenic factors, including VEGF, CXCL8/IL-8 and IL-6. In contrast there was ... Although C5a also caused moderate up-regulation of angiogenic factors, the effect was borderline significant. Furthermore the ...
Complement Factor H Polymorphism in Age-Related Macular Degeneration. By Robert J. Klein, Caroline Zeiss, Emily Y. Chew, Jen- ... Complement Factor H Polymorphism in Age-Related Macular Degeneration. By Robert J. Klein, Caroline Zeiss, Emily Y. Chew, Jen- ... Complement Factor H Polymorphism in Age-Related Macular Degeneration Message Subject. (Your Name) has forwarded a page to you ... Among 116,204 single-nucleotide polymorphisms genotyped, an intronic and common variant in the complement factor H gene (CFH) ...
Both age and smoking, two important risk factors for AMD, influence plasma levels of complement factor H (28). CFH sequences ... Complement Factor H Polymorphism in Age-Related Macular Degeneration. By Robert J. Klein, Caroline Zeiss, Emily Y. Chew, Jen- ... Complement Factor H Polymorphism in Age-Related Macular Degeneration. By Robert J. Klein, Caroline Zeiss, Emily Y. Chew, Jen- ... Complement Factor H Polymorphism in Age-Related Macular Degeneration Message Subject. (Your Name) has forwarded a page to you ...
Macrophage factor B was cleaved and activated to factor Bb- and Ba-like fragments by factor D and cobra venom factor [2]. ... Lack of complement factor C3, but not factor B, increases hyperlipidemia and atherosclerosis in apolipoprotein E-/- low-density ... Effects of complement factor D deficiency on the renal disease of MRL/lpr mice. Elliott, M.K., Jarmi, T., Ruiz, P., Xu, Y., ... Complement factor B gene regulation: synergistic effects of TNF-alpha and IFN-gamma in macrophages. Huang, Y., Krein, P.M., ...
Structures of native and complexed complement factor D: implications of the atypical His57 conformation and self-inhibitory ... R-Factor (R-Work). R-Factor (R-Free). R-Factor (R-Free Error). Percent Reflections (Observed). ... R-Factor (All). R-Factor (Observed). R-Work. R-Free. R-Free Selection Details. ...
Complement Factor H Antibody Biotin conjugate (L20/3), GAU 020-03B-01, from Invitrogen™. Species Reactivity: Human; ... Factor H is a regulatory factor of the alternative pathway of complement activation. Factor H is a glycoprotein consisting of a ... GAU 020-03B-01 detects Complement factor H (ß1H-globulin) in human samples. GAU 020-03B-01 has been successfully used in ELISA ... GAU 020-03B-01 immunogen is human Factor H. NOTE: Concentration is lot-dependent and can vary from 0.85-1.15 mg/mL ...
Mouse podocyte complement factor h: the functional analog to human complement receptor 1. Alexander, J.J., Wang, Y., Chang, A ... Complement factor H (Cfh) is a key plasma protein in humans and animals that serves to limit alternative pathway complement ... Complement factor h limits immune complex deposition and prevents inflammation and scarring in glomeruli of mice with chronic ... Distinct and separable roles of the complement system in factor h-deficient bone marrow chimeric mice with immune complex ...
  • Complement factor I and several related proteins protect healthy cells by preventing activation of the complement system when it is not needed. (medlineplus.gov)
  • Additional five factor H-related (FHR) proteins belong to the FH family and have been recognized as competitors of FH for binding to various ligands, thus modulating complement regulation. (frontiersin.org)
  • Methods and Results- Human coronary artery or human saphenous vein ECs were incubated with CRP (0 to 100 μg/mL, 0 to 72 hours), and the expression of the complement-inhibitory proteins decay-accelerating factor (DAF), membrane cofactor protein (CD46), and CD59 were measured by flow cytometry. (ahajournals.org)
  • The increased expression of complement-inhibitory proteins was functionally effective, resulting in significant reduction of complement-mediated lysis of antibody-coated human saphenous vein ECs. (ahajournals.org)
  • The complement system is a complex cascade of enzymes and regulatory proteins that normally participate in host defenses against microorganisms via opsonization, chemoattraction of leukocytes, cell lysis, and cell activation. (ahajournals.org)
  • Among these regulatory proteins is decay-accelerating factor (DAF, CD55), membrane cofactor protein (MCP, CD46), which binds to and facilitates the degradation of C3b and C4b, and CD59, which inhibits C5b-9. (ahajournals.org)
  • Because complement-inhibitory proteins protect the endothelium from complement-mediated injury and CRP is proatherogenic, in part because of its ability to activate complement, we hypothesized that the detrimental effect of CRP may involve the downregulation of these protective proteins. (ahajournals.org)
  • Initial studies assumed that these proteins are complement inhibitors similar to FH. (frontiersin.org)
  • However, recent evidence suggests that FHR proteins may rather enhance complement activation both directly and also by competing with the inhibitor FH for binding to certain ligands and surfaces. (frontiersin.org)
  • This mini review focuses on the role of the main alternative pathway regulator FH in host-pathogen interactions, as well as on the emerging role of the FHR proteins as enhancers of complement activation. (frontiersin.org)
  • The complement system, a major humoral arm of innate immunity, includes ~40 plasma and cell membrane-anchored proteins that act in a cascade-like manner to opsonize microbes and facilitate their phagocytosis, activate cellular responses, initiate inflammation, or directly lyse certain microbes by punching holes into them ( 1 ). (frontiersin.org)
  • (B) The five human FH-related (FHR) proteins retained domains homologous to complement control protein domains 6-9 and 18-20 of FH (showed by vertical alignment). (frontiersin.org)
  • Because complement is a powerful system to facilitate destruction of microbes or other target cells, host cells and tissues are protected by various combinations of fluid phase and membrane complement regulatory proteins that fine tune and/or block the activation steps of the complement cascade, restrict activation in both time and space, and prevent the potential deleterious effects of full-blown, excessive activation (Figure 1 A) ( 3 ). (frontiersin.org)
  • In analogy to the newly described neuroimmune regulatory proteins also known as "don't eat me" signals (CD200, CD47, CD22, fractalkine, semaphorins), we herein identify the key role of complement regulator factor H (fH) in controlling neuroinflammation initiated in an acute mouse model of Ab-dependent experimental autoimmune encephalomyelitis. (jimmunol.org)
  • Interactions of factor H with various endogenous ligands, such as pentraxins, extracellular matrix proteins and DNA are important in limiting local complement-mediated inflammation. (mdpi.com)
  • VEGF regulates local inhibitory complement proteins in the eye and kidney. (lsbio.com)
  • Unexpectedly, levels of factor H in bronchoalveolar samples correlate with levels of albumin, suggesting that the cause of factor H accumulation in bronchial fluid is not its direct secretion from tumor cells but the exudation of plasma proteins to the bronchial lumen. (aacrjournals.org)
  • Common polymorphisms in complement alternative pathway (AP) proteins C3 (C3R102G), factor B (fBR32Q), and factor H (fHV62I) are associated with age-related macular degeneration (AMD) and other pathologies. (csic.es)
  • Copy number variations in the complement factor H-related ( CFHR ) gene cluster on chromosome 1q32 and CFHR5 mutant proteins associate with this disease. (asnjournals.org)
  • Each of these mutated proteins assembled into large multimeric complexes and, compared to CFHR5, bound damaged human cell surfaces and properdin with greater intensity and exacerbated local complement activation. (asnjournals.org)
  • Factor H-related proteins (CFHRs) are important regulators of the human complement system. (asnjournals.org)
  • The complement system is a component of innate immunity produced locally in the CNS, since size restrictions from the blood brain barrier prevent complement proteins from easily passing through from the rest of the body. (uiowa.edu)
  • fH is a member of the genetically and structurally related regulators of complement activation family of proteins. (asm.org)
  • Some microbial pathogens utilize human complement regulatory proteins, such as factor H (FH) and factor H-like protein 1 (FHL-1), for immune evasion. (asm.org)
  • The common pathway for the development of pathogen immunity is to evade complement attack and opsonophagocytosis, which is often influenced or dictated by a pathogen's ability to bind complement regulatory proteins ( 1 , 7 , 8 , 12 , 13 , 15 , 17 , 28 ). (asm.org)
  • 26 ) have identified FbaA, which is expressed by a serotype M1 GAS isolate, 90-226, as the protein that mediates the binding of both human complement regulatory proteins FH and factor H-like protein (FHL-1) ( 25 ). (asm.org)
  • We used fluorescent microscopy, monospecific antibodies against complement components, fluorescent secondary antibodies, and the analysis of .150 images to quantify the attachment of HUVEC-released complement proteins to ULVWF strings secreted by, and anchored to, the HUVECs (under conditions of ADAMTS-13 inhibition). (rice.edu)
  • Complement acts as a danger‐sensing system in the innate immune system, and its activation initiates a strong inflammatory response and cleavage of the proteins C3 and C5 by proteolytic enzymes, the convertases. (embopress.org)
  • Activation of the proteolytic complement cascade triggers cleavage of the homologous 185-200 kDa proteins C3, C4, and C5 ( Supplementary Figure S1 ). (embopress.org)
  • Complement factor I deficiency is caused by mutations in the CFI gene. (medlineplus.gov)
  • This 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)
  • Mutations in the CFI gene that cause complement factor I deficiency result in abnormal, nonfunctional, or absent complement factor I. The lack (deficiency) of functional complement factor I protein allows uncontrolled activation of the complement system. (medlineplus.gov)
  • Complement factor I, also known as C3b/C4b inactivator, is a protein that in humans is encoded by the CFI gene. (wikipedia.org)
  • The gene for Factor I in humans is located on chromosome 4. (wikipedia.org)
  • Loss of function mutations in the Complement Factor I gene lead to low levels of factor I which results in increased complement activity. (wikipedia.org)
  • Complement factor B is a protein that in humans is encoded by the CFB gene. (wikipedia.org)
  • This gene encodes complement factor B, a component of the alternative pathway of complement activation. (wikipedia.org)
  • The polyadenylation site of this gene is 421 bp from the 5' end of the gene for complement component 2. (wikipedia.org)
  • Entrez Gene: CFB complement factor B". Ambrus JL, Peters MG, Fauci AS, Brown EJ (March 1990). (wikipedia.org)
  • Molecular cloning and characterization of the gene coding for human complement protein factor B". Proceedings of the National Academy of Sciences of the United States of America. (wikipedia.org)
  • This gene is a member of a small complement factor H (CFH) gene cluster on chromosome 1. (nih.gov)
  • Each member of this gene family contains multiple short consensus repeats (SCRs) typical of regulators of complement activation. (nih.gov)
  • The protein encoded by this gene has nine SCRs with the first two repeats having heparin binding properties, a region within repeats 5-7 having heparin binding and C reactive protein binding properties, and the C-terminal repeats being similar to a complement component 3 b (C3b) binding domain. (nih.gov)
  • Rare Variants in the Complement Factor H-Related Protein 5 Gene Contribute to Genetic Susceptibility to IgA Nephropathy. (nih.gov)
  • Atypical hemolytic uremic syndrome and genetic aberrations in the complement factor H-related 5 gene. (nih.gov)
  • Complement factor H gene associations with end-stage kidney disease in African Americans. (nih.gov)
  • Mutations in the complement factor H gene (CFH) region associate with renal-limited mesangial proliferative forms of glomerulonephritis including IgA nephropathy (IgAN), dense deposit disease (DDD) and C3 glomerulonephritis (C3GN). (nih.gov)
  • Complement factor 5 is a quantitative trait gene that modifies liver fibrogenesis in mice and humans," Nature Genetics , vol. 37, no. 8, pp. 835-843, 2005. (hindawi.com)
  • Wild-type C57 mice and factor B gene knockout C57 (FBKO) mice were sued. (arvojournals.org)
  • CFH / Complement Factor H is a member of the Regulator of Complement Activation (RCA) gene cluster and encodes a protein with twenty short consensus repeat (SCR) domains. (lsbio.com)
  • Four complement factor H gene polymorphisms in association with AMD: A meta-analysis. (semanticscholar.org)
  • Alternatively spliced transcripts of the human complement C2 gene. (semanticscholar.org)
  • This gene encodes a serine proteinase that is essential for regulating the complement cascade. (mybiosource.com)
  • Defects in this gene cause complement factor I deficiency, an autosomal recessive disease associated with a susceptibility to pyogenic infections. (mybiosource.com)
  • Factor I deficiency in turn leads to low levels of complement component 3 (C3), factor B, factor H and properdin. (wikipedia.org)
  • Properdin is a positive regulator of complement activation. (frontiersin.org)
  • Here, we identified CFHR5 as a pattern recognition protein that binds to damaged human endothelial cell surfaces and to properdin, the human complement activator. (asnjournals.org)
  • Enhanced properdin staining correlated with local complement activation with C3b and C5b-9 deposition on the mesangial cell surface in vitro . (asnjournals.org)
  • Complement can be activated by three major pathways, the classical, the lectin, and the alternative pathway. (frontiersin.org)
  • The recognition molecules of the pathways initiate activation by interacting with enzymatically active components that propagate the cascade and generate active complement fragments and complexes that mediate the biological effects of the system (Figure 1 A) ( 2 ). (frontiersin.org)
  • Complement pathways and the human factor H (FH) protein family. (frontiersin.org)
  • Schematic overview of the major complement activation and regulation pathways. (frontiersin.org)
  • Trypsin-like serine protease that plays an essential role in regulating the immune response by controlling all complement pathways. (uniprot.org)
  • You need to have more background on the various complement factors, their function, the 3 different pathways of activation etc) NCIPH is a liver disorder of vascular origin defined by a portal venous pressure exceeding 5mm Hg between portal vein and inferior vena cava (sanyal 2008), characterized by occlusion of the 3rd /4th order branches of the hepatic portal vein ( madhu 2008). (ukessays.com)
  • Complement Factor I (CFI) is a serum protease capable of inhibiting all complement pathways. (arvojournals.org)
  • Background: Vascular endothelial cells (ECs) express and release protein components of the complement pathways, as well as secreting and anchoring ultra-large von Willebrand factor (ULVWF) multimers in long string-like structures that initiate platelet adhesion during hemostasis and thrombosis. (rice.edu)
  • and C4 of the classical and lectin (but not alternative) complement pathways. (rice.edu)
  • Some bacteria are able to resist phagocytosis and interfere with the body's complement pathways. (ccbcmd.edu)
  • In all three complement pathways, the central molecule is C3, which, upon activation cleavage, forms the major opsonin C3b - the key component of complement. (mysciencework.com)
  • Each Complement Factor H Antibody is fully covered by our Guarantee+, to give you complete peace of mind and the support when you need it. (novusbio.com)
  • Choose from our Complement Factor H polyclonal antibodies and browse our Complement Factor H monoclonal antibody catalog. (novusbio.com)
  • Spitzer RE, Stitzel AE, Tsokos G. On the origin of C3 nephritic factor (antibody to the alternative pathway C3 convertase): evidence for the Adam and Eve concept of autoantibody production. (harvard.edu)
  • Immunoprecipitation analysis of human plasma using anti-Complement factor H antibody. (acris-antibodies.com)
  • AM20110PU-N Factor H antibody staining of Formalin-Fixed, Paraffin-Embedded Human Liver followed by biotinylated secondary antibody, alkaline phosphatase-streptavidin and chromogen. (acris-antibodies.com)
  • Complement Factor B antibody LS-C128908 is an unconjugated goat polyclonal antibody to human Complement Factor B (CFB). (lsbio.com)
  • Antigen - antibody complexes on heavily coated red cells in Warm autoimmune haemolytic anemia (WAIHA) often activates the complement pathway and red cells bound C3 complement component are encountered in complement associated WAIHA (CWAIHA). (springer.com)
  • Schreiber AD, Frank MM (1972) Role of antibody and complement in the immune clearance and destruction of erythrocytes I. in vivo effects of IgG and IgM complement-fixing sites. (springer.com)
  • Strains of group A streptococci lacking M protein are efficiently opsonized by the alternative pathway of complement, but in the absence of type-specific antibody neither the alternative nor the classical pathway is activated by strains expressing M protein ( 3 , 29 ). (asm.org)
  • Lysis is abolished by ethylenediaminetetraacetic acid, which chelates divalent cations and prevents complement activation, and reduced by an antibody to factor D, a key component of the alternative pathway. (rupress.org)
  • Anti-Factor I antibody IHC of human colon, vessels. (mybiosource.com)
  • Complement factor I deficiency is a disorder that affects the immune system. (medlineplus.gov)
  • Some people with complement factor I deficiency have a kidney disorder called glomerulonephritis with isolated C3 deposits. (medlineplus.gov)
  • Complement factor I deficiency can also be associated with autoimmune disorders such as rheumatoid arthritis or systemic lupus erythematosus (SLE). (medlineplus.gov)
  • Baracho GV, Nudelman V, Isaac L. Molecular characterization of homozygous hereditary factor I deficiency. (medlineplus.gov)
  • Grumach AS, Leitão MF, Arruk VG, Kirschfink M, Condino-Neto A. Recurrent infections in partial complement factor I deficiency: evaluation of three generations of a Brazilian family. (medlineplus.gov)
  • Ponce-Castro IM, González-Rubio C, Delgado-Cerviño EM, Abarrategui-Garrido C, Fontán G, Sánchez-Corral P, López-Trascasa M. Molecular characterization of Complement Factor I deficiency in two Spanish families. (medlineplus.gov)
  • The molecular basis of hereditary complement factor I deficiency. (medlineplus.gov)
  • We present a case suggesting that complement factor H deficiency, a known hereditary risk factor for TMA, may also favor development of AAG. (nih.gov)
  • We discuss the potential implications of factor H deficiency in the pathophysiology of renal allograft microvascular injury, leukocyte infiltration and formation of intraglomerular platelet thrombi. (nih.gov)
  • Homozygous complement factor deficiency and primary antiphospholipid syndrome: a clinical and serological study. (bmj.com)
  • Thus, deficiency of CFHR3 and CFHR1 results in a loss of complement control but enhances local regulation by factor H. Our findings allude to a critical balance between the complement regulators CFHR3, CFHR1 and factor H and further emphasize the central role of complement regulation in AMD pathology. (uni-regensburg.de)
  • 2. a preparation of factor VIII administered intravenously for the prevention or treatment of hemorrhage in patients with hemophilia A and the treatment of von Willebrand disease , hypofibrinogenemia , and coagulation factor XIII deficiency. (thefreedictionary.com)
  • Deficiency of factor H, or a functional defect in its N terminus, often leads to membranoproliferative glomerulonephritis and complement depletion, owing to continuous overconsumption of C3. (mysciencework.com)
  • Whereas much attention has been focused on the properties and activities of the TLRs in this process ( 13 ), many other innate immune molecules expressed by glia and neurons have been described (e.g., complement, lectins, scavenger receptors) ( 14 ). (jimmunol.org)
  • Complement is an essential part of innate immunity as it participates in host defense against infections, disposal of cellular debris and apoptotic cells, inflammatory processes and modulation of adaptive immune responses. (mdpi.com)
  • The complement system is a fundamental element of the innate immune system as well as the adaptive immune responses. (ukessays.com)
  • While a role for the complement system on innate as well as adaptive immunity has been documented, the implication of complement activation on the onset of the anti-FVIII immune response is unknown. (haematologica.org)
  • This protein is secreted into the bloodstream and has an essential role in the regulation of complement activation, restricting this innate defense mechanism to microbial infections. (lsbio.com)
  • The complement system, an important component of innate immunity, is the most widely accepted pathogenic pathway of the immune system implicated in AMD. (molvis.org)
  • have found one such mechanism-cross-talk between the alternative complement pathway and natural killer (NK) cells and innate lymphoid cells (ILCs). (sciencemag.org)
  • Despite its important role in innate immunity, very little is known about complement production, regulation, and function in the CNS of healthy or diseased individuals. (uiowa.edu)
  • Complement factor I (factor I) is a protein of the complement system, first isolated in 1966 in guinea pig serum, that regulates complement activation by cleaving cell-bound or fluid phase C3b and C4b. (wikipedia.org)
  • Neither M- nor M+ streptococci directly affected the formation or dissociation of the surface-bound C3b,Bb or the inactivation of surface-bound C3b by factor I. However, the activity of the serum control protein of the alternative complement pathway, factor H, in controlling streptococcus-bound C3b and C3b,Bb was 6-8 times stronger on M+ organisms than on M- organisms. (pnas.org)
  • The serum concentration of complement factor C9 (C9) was 260 +/- 47 micrograms/ml (+/- SE) in 14 mothers and less than 42 micrograms/ml in each of their 14 neonates. (nih.gov)
  • 1. The activation of the protease of blood serum or plasma by streptococcal fibrinolysin requires the presence of both a heat-stable and a heat-labile factor. (jimmunol.org)
  • 3. Useful sources of the heat-labile factor include guinea-pig serum, rabbit plasma, duck serum, and chicken serum. (jimmunol.org)
  • Complement-mediated borreliacidal effects were observed with particular combinations of host serum and Borrelia genospecies. (asm.org)
  • The present study sought to determine whether the generation of chemotactic factor by endotoxin in serum was dependent upon complement system activation. (eurekamag.com)
  • Preheating serum, incubating at 0 degrees C, or incubating in the presence of EDTA, all prevented chemotactic factor generation as well as complement fixation by endotoxin. (eurekamag.com)
  • These experiments demonstrate that generation of chemotactic factor by endotoxin in serum is dependent upon C' system activation involving at least C'5. (eurekamag.com)
  • Group A streptococci possess a wide variety of virulence factors, including M protein, hyaluronic acid capsule, serum opacity factor, C5a peptidase, and extracellular enzymes and toxins ( 8 ). (asm.org)
  • We will now look at the ability of bacteria to resist phagocytic destruction and complement serum lysis. (ccbcmd.edu)
  • 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)
  • We propose that unopposed complement activation is a risk factor for both immune and nonimmune forms of microvascular injuries in renal allografts. (nih.gov)
  • In this study we could show that the immune system and more specifically the complement system was dysregulated in a large fraction of patients. (diva-portal.org)
  • Development of neutralizing antibodies against therapeutic Factor VIII (FVIII) is the most serious complication of the treatment of hemophilia A. There is growing evidence to show the multifactorial origin of the anti-FVIII immune response, combining both genetic and environmental factors. (haematologica.org)
  • Here, using in vitro assays for FVIII endocytosis by human monocyte-derived dendritic cells and presentation to T cells, as well as in vivo complement depletion in FVIII-deficient mice, we show a novel role for complement C3 in enhancing the immune response against therapeutic FVIII. (haematologica.org)
  • The purple sea urchin, Strongylocentrotus purpuratus, possesses a non-adaptive immune system including elements homologous to C3 and factor B (Bf) of the vertebrate complement system. (semanticscholar.org)
  • The species-specific pattern of viability and/or lysis is highly consistent with the pattern of reservoir competence of hosts for B. burgdorferi sensu lato, suggesting a key role of complement in the global ecology of Lyme borreliosis. (asm.org)
  • In the present study, we examined the role of complement factor 3 (C3) in the development of PM-induced AHR and airway inflammation by comparing responses between C3-deficient (C3 -/- ) and wild-type mice. (elsevier.com)
  • Background- Because complement-mediated vascular injury participates in atherosclerosis and C-reactive protein (CRP) can activate the complement cascade, we sought to determine whether CRP affects the expression of the protective complement-inhibitory factors on the cell surface of endothelial cells (ECs). (ahajournals.org)
  • 11 DAF prevents the formation and accelerates the decay of the C3 and C5 convertases that act early within the complement cascade, 11 functioning to maintain vascular integrity as a key protector against complement-mediated cell lysis. (ahajournals.org)
  • The signaling cascade responsible for the production of angiogenic factors by C3a or C5a could be defined as activation of the rho cascade which was necessary for nuclear translocation of NF κ B p65 and of phospho-ERK1/2. (scirp.org)
  • One of the issues in developing a complement-based therapeutic modality for exudative AMD is to decide which molecule to target in the complement cascade,' he said. (ophthalmologytimes.com)
  • An investigational compound being developed by another company [Compstatin, Alcon] blocks activation of C3, which is involved earlier in the complement cascade than C5. (ophthalmologytimes.com)
  • In summary, this study shows that enhancive miR-146a can upregulate the inflammatory factor IL-1β in chronic TLE by downregulating CFH, and that upregulation of IL-1β plays an important feedback-regulating role in the expression of miR-146a and CFH, forming a miR-146a-CFH-IL-1β loop circuit that initiates a cascade of inflammation and then leads to the perpetuate inflammation in TLE. (biologists.org)
  • Inappropriate activation of the complement cascade has been implicated in the pathogenesis of AD. (qub.ac.uk)
  • Complement factor H (CFH) is a regulator of the cascade, but studies on plasma CFH levels in AD have provided mixed results. (qub.ac.uk)
  • In conclusion, on the basis of these and previous results, it appears that DAF plays a central role in preventing the amplification of the complement cascade on host cell surfaces. (rupress.org)
  • C3b is also essential for propagation of the complement cascade to the stage of the lytic terminal complement complexes. (mysciencework.com)
  • Thus, pathogenesis in NCIPH patients could be either defective Vwf multimeric processing by ADAMTS13, excess expression of multimeric vWF which overwhelms the cleavage capacity of ADAMTS13 or any other factor which interferes with this system. (ukessays.com)
  • FBKO mice were more vulnerable to fungal infection and showed a longer disease course than wild-type C57 mice, suggesting that factor B play an important role in the pathogenesis and prognosis of FK. (arvojournals.org)
  • The complement system has been implicated in the pathogenesis of Age-related Macular Degeneration (AMD). (arvojournals.org)
  • Age-related macular degeneration (AMD) is the leading cause of permanent vision loss among the elderly in many industrialized countries, and the complement system plays an important role in the pathogenesis of AMD. (molvis.org)
  • Post-infectious PGNMID and PIGN might share common clinical presentations and pathogenesis related to the complement pathway. (eurekamag.com)
  • The causes of C3G are heterogeneous, and defective complement regulation is often linked to C3G pathogenesis. (asnjournals.org)
  • 4 In diseased glomeruli where complement activation occurred on the glomerular surface, CFHR5 colocalized with both C3b and terminal complement complex C5b-9, 4 thus suggesting that CFHR5-directed complement activation in the glomeruli is relevant for disease pathogenesis. (asnjournals.org)
  • In this section on Bacterial Pathogenesis we are looking at virulence factors that promote bacterial colonization of the host . (ccbcmd.edu)
  • To prevent host cell damage, nucleated cells have developed membrane-bound regulators of complement activation. (ahajournals.org)
  • Several studies reported links between genetic variations of FH and disease, thus confirming the important role of FH in the regulation of complement activation. (frontiersin.org)
  • The complement Factor H protein is secreted into the bloodstream and acts in the regulation of complement activation. (acris-antibodies.com)
  • A heterozygous complement factor H mutation was detected by comprehensive genetic testing of alternative pathway regulatory genes, which might lead to persistent infection-triggered alternative pathway activation and account for severe glomerulonephritis. (eurekamag.com)
  • A frequent deletion of complement factor H (CFH)-related genes CFHR3 and CFHR1 (ΔCFHR3/CFHR1) is considered to have a protective effect against age-related macular degeneration (AMD), although the underlying mechanism remains elusive. (uni-regensburg.de)
  • The 155-KDa glycoprotein complement factor H (FH) was first described in 1965 and soon emerged as a major soluble inhibitor of the complement system. (frontiersin.org)
  • A growing number of pathogenic microorganisms have acquired the ability to bind the complement inhibitor factor H (FH) from body fluids and thus hijack its host protecting function. (frontiersin.org)
  • conducted a clinical trial of lampalizumab, an inhibitor of complement factor D, and noted a potential therapeutic effect on GA. (aao.org)
  • In this review we discuss current concepts on the physiological and pathophysiological roles of factor H in light of new data and recent developments in our understanding of the versatile roles of factor H as an inhibitor of complement activation and inflammation, as well as a mediator of cellular interactions. (mdpi.com)
  • Factor H is an inhibitor of complement activation and promotes tumor growth in vivo ( 18 ). (aacrjournals.org)
  • We determined the crystal structures of the C3b homologue cobra venom factor (CVF) in complex with C5, and in complex with C5 and the inhibitor SSL7 at 4.3 Å resolution. (embopress.org)
  • Atypical Hemolytic Uremic Syndrome Atypical hemolytic uremic syndrome is caused by complement overactivation. (wikipedia.org)
  • Impaired regulatory as well as ligand and cell recognition functions of factor H, caused by mutations or autoantibodies, are associated with the kidney diseases: atypical hemolytic uremic syndrome and dense deposit disease and the eye disorder: age-related macular degeneration. (mdpi.com)
  • Defect in the factor H C terminus leads to a dramatically increased risk of atypical hemolytic uremic syndrome. (mysciencework.com)
  • Evidence for non-traditional activation of complement factor C3 during murine liver regeneration," Molecular Immunology , vol. 45, no. 11, pp. 3125-3132, 2008. (hindawi.com)
  • C3a and C3b activation products of the third component of complement (C3) are critical for normal liver recovery after toxic injury," Journal of Immunology , vol. 173, no. 2, pp. 747-754, 2004. (hindawi.com)
  • Adipocyte lysis in the presence of NeF is associated with the generation of fluid-phase terminal complement complexes, the level of which correlates closely with the level of lactate dehydrogenase, a marker of cell lysis. (rupress.org)
  • Factor B which is part of the alternate pathway of the complement system is cleaved by factor D into 2 fragments: Ba and Bb. (abcam.com)
  • 3 CRP elicits a multitude of effects on endothelial biology that favor a proatherosclerotic phenotype, such as decreasing NO release, 4 upregulating adhesion molecules, 5 stimulating vascular smooth muscle cell proliferation and migration, 6 and activating the complement system. (ahajournals.org)
  • These include interactions with the host complement system that may facilitate pathogen entry into cells and tissues, expression of molecules that defuse the effector complement components and complexes, and acquisition of host complement inhibitors to downregulate complement activity on the surface of the pathogen. (frontiersin.org)
  • The complement system has been shown to mediate renal ischemia-reperfusion (I/R) injury. (rug.nl)
  • Factor H is a major regulatory protein of the complement system. (acris-antibodies.com)
  • CFD, also known as adipsin, is involved in the alternative complement pathway of the complement system where it cleaves factor B. This protein is a member of the trypsin family of peptidases. (acris-antibodies.com)
  • Interactions of the complement system with endotoxic lipopolysaccharide. (eurekamag.com)
  • Endotoxic lipopolysaccharide has recently been shown to fix large amounts of the complement components related to the biologic activities mediated by that system. (eurekamag.com)
  • Molecular sieve chromatography and sucrose density gradient ultracentrifugation demonstrated that the chemotactic factor was a relatively low molecular weight product (15,000-30,000) and as such different from previously scribed C' system-derived chemotactic factors. (eurekamag.com)
  • It plays a critical role in the homeostasis of the complement system in plasma and in the protection of bystander host cells and tissues from damage by complement activation. (ptglab.com)
  • demonstrated that M6 protein and other M serotypes bind factor H (fH), a regulatory protein of the complement system, resulting in reduced deposition of C3b on the streptococcal surface ( 15 ). (asm.org)
  • The alternative complement pathway (AP) is an important nonantibody- requiring host defense system. (rice.edu)
  • Recent data indicate a previously unsuspected link between the complement system and adipocyte biology. (rupress.org)
  • Coelomocytes express SpBf, a homologue of factor B, the second component in the sea urchin complement system. (semanticscholar.org)
  • Echinoderm immunity and the evolution of the complement system. (semanticscholar.org)
  • The ancestral complement system in sea urchins. (semanticscholar.org)
  • C3b and factor H: key components of the complement system. (mysciencework.com)
  • Production and interferon-gamma-mediated regulation of complement component C2 and factors B and D by the astroglioma cell line U105-MG". The Biochemical Journal. (wikipedia.org)
  • It is a component of the alternative complement pathway best known for its role in humoral suppression of infectious agents. (acris-antibodies.com)
  • CFB / Complement Factor B is complement factor B, a component of the alternative pathway of complement activation. (lsbio.com)
  • Chemotactic factor could not be generated by endotoxin in sera of mice congenitally deficient in the C'S component of complement, while chemotactic factor was generated by endotoxin in the sera of coisogenic mice with normal complement levels for that species. (eurekamag.com)
  • Furthermore, the relatively low molecular weight of this factor suggests that it might be derived from activation of a single complement component rather than from complexing of multiple complement components. (eurekamag.com)
  • It recognizes the complement component 3b (C3b) and 3d (C3d) fragments in addition to self cell markers (i.e. glycosaminoglycans, sialic acid) to distinguish host cells that deserve protection from pathogens that should be eliminated. (biochemj.org)
  • Structural and functional analysis of the complement component factor H with the use of different enzymes and monoclonal antibodies to factor H. (semanticscholar.org)
  • Sea urchin coelomocytes specifically express a homologue of the complement component C3. (semanticscholar.org)
  • In addition, CFHR3 and CFHR1 compete with factor H for binding to the central complement component C3. (uni-regensburg.de)
  • the activated complex of the fifth component of complement C5a induces chemotaxis in the case of polymorphonuclear leukocytes. (thefreedictionary.com)
  • factor B a complement component that participates in the alternative complement pathway. (thefreedictionary.com)
  • Upon activation of the alternative pathway, it is cleaved by complement factor D yielding the noncatalytic chain Ba and the catalytic subunit Bb. (wikipedia.org)
  • Factor H functions as a cofactor in the inactivation of C3b by factor I and also increases the rate of dissociation of the C3bBb complex (C3 convertase) and the (C3b)NBB complex (C5 convertase) in the alternative complement pathway. (rcsb.org)
  • Isolated complement components were used to study the regulation of the alternative complement pathway C3 convertase (EC, also called C3b,Bb, on M protein-carrying (M+) and M protein-lacking (M-) streptococci. (pnas.org)
  • The classical pathway of complement may be activated by CRP bound to enzymatically degraded low-density lipoprotein. (ahajournals.org)
  • Factor D cleaves factor B when the latter is complexed with factor C3b, activating the C3bbb complex, which then becomes the C3 convertase of the alternate pathway. (uniprot.org)
  • Although rho was only transiently activated, inhibition of the rho or "downstream of it" of the NF κ B pathway, prevented C3a-and C5a-induced up-regulation of angiogenic factors. (scirp.org)
  • Factor H is a regulatory factor of the alternative pathway of complement activation. (fishersci.com)
  • Increased circulating fH and fB concentrations in subjects with altered glucose tolerance could reflect increased SVC-induced activation of the alternative pathway of complement in omental adipose tissue linked to insulin resistance and metabolic disturbances. (diabetesjournals.org)
  • Factor H is a major soluble regulator of the alternative complement pathway, but it can also bind to host cells and tissues, protecting them from complement attack. (mdpi.com)
  • Complement Factor D, also known as adipsin, is a serine protease that catalyzes the initial proteolytic step in the alternative pathway of complement. (rndsystems.com)
  • Complement and the alternative pathway play an important role in LPS/D-GalN-induced fulminant hepatic failure," PloS ONE , vol. 6, no. 11, Article ID e26838, 2011. (hindawi.com)
  • Inhibition of complement factor B, a key regulator of the alternative pathway, is implicated as a potential therapeutic intervention for AMD. (molvis.org)
  • The results suggest that inhibition of liver factor B mRNA by factor B ASOs would reduce systemic alternative complement pathway activation and has potential to be used as a novel therapy for AMD. (molvis.org)
  • Complement factor H (CFH) is an abundant plasma glycoprotein that regulates the function of the alternative complement pathway in fluid phase and on cellular surfaces (PMID: 2963625). (ptglab.com)
  • Factor H binds to C3b, accelerates the decay of the alternative pathway C3-convertase (C3bBb) and acts as a cofactor for the factor I-mediated proteolytic inactivation of C3b (PMID: 15163532). (ptglab.com)
  • They report that complement factor P (CFP), a positive regulator of the alternative complement pathway, binds NKp46, which is expressed on subsets of NK cells and ILC1 and ILC3. (sciencemag.org)
  • These data suggest that ILCs and the alternative complement pathway cooperate to fight off bacterial infection. (sciencemag.org)
  • The complement pathway contributes to inflammatory cell recruitment, cell lysis, and opsonization, and thus requires regulation to avoid inappropriate activation. (uiowa.edu)
  • a regulator of the alternative pathway of complement activation. (uiowa.edu)
  • fH regulates complement activation by acting as a cofactor for factor I-dependent cleavage of C3b ( 27 ) and by disrupting the alternative pathway C3 convertase ( 35 , 37 ). (asm.org)
  • Complement factor H (CFH) is a soluble complement regulatory protein essential for the down-regulation of the alternative pathway on interaction with specific markers on the host cell surface. (biochemj.org)
  • Murine adipocytes produce key components of the alternative pathway of complement and are able to activate this pathway. (rupress.org)
  • This suggested to us an explanation for adipose tissue loss in partial lipodystrophy, a rare human condition usually associated with the immunoglobulin G(IgG) autoantibody nephritic factor (NeF) which leads to enhanced alternative pathway activation in vivo. (rupress.org)
  • The alternative pathway (AP) C3 convertase C3bBb is formed when factor B (fB) combines with C3b, after which fB is activated by factor D (fD) ( Figure 1A ). (embopress.org)
  • The DAF inhibitory effect on EAC14 or EAC43 was not overcome by supplying an excess of C2 or factor B, but the alternative pathway C3 convertase could be assembled in the presence of Ni++, or nonphysiological concentrations of Mg++, which enhances the binding affinity of factor B for C3b. (rupress.org)
  • Factor I is a glycoprotein heterodimer consisting of a disulfide linked heavy chain and light chain. (wikipedia.org)
  • Factor H is a glycoprotein consisting of a single polypeptide chain with a molecular mass of 150 kDa. (fishersci.com)
  • Kinetic analysis of the interactions of complement receptor 2 (CR2, CD21) with its ligands C3d, iC3b, and the EBV glycoprotein gp350/220. (semanticscholar.org)
  • Inhibition of complement activation on the surface of cells after incorporation of decay-accelerating factor (DAF) into their membranes. (rupress.org)
  • 9 This is supported by the presence of activated complement components in atherosclerotic plaques, such as the membrane attack complex (MAC, C5b-9), which promotes cellular activation, upregulates adhesion molecules, stimulates chemokine secretion, and can cause cell lysis. (ahajournals.org)
  • Complement-mediated adipocyte lysis by nephritic factor sera. (rupress.org)
  • We hypothesized that in the presence of NeF, there is dysregulated complement activation at the membrane of the adipocyte, leading to adipocyte lysis. (rupress.org)
  • When incorporated into the membrane of rabbit erythrocytes, human DAF inhibited their lysis by human complement. (rupress.org)
  • We isolated cDNA clones coding for the functionally important tryptic N-terminal38- kDa fragment of human complement control protein factor H using polyclonal and monoclonal antibodies to screen a human liver cDNA library cloned in a bacterial expression vector, PEX-1. (uni-muenchen.de)
  • Human polyclonal and monoclonal IgG and IgM complement 3 nephritic factors: evidence for idiotypic commonality. (harvard.edu)
  • Human complement factor H. isolation of cDNA clones and partial cDNA sequence of the 38-kDa tryptic fragment containing the binding site for C3b. (uni-muenchen.de)
  • Complement factor B Bb fragment. (abcam.com)
  • Immunochemical studies of the alkaline-denatured C3 suggested that factor H interacts with surfaces of C3 that are situated within the C3c fragment and that are defined by C3(SN) antigens, while factor I predominantly interacts with C3(SN) antigens associated with the C3d fragment and with C3(D) antigens hidden in native C3. (diva-portal.org)
  • The factor I heavy chain has four domains: an FI membrane attack complex (FIMAC) domain, CD5 domain, and low density lipoprotein receptor 1 and 2 (LDLr1 and LDLr2) domains. (wikipedia.org)
  • the heavy chain plays an inhibitory role in maintaining the enzyme inactive until it meets the complex formed by the substrate (either C3b or C4b) and a cofactor protein (Factor H, C4b-binding protein, complement receptor 1, and membrane cofactor protein). (wikipedia.org)
  • We therefore characterized the CFHR3 function and identified CFHR3 as a novel human complement regulator that inhibits C3 convertase activity. (uni-regensburg.de)
  • Essential cofactors for these reactions include factor H and C4BP in the fluid phase and membrane cofactor protein/CD46 and CR1 on cell surfaces. (uniprot.org)
  • Our published work showed that fBR32Q influences C3 convertase formation, whereas fHV62I affects factor I cofactor activity. (csic.es)
  • The action of six different enzymes on the function and structure of Factor H was investigated by use of sodium dodecyl sulphate/polyacrylamide-gel electrophoresis, haemagglutination, two enzyme-linked immunosorbent assay systems and an assay for Factor I cofactor activity. (semanticscholar.org)
  • Plasma complement components and activation fragments: associations with age-related macular degeneration genotypes and phenotypes. (umassmed.edu)
  • Preliminary results of a phase I/II study suggest that targeting complement factor 5 in combination with anti-vascular endothelial growth factor therapy may have a role for improving visual acuity outcomes to eyes with exudative age-related macular degeneration. (ophthalmologytimes.com)
  • In addition, recently, a polymorphism in the middle part of factor H (Y402H) has been shown to be the major risk factor for the most common cause of blindness in the industrialized world: age-related macular degeneration. (mysciencework.com)
  • Inactivates complement subcomponents C3b, iC3b and C4b by proteolytic cleavage. (uniprot.org)
  • Factor H is released or modified following this cleavage. (acris-antibodies.com)
  • In vitro , complement C3 and its cleavage product C3b enhanced FVIII endocytosis by dendritic cells and presentation to a FVIII-specific CD4 + T-cell hybridoma. (haematologica.org)
  • While other fluid-phase and cell membrane-bound regulators of complement have been identified, FH is essential for controlling complement activation on various cellular and non-cellular surfaces, including basement membranes. (frontiersin.org)
  • We offer Complement Factor H Lysates for use in common research applications: Western Blot. (novusbio.com)
  • Endotoxoids" deficient in complement-firing activity were also deficient in chemotactic factor generation. (eurekamag.com)
  • The chemotactic factor induced by endotoxin was heat stable and nondialyzable. (eurekamag.com)
  • In order to prevent damage to self cells and tissues and restrict overconsumption of the complement components, C3b molecules need to be controlled by factor H. Defect in C3 functions leads to compromised microbial defence and increased susceptibility to certain autoimmune diseases. (mysciencework.com)
  • Moreover, numerous pathogenic microbes and some tumor cells have developed the ability to exploit FH for complement evasion. (frontiersin.org)
  • 3. FH in complement evasion by microbes or tumor cells. (frontiersin.org)
  • Our findings imply that SdrE functions as a 'clamp' to capture CFH's C-terminal tail via a unique CDLL mechanism and sequesters CFH on the surface of S. aureus for complement evasion. (biochemj.org)
  • Staphylococcus epidermidis induces complement activation, tumor necrosis factor and interleukin-1, a shock-like state and tissue injury in rabbits without endotoxemia. (jci.org)
  • Tumor necrosis factor (TNF) and IL-1 are thought to mediate many of the pathophysiologic changes of endotoxemia and Gram-negative bacteremia. (jci.org)
  • CTRP1 (Complement C1q tumor necrosis factor-related protein 1) is a member of the CTRP superfamily and is localized to human chromosome 17. (biovendor.com)
  • The factor I light chain contains only the serine protease domain. (wikipedia.org)
  • Bb, a serine protease, then combines with complement factor 3b to generate the C3 or C5 convertase. (abcam.com)
  • It is an exceptionally specific protease and the only known protein substrate is factor B in complex with C3.3 Factor D protease activity is regulated by reversible conformational changes, which differs from the majority of serine proteases whose regulation involves either activation by processing of the zymogens or inactivation by binding of the inhibitors. (rndsystems.com)
  • Bb, a serine protease, is generated when factor B is cleaved by COMPLEMENT FACTOR D into Ba and Bb. (umassmed.edu)
  • COMPLEMENT FACTOR I). This abnormally stabilized enzyme induces a continuous COMPLEMENT ACTIVATION and generation of C3b thereby promoting the assembly of MEMBRANE ATTACK COMPLEX and cytolysis. (harvard.edu)
  • The activated complex of the fifth, sixth, and seventh components of complement (C567), which induces chemotaxis of polymorphonuclear leukocytes. (thefreedictionary.com)
  • The 170 kDa protein was identified as the human complement protein Factor H. Human Factor H, isolated by a different method, was shown to bind specifically to L-selectin in the presence of CaCl 2 , and binding was inhibited by anti-L-selectin antibodies, fucoidan and lipopolysaccharide. (biochemj.org)
  • Streptococcus pyogenes evades complement by binding the complement-regulatory protein factor H (fH) via the central conserved C-repeat region of M protein. (asm.org)
  • 11 ) also confirmed that the acquisition of complement regulatory protein factor H (FH) by GAS contributes to the bacterium's capacity to evade phagocytosis by polymorphonuclear leukocytes (PMNs). (asm.org)
  • Furthermore, a study, using well-characterized human donor eyes, showed that AMD disease severity and complement genotypes are associated with complement activation in the eye. (molvis.org)
  • Neutralization of the complement factor C3-dependent antichlamydial activity was dependent on the proteolytic activity of Chlamydia trachomatis CPAF and correlated with the CPAF-mediated degradation of complement factor C3 and factor B (zeige ATP5S ELISA Kits ). (antikoerper-online.de)
  • Conventional protease inhibitors do not completely inactivate Factor I but they can do so if the enzyme is pre-incubated with its substrate: this supports the proposed rearrangement of the molecule upon binding to the substrate. (wikipedia.org)
  • 4. The activation of blood protease by streptococcal fibrinolysin is prevented by many reagents that remove or destroy midpiece of complement. (jimmunol.org)
  • C4b2a is the widely used short‐hand nomenclature for the C4b-C2a complex in the complement field, and similar nomenclature is used for the remaining complexes in the following). (embopress.org)
  • These results suggest that exposure to PM may induce AHR through activation of complement factor 3 in the airways. (elsevier.com)