Complement Factor B: A glycine-rich, heat-labile serum glycoprotein that contains a component of the C3 CONVERTASE ALTERNATE PATHWAY (C3bBb). Bb, a serine protease, is generated when factor B is cleaved by COMPLEMENT FACTOR D into Ba and Bb.Complement C2: A component of the CLASSICAL COMPLEMENT PATHWAY. C2 is cleaved by activated COMPLEMENT C1S into COMPLEMENT C2B and COMPLEMENT C2A. C2a, the COOH-terminal fragment containing a SERINE PROTEASE, combines with COMPLEMENT C4B to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).Complement Factor H: An important soluble regulator of the alternative pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It is a 139-kDa glycoprotein expressed by the liver and secreted into the blood. It binds to COMPLEMENT C3B and makes iC3b (inactivated complement 3b) susceptible to cleavage by COMPLEMENT FACTOR I. Complement factor H also inhibits the association of C3b with COMPLEMENT FACTOR B to form the C3bB proenzyme, and promotes the dissociation of Bb from the C3bBb complex (COMPLEMENT C3 CONVERTASE, ALTERNATIVE PATHWAY).Complement Factor D: A serum protein which is important in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY. This enzyme cleaves the COMPLEMENT C3B-bound COMPLEMENT FACTOR B to form C3bBb which is ALTERNATIVE PATHWAY C3 CONVERTASE.Complement C3b: The larger fragment generated from the cleavage of COMPLEMENT C3 by C3 CONVERTASE. It is a constituent of the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb), and COMPLEMENT C5 CONVERTASES in both the classical (C4b2a3b) and the alternative (C3bBb3b) pathway. C3b participates in IMMUNE ADHERENCE REACTION and enhances PHAGOCYTOSIS. It can be inactivated (iC3b) or cleaved by various proteases to yield fragments such as COMPLEMENT C3C; COMPLEMENT C3D; C3e; C3f; and C3g.Complement C3 Convertase, Alternative Pathway: 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.Complement C3: A glycoprotein that is central in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C3 can be cleaved into COMPLEMENT C3A and COMPLEMENT C3B, spontaneously at low level or by C3 CONVERTASE at high level. The smaller fragment C3a is an ANAPHYLATOXIN and mediator of local inflammatory process. The larger fragment C3b binds with C3 convertase to form C5 convertase.Complement Pathway, Alternative: Complement activation initiated by the interaction of microbial ANTIGENS with COMPLEMENT C3B. When COMPLEMENT FACTOR B binds to the membrane-bound C3b, COMPLEMENT FACTOR D cleaves it to form alternative C3 CONVERTASE (C3BBB) which, stabilized by COMPLEMENT FACTOR P, is able to cleave multiple COMPLEMENT C3 to form alternative C5 CONVERTASE (C3BBB3B) leading to cleavage of COMPLEMENT C5 and the assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.Complement Activation: The sequential activation of serum COMPLEMENT PROTEINS to create the COMPLEMENT MEMBRANE ATTACK COMPLEX. Factors initiating complement activation include ANTIGEN-ANTIBODY COMPLEXES, microbial ANTIGENS, or cell surface POLYSACCHARIDES.Complement Factor I: A plasma serine proteinase that cleaves the alpha-chains of C3b and C4b in the presence of the cofactors COMPLEMENT FACTOR H and C4-binding protein, respectively. It is a 66-kDa glycoprotein that converts C3b to inactivated C3b (iC3b) followed by the release of two fragments, C3c (150-kDa) and C3dg (41-kDa). It was formerly called KAF, C3bINF, or enzyme 3b inactivator.Macular Degeneration: 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.Cobra Venoms: Venoms from snakes of the genus Naja (family Elapidae). They contain many specific proteins that have cytotoxic, hemolytic, neurotoxic, and other properties. Like other elapid venoms, they are rich in enzymes. They include cobramines and cobralysins.Complement System Proteins: Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY).Complement C4: A glycoprotein that is important in the activation of CLASSICAL COMPLEMENT PATHWAY. C4 is cleaved by the activated COMPLEMENT C1S into COMPLEMENT C4A and COMPLEMENT C4B.Complement C3b Inactivator Proteins: Endogenous proteins that inhibit or inactivate COMPLEMENT C3B. They include COMPLEMENT FACTOR H and COMPLEMENT FACTOR I (C3b/C4b inactivator). They cleave or promote the cleavage of C3b into inactive fragments, and thus are important in the down-regulation of COMPLEMENT ACTIVATION and its cytolytic sequence.Complement C5: C5 plays a central role in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C5 is cleaved by C5 CONVERTASE into COMPLEMENT C5A and COMPLEMENT C5B. The smaller fragment C5a is an ANAPHYLATOXIN and mediator of inflammatory process. The major fragment C5b binds to the membrane initiating the spontaneous assembly of the late complement components, C5-C9, into the MEMBRANE ATTACK COMPLEX.Enzyme Precursors: Physiologically inactive substances that can be converted to active enzymes.Complement C5a: The minor fragment formed when C5 convertase cleaves C5 into C5a and COMPLEMENT C5B. C5a is a 74-amino-acid glycopeptide with a carboxy-terminal ARGININE that is crucial for its spasmogenic activity. Of all the complement-derived anaphylatoxins, C5a is the most potent in mediating immediate hypersensitivity (HYPERSENSITIVITY, IMMEDIATE), smooth MUSCLE CONTRACTION; HISTAMINE RELEASE; and migration of LEUKOCYTES to site of INFLAMMATION.Complement C1q: A subcomponent of complement C1, composed of six copies of three polypeptide chains (A, B, and C), each encoded by a separate gene (C1QA; C1QB; C1QC). This complex is arranged in nine subunits (six disulfide-linked dimers of A and B, and three disulfide-linked homodimers of C). C1q has binding sites for antibodies (the heavy chain of IMMUNOGLOBULIN G or IMMUNOGLOBULIN M). The interaction of C1q and immunoglobulin activates the two proenzymes COMPLEMENT C1R and COMPLEMENT C1S, thus initiating the cascade of COMPLEMENT ACTIVATION via the CLASSICAL COMPLEMENT PATHWAY.Receptors, Complement: Molecules on the surface of some B-lymphocytes and macrophages, that recognize and combine with the C3b, C3d, C1q, and C4b components of complement.Complement C3-C5 Convertases: Serine proteases that cleave COMPLEMENT C3 into COMPLEMENT C3A and COMPLEMENT C3B, or cleave COMPLEMENT C5 into COMPLEMENT C5A and COMPLEMENT C5B. These include the different forms of C3/C5 convertases in the classical and the alternative pathways of COMPLEMENT ACTIVATION. Both cleavages take place at the C-terminal of an ARGININE residue.Complement Pathway, Classical: Complement activation initiated by the binding of COMPLEMENT C1 to ANTIGEN-ANTIBODY COMPLEXES at the COMPLEMENT C1Q subunit. This leads to the sequential activation of COMPLEMENT C1R and COMPLEMENT C1S subunits. Activated C1s cleaves COMPLEMENT C4 and COMPLEMENT C2 forming the membrane-bound classical C3 CONVERTASE (C4B2A) and the subsequent C5 CONVERTASE (C4B2A3B) leading to cleavage of COMPLEMENT C5 and the assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.Complement C9: A 63-kDa serum glycoprotein encoded by gene C9. Monomeric C9 (mC9) binds the C5b-8 complex to form C5b-9 which catalyzes the polymerization of C9 forming C5b-p9 (MEMBRANE ATTACK COMPLEX) and transmembrane channels leading to lysis of the target cell. Patients with C9 deficiency suffer from recurrent bacterial infections.Complement Inactivator Proteins: Serum proteins that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host. The complement system is tightly regulated by inactivators that accelerate the decay of intermediates and certain cell surface receptors.Complement Membrane Attack Complex: A product of COMPLEMENT ACTIVATION cascade, regardless of the pathways, that forms transmembrane channels causing disruption of the target CELL MEMBRANE and cell lysis. It is formed by the sequential assembly of terminal complement components (COMPLEMENT C5B; COMPLEMENT C6; COMPLEMENT C7; COMPLEMENT C8; and COMPLEMENT C9) into the target membrane. The resultant C5b-8-poly-C9 is the "membrane attack complex" or MAC.Complement C3a: The smaller fragment generated from the cleavage of complement C3 by C3 CONVERTASE. C3a, a 77-amino acid peptide, is a mediator of local inflammatory process. It induces smooth MUSCLE CONTRACTION, and HISTAMINE RELEASE from MAST CELLS and LEUKOCYTES. C3a is considered an anaphylatoxin along with COMPLEMENT C4A; COMPLEMENT C5A; and COMPLEMENT C5A, DES-ARGININE.Amino Acid Sequence: The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.Complement Activating Enzymes: Enzymes that activate one or more COMPLEMENT PROTEINS in the complement system leading to the formation of the COMPLEMENT MEMBRANE ATTACK COMPLEX, an important response in host defense. They are enzymes in the various COMPLEMENT ACTIVATION pathways.Properdin: A 53-kDa protein that is a positive regulator of the alternate pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It stabilizes the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb) and protects it from rapid inactivation, thus facilitating the cascade of COMPLEMENT ACTIVATION and the formation of MEMBRANE ATTACK COMPLEX. Individuals with mutation in the PFC gene exhibit properdin deficiency and have a high susceptibility to infections.Peptide Fragments: Partial proteins formed by partial hydrolysis of complete proteins or generated through PROTEIN ENGINEERING techniques.Complement C3d: A 302-amino-acid fragment in the alpha chain (672-1663) of C3b. It is generated when C3b is inactivated (iC3b) and its alpha chain is cleaved by COMPLEMENT FACTOR I into C3c, and C3dg (955-1303) in the presence COMPLEMENT FACTOR H. Serum proteases further degrade C3dg into C3d (1002-1303) and C3g (955-1001).Molecular Sequence Data: Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.Receptors, Complement 3b: Molecular sites on or in some B-lymphocytes and macrophages that recognize and combine with COMPLEMENT C3B. The primary structure of these receptors reveal that they contain transmembrane and cytoplasmic domains, with their extracellular portion composed entirely of thirty short consensus repeats each having 60 to 70 amino acids.Complement Hemolytic Activity Assay: A screening assay for circulating COMPLEMENT PROTEINS. Diluted SERUM samples are added to antibody-coated ERYTHROCYTES and the percentage of cell lysis is measured. The values are expressed by the so called CH50, in HEMOLYTIC COMPLEMENT units per milliliter, which is the dilution of serum required to lyse 50 percent of the erythrocytes in the assay.Complement Inactivating Agents: Compounds that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host.Hemolytic-Uremic Syndrome: 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.Complement C1: The first complement component to act in the activation of CLASSICAL COMPLEMENT PATHWAY. It is a calcium-dependent trimolecular complex made up of three subcomponents: COMPLEMENT C1Q; COMPLEMENT C1R; and COMPLEMENT C1S at 1:2:2 ratios. When the intact C1 binds to at least two antibodies (involving C1q), C1r and C1s are sequentially activated, leading to subsequent steps in the cascade of COMPLEMENT ACTIVATION.Complement C4b: The large fragment formed when COMPLEMENT C4 is cleaved by COMPLEMENT C1S. The membrane-bound C4b binds COMPLEMENT C2A, a SERINE PROTEASE, to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).Positive Transcriptional Elongation Factor B: 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.Receptor, Anaphylatoxin C5a: A G-protein-coupled receptor that signals an increase in intracellular calcium in response to the potent ANAPHYLATOXIN peptide COMPLEMENT C5A.Complement C6: A 105-kDa serum glycoprotein with significant homology to the other late complement components, C7-C9. It is a polypeptide chain cross-linked by 32 disulfide bonds. C6 is the next complement component to bind to the membrane-bound COMPLEMENT C5B in the assembly of MEMBRANE ATTACK COMPLEX. It is encoded by gene C6.Complement C3c: A 206-amino-acid fragment in the alpha chain (672-1663) of C3b. It is generated when C3b is inactivated (iC3b) and its alpha chain is cleaved by COMPLEMENT FACTOR I into C3c (749-954), and C3dg (955-1303) in the presence COMPLEMENT FACTOR H.Complement C7: A 93-kDa serum glycoprotein encoded by C7 gene. It is a polypeptide chain with 28 disulfide bridges. In the formation of MEMBRANE ATTACK COMPLEX; C7 is the next component to bind the C5b-6 complex forming a trimolecular complex C5b-7 which is lipophilic, resembles an integral membrane protein, and serves as an anchor for the late complement components, C8 and C9.Complement Pathway, Mannose-Binding Lectin: 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.Antigens, CD55: GPI-linked membrane proteins broadly distributed among hematopoietic and non-hematopoietic cells. CD55 prevents the assembly of C3 CONVERTASE or accelerates the disassembly of preformed convertase, thus blocking the formation of the membrane attack complex.Complement C1 Inhibitor Protein: An endogenous 105-kDa plasma glycoprotein produced primarily by the LIVER and MONOCYTES. It inhibits a broad spectrum of proteases, including the COMPLEMENT C1R and the COMPLEMENT C1S proteases of the CLASSICAL COMPLEMENT PATHWAY, and the MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES. C1-INH-deficient individuals suffer from HEREDITARY ANGIOEDEMA TYPES I AND II.Complement C8: A 150-kDa serum glycoprotein composed of three subunits with each encoded by a different gene (C8A; C8B; and C8G). This heterotrimer contains a disulfide-linked C8alpha-C8gamma heterodimer and a noncovalently associated C8beta chain. C8 is the next component to bind the C5-7 complex forming C5b-8 that binds COMPLEMENT C9 and acts as a catalyst in the polymerization of C9.Databases, Protein: Databases containing information about PROTEINS such as AMINO ACID SEQUENCE; PROTEIN CONFORMATION; and other properties.Internet: 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.User-Computer Interface: The portion of an interactive computer program that issues messages to and receives commands from a user.Software: Sequential operating programs and data which instruct the functioning of a digital computer.Proteins: 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.Sequence Analysis, 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.
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 ...
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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?
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 ...
SR GROUP - Exporter, Importer, Manufacturer, Distributor, Supplier, Trading Company of Rat CFH(Complement Factor H) ELISA Kit based in Delhi, India
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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; ...
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。
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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 ...
... 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。
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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... is an interesting technique that complements gene transfection and RNA interference for studying protein function and cellular pathways.
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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 = ...
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 ...
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. ...
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. ...
highlighting the link between complement gene expression and IQ, in both schizophrenic patients and healthy controls. The study demonstrated a broad association between variations in complement gene expression and perturbed neurodevelopment. We were particularly interested to see that the final sentence of the paper stated that When complement gene-sets are taken as a whole, their relevance is to neurodevelopment, not illness . Their conclusion rightly touches on the emerging understanding of non-immune roles for complement in diverse biological processes (Hawksworth et al., 2018) .The complement group of proteins is ancient in evolutionary terms. Recognised complement components are found strewn throughout the animal kingdom including in rudimentary diploblastic species (Kimura et al., 2009) . In the human adult, complement functions as an essential pillar of the innate immune system. It is activated by various noxious stimuli and results in the production of the anaphylatoxins and membrane attack
Purpose.: Increasing evidence supports a role for complement in the pathogenesis of age-related macular degeneration (AMD). This study evaluated retinal microglia, T-lymphocytes, and complement deposition in a light-induced retinopathy model. The effect of a serotonin (5-hydroxytryptamine, 5-HT1A) agonist on these processes was investigated. Methods.: Rats were dark adapted for 24 hours before a 6-hour blue light exposure. Some animals were predosed subcutaneously with AL-8309A. Retinas were evaluated at different times after light exposure. Paraffin sections were stained with antibody for a microglial marker (Iba1), a T-lymphocyte marker (CD3), and complement components C1q, C3, factor B, factor H, and membrane attack complex (MAC). Results.: Light exposure resulted in substantial photoreceptor and RPE loss. Robust microglia activation and migration to the outer retina occurred rapidly. Substantial T-lymphocyte recruitment did not occur. Complement alternative pathway was strongly activated, ...
The complement system is a major humoral component of vertebrate defenses for tagging and killing target microorganisms. Recent molecular analyses have uncovered a striking feature of bony fish complement, namely that several complement components are encoded by multiple genes. In this review, the structural diversity of C3, C4, C5, factor B, C2, C1r/s and MASP are discussed with special reference to their functional differentiation, mainly focusing on the common carp (Cyprinus carpio), a tetraploidized teleost. In carp, all the members (C3, C4, C5 and a non-complement protein alpha2-macroglobulin) of the thioester-containing protein family are present in multiple isotypes, differing in the primary structures of various functional sites. Three factor B/C2-like isotypes identified in carp showed distinct expression pattern (sites and inducibility), with one behaving as an acute-phase reactant. Two C1r/C1s/MASP2-like isotypes also contain an amino acid substitution that likely affects their substrate
Author Summary Staphylococcus aureus is an important human commensal, present permanently in the noses of about 20% of the population and representing a significant risk factor for infection. The host and bacterial factors that facilitate nasal colonisation remain to be fully characterised. S. aureus adheres to the squamous epithelial cells found in the nose. Proteins expressed on the surface of S. aureus, including clumping factor B (ClfB), are responsible for this interaction. We demonstrate that loricrin, a major component of the squamous epithelial cell envelope, represents the primary ligand for ClfB and that the interaction between ClfB and loricrin is required for efficient nasal colonisation by S. aureus. Using purified proteins we have demonstrated that ClfB binds loricrin and propose a mechanism by which this binding occurs. We have established a murine model of S. aureus nasal colonisation and have demonstrated reduced colonisation in loricrin-deficient mice compared to wild-type mice which
Acts as complement inhibitor by disrupting the formation of the classical C3 convertase. Isoform 3 inhibits the classical complement pathway, while membrane-bound isoform 1 inhibits deposition of C3b via both the classical and alternative complement pathways.
By Brenden Schild In Portfolio Louisville, Kentucky - August 21st, 2013 - Rock Spring Ventures has increased its investment in Apellis Pharmaceuticals Inc., an early-stage biotechnology company focused on developing a novel pipeline of anti-inflammatory products through a combination of in-licensing of preclinical compounds and in-house research and development.. Apellis aims to bring a new class of anti-inflammatory drugs to the market to address major diseases by exploring novel mechanisms to modify these diseases by inhibiting complement activation. Apellis currently has two programs centered on the use of APL-1 in extra-ocular indications. APL-1 is a derivative of the cyclic peptide Compstatin, discovered at the University of Pennsylvania. APL-1 is a small cyclic peptide that binds to human complement factor C3 and prevents its activation, resulting in broad and potent complement activation inhibition. APL-1 is also known as POT-4, originally developed by Potentia Pharmaceuticals.. As an ...
Successful with- drawal of oral long-acting nitrates to facilitate phos- phodiesterase type 5 inhibitor use in stable coronary disease patients buy viagra at store 918540 erectile dysfunction. Hyperconsumption indicates activation of the alternative complement pathway. Malbran E, Dodds R, Hulsbus R Traumatic retinal detachment. Cell 1980; 19821.
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Alternative pathway definition, the activation of complement by contact with polysaccharides on bacteria, protozoa, or yeast cells: a nonspecific immune response. See more.
AccessGUDID - Anti Human Properdin FITC (B1761082)- Fluorescein conjugated polyclonal goat antiserum to Human Complement Properdin
Mouse Factor D ELISA Kit is a sensitive (0.03 ng/ml) immunoassay suitable for the quantification of Factor D in Cell culture supernatant, Urine, Serum, Plasma samples.
Complete information for ATP5S gene (Protein Coding), ATP Synthase, H+ Transporting, Mitochondrial Fo Complex Subunit S (Factor B), including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene Compendium
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Yeast RNA polymerase II initiation factor b copurifies with three polypeptides of 85, 73, and 50 kilodaltons and with a protein kinase that phosphorylates the carboxyl-terminal repeat domain (CTD) of the largest polymerase subunit. The gene that encodes the 73-kilodalton polypeptide, designated TFB1, was cloned and found to be essential for cell growth. The deduced protein sequence exhibits no similarity to those of protein kinases. However, the sequence is similar to that of the 62-kilodalton subunit of the HeLa transcription factor BFT2, suggesting that this factor is the human counterpart of yeast factor b. Immunoprecipitation experiments using antibodies to the TFB1 gene product demonstrate that the transcriptional and CTD kinase activities of factor b are closely associated with an oligomer of the three polypeptides. Photoaffinity labeling with 3-O-(4-benzoyl)benzoyl-ATP (adenosine triphosphate) identified an ATP-binding site in the 85-kilodalton polypeptide, suggesting that the ...
Saliva of the blood feeding sand fly Lutzomyia longipalpis was previously shown to inhibit the alternative pathway (AP) of the complement system. Here, we have identified Lufaxin, a protein component in saliva, as the inhibitor of the AP. Lufaxin inhibited the deposition of C3b, Bb, Properdin, C5b and C9b on agarose-coated plates in a dose dependent manner. It also inhibited the activation of factor B in normal serum, but had no effect on the components of the membrane attack complex. Surface plasmon resonance (SPR) experiments demonstrated that Lufaxin stabilizes the C3b-B proconvertase complex when passed over a C3b surface in combination with factor B. Lufaxin was also shown to inhibit the activation of factor B by factor D in a reconstituted C3b-B, but did not inhibit the activation of C3 by reconstituted C3b-Bb. Proconvertase stabilization does not require the presence of divalent cations, but addition of Ni2+ increases the stability of complexes formed on SPR surfaces. Stabilization of the C3b-B
The NGF (nerve growth factor) from Naja sputatrix has been purified by gel filtration followed by reversed-phase HPLC. The protein showed a very high ability to induce neurite formation in PC12 cells relative to the mouse NGF. Two cDNAs encoding isoforms of NGF have been cloned and an active recombinant NGF, sputa NGF, has been produced in Escherichia coli as a His-tagged fusion protein. Sputa NGF has been found to be non-toxic under both in vivo and in vitro conditions. The induction of neurite outgrowth by this NGF has been found to involve the high-affinity trkA-p75NTR complex of receptors. The pro-survival mechanism of p75NTR has been mediated by the activation of nuclear factor κB gene by a corresponding down-regulation of inhibitory κB gene. Real-time PCR and protein profiling (by surface-enhanced laser-desorption-ionization time-of-flight) have confirmed that sputa NGF up-regulates the expression of the endogenous NGF in PC12 cells. Preliminary microarray analysis has also shown that ...
The surface plasmon resonance (SPR) phenomenon is utilized in a number of new real time biosensors. In this study, we have used this technique to study interactions between the central complement component C3b and its multiple ligands by using the Biacore equipment. The SPR technique is particularly suitable for analysis of the alternative complement pathway (AP) because the inherent nature of the latter is to amplify deposition of C3b on various surfaces. C3b was coupled onto the sensor surface and the coupling efficiency was compared under various conditions on both polystyrene and carboxymethylated dextran surfaces. After enzymatic C3b coupling or standard amine C3b coupling, we analyzed and compared the binding of four C3b ligands to the surface: factor B, factor H, C5 and the soluble complement receptor 1 (sCR1, CD35). Binding of each ligand to C3b was detected when C3b had been coupled either enzymatically or using the amine coupling, but the half-lives of the interactions were found to ...
The complement system provides a fundamental component of the body's immune response to invading microorganisms. This chapter highlights the various roles of the complement system in the orchestration of the immune response towards microbial infections, gives examples of microbial strategies to evade complement-mediated clearance, and discusses how acquired and inherited complement deficiencies may predispose an organism to infectious disease. Complement is activated by three pathways: the classical pathway, the alternative pathway, and the lectin pathway. The lectin pathway is activated by carbohydrate recognition molecules that bind to polysaccharide on the surface of a pathogen. Factor B, factor D, and properdin (factor P) are specific components of the alternative pathway of complement activation. The complement activation is tightly regulated by membrane-bound and fluid-phase regulatory components to avoid runaway activation of the enzymatic cascade that could lead to excess host tissue damage
Interleukin-6 (IL6) is a multifunctional cytokine that regulates immune and inflammatory responses. Multiple transcription factors, including nuclear factor κB (NF-κB) and nuclear factor E2-related factor 2 (Nrf2), regulate IL6 transcription. Kelch-like ECH-associated protein 1 (Keap1) is a substrate adaptor protein for the Cullin 3-dependent E3 ubiquitin ligase complex, which regulates the degradation of many proteins, including Nrf2 and IκB kinase β (IKKβ). Here, we found that stable knockdown of Keap1 (Keap1-KD) in RAW 264.7 (RAW) mouse macrophages and human monocyte THP-1 cells significantly increased expression of Il6, and Nrf2-target genes, under basal and lipopolysaccharide (LPS, 0.001-0.1 μg/ml)-challenged conditions. However, Nrf2 activation alone, by tert-butylhydroquinone treatment of RAW cells, did not increase expression of Il6. Compared to cells transduced with scrambled non-target negative control shRNA, Keap1-KD RAW cells showed enhanced protein levels of IKKβ and ...
Complement component 4B (Chido blood group) is a protein that in humans is encoded by the C4B gene.[1] This gene encodes the basic form of complement factor 4, part of the classical activation pathway. The protein is expressed as a single chain precursor which is proteolytically cleaved into a trimer of alpha, beta, and gamma chains prior to secretion. The trimer provides a surface for interaction between the antigen-antibody complex and other complement components. The alpha chain may be cleaved to release C4 anaphylatoxin, a mediator of local inflammation. Deficiency of this protein is associated with systemic lupus erythematosus. This gene localizes to the major histocompatibility complex (MHC) class III region on chromosome 6. Varying haplotypes of this gene cluster exist, such that individuals may have 1, 2, or 3 copies of this gene. In addition, this gene exists as a long form and a short form due to the presence or absence of a 6.4 kb endogenous HERV-K retrovirus in intron 9. [provided by ...
This gene encodes a plasma glycoprotein that positively regulates the alternative complement pathway of the innate immune system. This protein binds to many microbial surfaces and apoptotic cells and stabilizes the C3- and C5-convertase enzyme complexes in a feedback loop that ultimately leads to formation of the membrane attack complex and lysis of the target cell. Mutations in this gene result in two forms of properdin deficiency, which results in high susceptibility to meningococcal infections. Multiple alternatively spliced variants, encoding the same protein, have been identified.[provided by RefSeq, Feb 2009 ...
Anti-CFI / Complement Factor I Antibody (Internal), Rabbit Anti Human Polyclonal Antibody validated in WB, IHC-P (ALS17749), Abgent
C3 glomerulonephritis is a recently described entity which is due to dysregulation in the alternative complement pathway. Patients typically present with hematuria and/or proteinuria in the face of persistently low serum levels of C3. The annual incidence of biopsy-proven disease is 1 to 2 per million with both sexes affected equally. The median age of diagnosis is 21 years of age, but there is a second spike after the age of 50 due to paraprotein-associated disease. The most common glomerular disease pattern is a membranoproliferative pattern. The hallmark of the disease is dominant C3 staining on immunofluorescence which is defined ...
The alternative pathway is continuously activated at a low level, analogous to a car engine at idle, as a result of spontaneous C3 hydrolysis due to the breakdown of the internal thioester bond (C3 is mildly unstable in aqueous environment). The alternative pathway does not rely on pathogen-binding antibodies like the other pathways.[2] C3b that is generated from C3 by a C3 convertase enzyme complex in the fluid phase is rapidly inactivated by factor H and factor I, as is the C3b-like C3 that is the product of spontaneous cleavage of the internal thioester. In contrast, when the internal thioester of C3 reacts with a hydroxyl or amino group of a molecule on the surface of a cell or pathogen, the C3b that is now covalently bound to the surface is protected from factor H-mediated inactivation. The surface-bound C3b may now bind factor B to form C3bB. This complex in the presence of factor D will be cleaved into Ba and Bb. Bb will remain associated with C3b to form C3bBb, which is the alternative ...
From NCBI Gene:. This gene encodes a member of the S1, or chymotrypsin, family of serine peptidases. This protease catalyzes the cleavage of factor B, the rate-limiting step of the alternative pathway of complement activation. This protein also functions as an adipokine, a cell signaling protein secreted by adipocytes, which regulates insulin secretion in mice. Mutations in this gene underlie complement factor D deficiency, which is associated with recurrent bacterial meningitis infections in human patients. Alternative splicing of this gene results in multiple transcript variants. At least one of these variants encodes a preproprotein that is proteolytically processed to generate the mature protease. [provided by RefSeq, Nov 2015]. From UniProt: ...
Envisioning A Cure For AMD (by Rick Trevino, OD). I would like to quote from an article by Dr. Rick Travino, who is active in our online community and who hosts one of the most informative web sites about vision research and developments. In his commentary, "Envisioning A Cure For AMD," he wrote:. "One of the most exciting developments in the field of AMD research has been the discovery of a relatively small number of genes that seems to control a large amount of the risk of developing the disease.. "Most of the genes that are known to influence the risk of developing AMD involve various components of the complement cascade. Most prominent among these is complement factor H (CFH); however, variants in genes coding for other components of the complement system have also been discovered and shown to be associated with AMD risk and protection. The complement system is very important in regulating the bodys immune system and directing inflammatory processes. Several lines of evidence suggest that ...
An external mixer assembly is provided which externally mixes and delivers a first and a second component of a biological adhesive to tissues or organs for sealing wounds, stopping bleeding and the like. The first and second components are mixed immediately after exiting from separate outlet ports disposed in fluid communication with component reservoirs. In on embodiment, the external mixer assembly includes a housing having a housing head for enclosing therein a first reservoir containing the first component, and a second reservoir containing the second component. The housing further includes a discharge nozzle defining a longitudinal axis for enclosing therein a conduit assembly having a first and a second conduit in communication with the first and second reservoir, respectively. A deflector assembly is connected to the discharge nozzle. The deflector assembly includes a deflector plate to provide a space for initial mixing of the first and second components. The deflector plate is oriented in
Inhibition of Delta pH pathway protein transport by antibodies to Hcf106. (A) Maize thylakoids were preincubated with 0.1 mg/ml anti-Hcf106 or preimmune (PI) Ig
Complete information for CFI gene (Protein Coding), Complement Factor I, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene Compendium
Engaging math & science practice! Improve your skills with free problems in Alternative Pathways for Photosynthesis and thousands of other practice lessons.
TY - JOUR. T1 - Synthesis of complement factor H by retinal pigment epithelial cells is down-regulated by oxidized photoreceptor outer segments. AU - Chen, Mei. AU - Forrester, John Vincent. AU - Xu, Heping. PY - 2007/4. Y1 - 2007/4. N2 - Complement activation is thought to be involved in the pathogenesis of age-related macular degeneration (AMD), in part because certain gene polymorphisms in complement factor H (CFH), an important regulator of the alternative complement activation pathway, are high risk factors for AMD. How CFH is regulated locally at the retina/choroid interface and how this contributes to AMD development remain unknown. In the present study, we have confirmed that CFH was detectable by immunohistochemistry in the choroid, and at low levels in the RPE cell and interphotoreceptor matrix, but appeared to be concentrated in dense patches in Bruchs membrane. In vitro, cultured human and mouse RPE cells expressed high levels of CFH as evidenced by immunohistochemistry and western ...
Kőhalmi, Kinga Viktória and Veszeli, Nóra and Luczay, Andrea and Varga, Lilian and Farkas, Henriette (2017) A danazolkezelés hatása C1-inhibitor-hiány okozta hereditaer angiooedemás gyermekek növekedésére , Effect of danazol treatment on growth in pediatric patients with hereditary angioedema due to C1-inhibitor deficiency. Orvosi Hetilap, 158 (32). pp. 1269-1276. ISSN 0030-6002 Csuka, Dorottya and Veszeli, Nóra and Varga, Lilian and Prohászka, Zoltán and Farkas, Henriette (2017) The role of the complement system in hereditary angioedema. Molecular Immunology, 89. pp. 59-68. ISSN 0161-5890 Horváth, Zsófia and Csuka, Dorottya and Vargova, Katarina and Kovács, Andrea and Lee, Sarolta and Varga, Lilian and Prohászka, Zoltán and Kiss, Róbert Gábor and Préda, István and Tóth Zsámboki, Emese (2016) Alternative complement pathway activation during invasive coronary procedures in acute myocardial infarction and stable angina pectoris. CLINICA CHIMICA ACTA, 463. pp. 138-144. ISSN ...
Complement factor C3, recently found to contain covalently bound phosphate, was phosphorylated in vitro by cyclic AMP-dependent protein kinase (protein kinase A) and Ca2+-activated, phospholipid-dependent protein kinase (protein kinase C). Both protein kinases phosphorylated the same serine residue(s) located in the C3a portion of the alpha-chain. In addition, protein kinase C phosphorylated the beta-chain to a lesser extent. Protein kinase A gave a maximal incorporation of 1 mol of phosphate/mol of C3 while that value with protein kinase C was 1.5 mol of phosphate/mol of C3. The velocity in pmol of [32P]phosphate/(min x unit kinase) was 20 times higher for protein kinase C than for protein kinase A although a 10 times lower ratio of protein kinase to C3 was used in the former case. The apparent Kmfor C3 was 2.6 µM when protein kinase C was used. The phosphorylated C3 was found to be more resistant to partial degradation by trypsin than unphosphorylated C3. It was also found that ...
Complement pathways function to identify and remove pathogens and infected cells. There are three complement pathways: the classical, lectin and alternative pathway (AP). While all pathways are activated following pathogen stimuli, the AP is constitutively active and tightly controlled by activators (e.g., Factor B, Factor D) and negative regulators (e.g., Factor H). Complement activity can be measured by well-established methods that are often used in a diagnostic setting to determine the CH50 (50% complement hemolytic activity) or AP50, specifically to measure AP activity. The protocol here has adapted the traditional AP50 method designed to measure AP activity in human sera, to measure the positive or negative AP regulatory activity within a given test sample. The assay relies on the ability of AP components in human serum to lyse rabbit erythrocytes under in vitro conditions specific for the AP with subsequent release of hemoglobin that is quantitated by measurement of optical density. Our method
Results of studies published since 2002 reveal that T cells and antigen-presenting cells (APCs) produce complement proteins. The immune cell-derived, alternative pathway complement components activate
Age-related macular degeneration (AMD) and Alzheimer disease (AD) are both neurodegenerative diseases of aging, with loss of photoreceptors and CNS neurons, respectively. A number of recent studies have shown that polymorphisms of several complement proteins in the alternative pathway of complement activation (CFB, C2, and CFH) enhance susceptibility to AMD. Somewhat similarly, in AD there is a profound increase in the levels of the initiating protein of the complement cascade called C1q, a prominent upregulation of which has also recently been reported to accompany glaucoma, which is a neurodegenerative retinal disease of aging. All of these changes, in AMD and AD, ultimately lead to activation of the pivotal complement protein called C3. Upon activation, C3 is fragmented into several pieces. One is called C3a, a small cytokine-like molecule that activates microglia and stimulates angiogenesis, whereas a larger fragment, called C3b, opsonizes the cell or debris where it was generated, leading ...
Takematsu et al reported that disruption of the epidermal wall of Henderson-Paterson bodies induces acute inflammatory changes by activation of the alternative complement pathway on exposure to the ti... more
Application Index: Complement Proteins offered by Sigma-alderich online.The complement system is a complex cascade involving proteolytic cleavage of serum glycoproteins often activated by cell receptors. This cascade ultimately results in induction of the inflammatory response, phagocyte chemotaxis and opsonization, and cell lysis.
aHUS patients raised the question " are the predisposing genetic factors of aHUS fully catalogued?" as a topic of research which matters to them in their Global Research Agenda.. Those affected by aHUS know well that it is imperfections in components of the Complement System that made them susceptible to the disease when one of many "triggering hits" over their lives caused a catastrophic onset of aHUS.. They know that there are different imperfections in different aHUS patients, some not yet found. But how many and who is keeping a record of what to look for as an aHUS "susceptibility imperfection" , or "mutation" or "significant variant".. At University College London, the Department of Structural and Molecular Biology has been collating variants in the Complement System for 15 years and creating a database of information about them. It is known as the Database of Complement Gene Variants and can seen online , click here.. Designed for use by scientist and clinicians the information held is ...
An apparatus and method for implanting a prosthesis includes implanting a first component into a recess in a bone. The first component defines a main body defining a receiving portion and a locating bore. A second component is located into engagement with the first component, the second component defining a passage therethrough. A rod is inserted through the passage defined on the second component and into the locating bore of the first component. A handle associated with the rod is slidably actuated into contact with the second component to matingly lock the first component to the second component.
Kossmann, T., Stahel, P.F., Morganti-Kossmann, M.C., Jones, J.L. and Barnum, S.R. (1997) Elevated levels of the complement components C3 and factor B in ventricular cerebrospinal fluid of patients with traumatic brain injury. Jo- urnal of Neuroimmunology, 73, 63-69.
For performing VDRL of serum , we heat serum to inactivate complement proteins which may otherwise interfere , but why dont we do same for CSF even though it too has complement proteins in it?. Is it because of lesser stability of WBCs in CSF( which is hypotonic) and on heating may rupture to release cardiolipin? But I cant comprehend how its worse than having complement proteins?. ...
Maus Monoklonal Complement Factor H Antikörper für ELISA, WB. Jetzt diesen anti-Complement Factor H Antikörper bestellen. | Produkt ABIN4264777
Swanson, J., Van Dorn, R., Swartz, M., Smith, A., Elbogen, E., & Monahan, J. (2008). Alternative pathways to violence in persons with schizophrenia: The role of childhood conduct problems. Law and Human Behavior, 32(3), 228 - 240 ...
Factor I antibody [OX-21] (complement factor I) for ELISA, IP, RIA, WB. Anti-Factor I mAb (GTX41626) is tested in Human samples. 100% Ab-Assurance.
This is an interesting follow-up study from Lukiws group on the possible role of microRNAs in AD. Here, the authors identify a novel regulatory pathway (NFκB , miR-146a , complement factor H) potentially involved in the inflammation response in AD brain. Hence, apart from modulating directly BACE1 and Aβ levels (as shown by us and Peter Nelsons group), the microRNA network is emerging as a potential important contributor to AD pathology.. The findings presented in this paper originate from the observation that miR-146a levels are elevated in AD brain. Whether these changes in miR-146a are confirmed in an independent set of patient samples remains to be seen. Indeed, current studies in humans suggest no or minimal overlap in changes in microRNA expression profiles in AD brain. While this does not exclude a role for microRNAs in neurodegeneration-which is clearly supported by studies in animals-it is important to take into consideration the experimental variables which may explain these ...
Read about how combining imaging data of MS patients eyes with genetic analysis uncovered immune complement genes linked to disease progression.
The p53 pathway is composed of genes and their products that are targeted to respond to stress signals. More than seven negative and three positive feedback loops in the p53 pathway have identified.
And 1.0 M KCL) . The observations provided an interesting possibility that additional inputs into Pbs2 may exist . To identify the alternative pathway, we
... Exp Neurol. 2017 Jun 07;: Authors: Wyatt SK, Witt T, Barbaro NM, Cohen-Gadol AA, Brewster AL Abstract Microglia-mediated neuroinflammation is widely associated with seizures and epilepsy. Although microglial cells are professional phagocytes, less is known about th...
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 ...
It binds to complement factor D (CFD). Enrollment in Phase 3 clinical trials was initiated in 2014. Phase 3 trials are usually ... These two failures have called into question whether complement inhibition is a sound strategy for geographic atrophy. World ...
... 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: ...
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 ...
Díaz-Guillén MA, Rodríguez de Córdoba S, Heine-Suñer D (1999). "A radiation hybrid map of complement factor H and factor H- ... Zipfel PF, Skerka C (1994). "Complement factor H and related proteins: an expanding family of complement-regulatory proteins ... "Two additional human serum proteins structurally related to complement factor H. Evidence for a family of factor H-related ... Complement factor H-related protein 2 is a protein that in humans is encoded by the CFHR2 gene. GRCh38: Ensembl release 89: ...
"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 ...
These SNPs were located in the gene encoding complement factor H, which was an unexpected finding in the research of ARMD. The ... "Complement factor H variant increases the risk of age-related macular degeneration". Science. 308 (5720): 419-21. doi:10.1126/ ... "Complement factor H polymorphism in age-related macular degeneration". Science. 308 (5720): 385-9. doi:10.1126/science.1109557 ... In addition to the conceptual framework several additional factors enabled the GWA studies. One was the advent of biobanks, ...
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 ...
Complement factors are decreased in rheumatoid arthritis and lupus arthritis. Microscopic analysis of synovial fluid is ... Jay, GD; Britt, DE; Cha, CJ (March 2000). "Lubricin is a product of megakaryocyte stimulating factor gene expression by human ...
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 ...
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 ... A low level of adiponectin is an independent risk factor for developing: Metabolic syndrome Diabetes mellitus Lower levels of ... Renaldi O, Pramono B, Sinorita H, Purnomo LB, Asdie RH, Asdie AH (January 2009). "Hypoadiponectinemia: a risk factor for ...
"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. ...
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 ...
The genes for the complement system proteins factor H (CFH), factor B (CFB) and factor 3 (C3) are strongly associated with a ... Despriet DD, Klaver CC, Witteman JC, Bergen AA, Kardys I, de Maat MP (2006). "Complement factor H polymorphism, complement ... on chromosome 10 at location 10q26 Complement Factor B/Complement Component 2 (CFB/CC2) on chromosome 6 at 6p21.3 Polymorphisms ... Absence of the complement factor H-related genes R3 and R1 protects against AMD. Two independent studies in 2007 showed a ...
Malhotra R, Ward M, Sim RB, Bird MI (1999). "Identification of human complement Factor H as a ligand for L-selectin". Biochem. ... The molecule is composed of multiple domains: one homologous to lectins, one to epidermal growth factor, and two to the ... The embryo secretes human chorionic gonadotropin (hCG), which downregulates anti-adhesion factor, MUC-1, located on the uterine ...
"Complement Factor H Variant Increases the Risk of Age-Related Macular Degeneration". Science. 308 (5720): 419-21. doi:10.1126/ ... Some of the factors that should be considered are the level of efficacy of various genetic tests in the general population, ... "individual risk factors". Perhaps the most critical issue with the commercialization of personalised medicine is the protection ... there are a number of factors that must be considered. The detailed account of genetic information from the individual will ...
Diaz-Guillen MA, Rodriguez de Cordoba S, Heine-Suner D (Jul 1999). "A radiation hybrid map of complement factor H and factor H- ... CFHR4 complement factor H-related 4". Hageman GS, Hancox LS, Taiber AJ, et al. (2007). "Extended Haplotypes in the Complement ... Complement factor H-related protein 4 is a protein that in humans is encoded by the CFHR4 gene. GRCh38: Ensembl release 89: ... 2000). "Functional properties of complement factor H-related proteins FHR-3 and FHR-4: binding to the C3d region of C3b and ...
Opsonins include Mfge8, Gas6, Protein S, antibodies and complement factors C1q and C3b. Phagoptosis has multiple functions ... Pathogenic cells such as bacteria can be opsonised by antibodies or complement factors, enabling their phagocytosis and ...
1989). "20 KDa homologous restriction factor of complement resembles T cell activating protein". Biochem. Biophys. Res. Commun ... When complement activation leads to deposition of C5b678 on host cells, CD59 can prevent C9 from polymerizing and forming the ... Ninomiya H, Sims PJ (1992). "The human complement regulatory protein CD59 binds to the alpha-chain of C8 and to the "b"domain ... 1992). "Complement regulatory proteins at the feto-maternal interface during human placental development: distribution of CD59 ...
Lee FJ, Moss J, Vaughan M (1992). "Human and Giardia ADP-ribosylation factors (ARFs) complement ARF function in Saccharomyces ... ADP-ribosylation factor 5 is a protein that in humans is encoded by the ARF5 gene. ADP-ribosylation factor 5 (ARF5) is a member ... Shin, O H; Exton J H (August 2001). "Differential binding of arfaptin 2/POR1 to ADP-ribosylation factors and Rac1". Biochem. ... "Entrez Gene: ARF5 ADP-ribosylation factor 5". Kanoh, H; Williger B T; Exton J H (February 1997). "Arfaptin 1, a putative ...
"Entrez Gene: CFHR3 complement factor H-related 3". Zipfel PF, Skerka C (1994). "Complement factor H and related proteins: an ... Diaz-Guillen MA, Rodriguez de Cordoba S, Heine-Suner D (Jul 1999). "A radiation hybrid map of complement factor H and factor H- ... Complement factor H-related protein 3 is a protein that in humans is encoded by the CFHR3 gene. GRCh38: Ensembl release 89: ... 2000). "Complement factor H: sequence analysis of 221 kb of human genomic DNA containing the entire fH, fHR-1 and fHR-3 genes ...
Lee FJ, Moss J, Vaughan M (1992). "Human and Giardia ADP-ribosylation factors (ARFs) complement ARF function in Saccharomyces ... ADP-ribosylation factor 4 is a protein that in humans is encoded by the ARF4 gene. ADP-ribosylation factor 4 (ARF4) is a member ... "ADP-ribosylation factor 4 small GTPase mediates epidermal growth factor receptor-dependent phospholipase D2 activation". J. ... "ADP-ribosylation factor 4 small GTPase mediates epidermal growth factor receptor-dependent phospholipase D2 activation". J. ...
Complement factor H-related protein 5 (CFHR5) nephropathy (also known as Troodos nephropathy) is a form of inherited kidney ... 26 August 2010). "Identification of a mutation in complement factor H-related protein 5 in patients of Cypriot origin with ... "Recurrence of Complement Factor H-Related Protein 5 Nephropathy in a Renal Transplant". American Journal of Transplantation. 11 ... A kidney biopsy in patients with CFHR5 nephropathy classically shows deposition of complement C3 in the kidney, without any ...
Lee FJ, Moss J, Vaughan M (1992). "Human and Giardia ADP-ribosylation factors (ARFs) complement ARF function in Saccharomyces ... ADP-ribosylation factor 3 is a protein that in humans is encoded by the ARF3 gene. ADP-ribosylation factor 3 (ARF3) is a member ... Williger BT, Provost JJ, Ho WT, Milstine J, Exton JH (July 1999). "Arfaptin 1 forms a complex with ADP-ribosylation factor and ... Lee CM, Haun RS, Tsai SC, Moss J, Vaughan M (1992). "Characterization of the human gene encoding ADP-ribosylation factor 1, a ...
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 ...
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. ...
Jetzt diesen anti-Complement Factor I Antikörper bestellen. , Produkt ABIN4264851 ... Maus Monoklonal Complement Factor I Antikörper für ELISA, WB. ... anti-Complement Factor H Antikörper * anti-Complement Factor D ... Target Details Complement Factor I Handhabung Anwendungsinformationen zurück nach oben Target Details Complement Factor I ... anti-Complement Factor I Antikörper (CFI) (DyLight 755) Complement Factor I Antikörper (CFI) (DyLight 755). Details for Product ...
Structure of a complex between CCPs 6 and 7 of Human Complement Factor H and Neisseria meningitidis FHbp Variant 3 Wild type ... Structure of a complex between CCPs 6 and 7 of Human Complement Factor H and Neisseria meningitidis FHbp Variant 3 Wild type. * ... Factor H binding protein (fHbp) is a key antigen that elicits protective immunity against the meningococcus and recruits the ... Factor H binding protein (fHbp) is a key antigen that elicits protective immunity against the men ... ...
... the treatment of atypical hemolytic uremic syndrome recurrence after kidney transplantation associated with complement factor H ... Le Quintrec M, Lionet A, Kamar N, Karras A, Barbier S, Buchler M et al (2008) Complement mutation-associated de novo thrombotic ...
Complement Factor I Antibody (Internal), Rabbit Anti Human Polyclonal Antibody validated in WB, IHC-P (ALS17749), Abgent ... Complement component factor i, Factor I, FI, KAF, I factor, I factor (complement), C3b-INA, Complement factor I, Light chain of ... Anti-CFI / Complement Factor I Antibody (Internal) is for research use only and not for use in diagnostic or therapeutic ... home , Products , Primary Antibodies , Antibody Collections , GPCR Antibodies , Anti-CFI / Complement Factor I Antibody ( ...
The Correlation of Plasma Cytokines with Complement Factor H polymorphism Y402H, Choroidal Thickness and Drusen Load in Dry Age ... The Correlation of Plasma Cytokines with Complement Factor H polymorphism Y402H, Choroidal Thickness and Drusen Load in Dry Age ... including the Y402H variation in the gene encoding complement factor H (CFH), which confers a several-fold increased risk for ... The Correlation of Plasma Cytokines with Complement Factor H polymorphism Y402H, Choroidal Thickness and Drusen Load in Dry Age ...
Complement Factor I, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene ... CFI (Complement Factor I) is a Protein Coding gene. Diseases associated with CFI include Complement Factor I Deficiency and ... Pathogenic, Complement factor I deficiency (CFI deficiency) [MIM:610984]. 109,746,398(-). TTTCC(A/T)TGAAA. reference, missense ... Pathogenic, Complement factor I deficiency (CFI deficiency) [MIM:610984]. 109,760,567(-). TGATG(A/G)TATCA. reference, missense ...
keywords = "complement, complement factor H, age-related macular degeneration, retinal pigment epithelial cells, photoreceptor ... in part because certain gene polymorphisms in complement factor H (CFH), an important regulator of the alternative complement ... in part because certain gene polymorphisms in complement factor H (CFH), an important regulator of the alternative complement ... in part because certain gene polymorphisms in complement factor H (CFH), an important regulator of the alternative complement ...
Ethnic and Phenotypic Frequencies of Complement Factor H Polymorphism Y402H M.A. Grassi; J.H. Fingert; J.C. Folk; T.E. Scheetz ... Ethnic and Phenotypic Frequencies of Complement Factor H Polymorphism Y402H You will receive an email whenever this article is ... Purpose: : Previous studies have demonstrated that the Y402H polymorphism in the complement factor H (CFH) gene is associated ... Ethnic and Phenotypic Frequencies of Complement Factor H Polymorphism Y402H . Invest. Ophthalmol. Vis. Sci. 2006;47(13):4164. ...
Complement Factor H) ELISA Kit based in Delhi, India ... Complement Factor H) ELISA Kit Rat CFH(Complement Factor H) ... Bulk Request Rat GM-CSF(Granulocyte-Macrophage Colony Stimulating Factor) ELISA Kit ... Rat CFB(Complement Factor B) ELISA Kit. *Rat CFH(Complement Factor H) ELISA Kit ...
Human complement factor H Y402H polymorphism causes an age-related macular degeneration phenotype and lipoprotein dysregulation ... Human complement factor H Y402H polymorphism causes an age-related macular degeneration phenotype and lipoprotein dysregulation ... One of the strongest susceptibility genes for age-related macular degeneration (AMD) is complement factor H (CFH); however, its ... but not complement activation, which correlated with the AMD-like phenotype in old CFH-H/H mice. Specifically, apolipoproteins ...
Complement factor H (CFH) protein is an inhibitor of the alternative pathway of complement (AP) both in the fluid phase and on ... Mouse and human complement factor H-related (CFHR) proteins also belong to the fH family of plasma glycoproteins. The main goal ... Mehta G, Ferreira VP, Skerka C, Zipfel PF, Banda NK (2014) New insights into disease-specific absence of complement factor H ... New insights into disease-specific absence of complement factor H related protein C in mouse models of spontaneous autoimmune ...
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 ...
... 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: ...
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. ...
... Jean Charchaflieh,1 Julie Rushbrook,2 Samrat Worah,2 and Ming Zhang ...
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 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 ...
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 ...
  • Non-functional V1 fHbps were further characterised by binding and structural studies, and shown in non-transgenic and transgenic mice (expressing chimeric fH that binds fHbp and precisely regulates complement system) to retain their immunogenicity. (rcsb.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)
  • It is not surprising therefore that mutations or single nucleotide polymorphisms (SNPs) in factor H often result in pathologies. (wikipedia.org)
  • Instead, we show that CRP stimulates DAF expression on endothelial cells and thus may protect these cells from complement-mediated cell injury. (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 major difference in backbone structure between Factor D and the other serine proteases of the chymotrpsin family is in the surface loops connecting the secondary structural elements. (wikipedia.org)
  • CD46) and factor H with the development of atypical hemolytic uremic syndrome (aHUS) ( 9 , 10 ). (jimmunol.org)