Complement C3 Nephritic Factor: An IgG autoantibody against the ALTERNATIVE PATHWAY C3 CONVERTASE, found in serum of patients with MESANGIOCAPILLARY GLOMERULONEPHRITIS. The binding of this autoantibody to C3bBb stabilizes the enzyme thus reduces the actions of C3b inactivators (COMPLEMENT FACTOR H; COMPLEMENT FACTOR I). This abnormally stabilized enzyme induces a continuous COMPLEMENT ACTIVATION and generation of C3b thereby promoting the assembly of MEMBRANE ATTACK COMPLEX and cytolysis.Complement C3: A glycoprotein that is central in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C3 can be cleaved into COMPLEMENT C3A and COMPLEMENT C3B, spontaneously at low level or by C3 CONVERTASE at high level. The smaller fragment C3a is an ANAPHYLATOXIN and mediator of local inflammatory process. The larger fragment C3b binds with C3 convertase to form C5 convertase.Complement C4: A glycoprotein that is important in the activation of CLASSICAL COMPLEMENT PATHWAY. C4 is cleaved by the activated COMPLEMENT C1S into COMPLEMENT C4A and COMPLEMENT C4B.Complement 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 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 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 C5: C5 plays a central role in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C5 is cleaved by C5 CONVERTASE into COMPLEMENT C5A and COMPLEMENT C5B. The smaller fragment C5a is an ANAPHYLATOXIN and mediator of inflammatory process. The major fragment C5b binds to the membrane initiating the spontaneous assembly of the late complement components, C5-C9, into the MEMBRANE ATTACK COMPLEX.Complement 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 C4a: The smaller fragment formed when complement C4 is cleaved by COMPLEMENT C1S. It is an anaphylatoxin that causes symptoms of immediate hypersensitivity (HYPERSENSITIVITY, IMMEDIATE) but its activity is weaker than that of COMPLEMENT C3A or COMPLEMENT C5A.Complement 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.Complement C5 Convertase, Classical Pathway: A serine protease that cleaves multiple COMPLEMENT 5 into COMPLEMENT 5A (anaphylatoxin) and COMPLEMENT 5B in the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. It is a complex of CLASSICAL PATHWAY C3 CONVERTASE (C4b2a) with an additional COMPLEMENT C3B, or C4b2a3b.Glomerulonephritis, Membranoproliferative: Chronic glomerulonephritis characterized histologically by proliferation of MESANGIAL CELLS, increase in the MESANGIAL EXTRACELLULAR MATRIX, and a thickening of the glomerular capillary walls. This may appear as a primary disorder or secondary to other diseases including infections and autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Various subtypes are classified by their abnormal ultrastructures and immune deposits. Hypocomplementemia is a characteristic feature of all types of MPGN.Properdin: A 53-kDa protein that is a positive regulator of the alternate pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It stabilizes the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb) and protects it from rapid inactivation, thus facilitating the cascade of COMPLEMENT ACTIVATION and the formation of MEMBRANE ATTACK COMPLEX. Individuals with mutation in the PFC gene exhibit properdin deficiency and have a high susceptibility to infections.Complement C3a: The smaller fragment generated from the cleavage of complement C3 by C3 CONVERTASE. C3a, a 77-amino acid peptide, is a mediator of local inflammatory process. It induces smooth MUSCLE CONTRACTION, and HISTAMINE RELEASE from MAST CELLS and LEUKOCYTES. C3a is considered an anaphylatoxin along with COMPLEMENT C4A; COMPLEMENT C5A; and COMPLEMENT C5A, DES-ARGININE.Complement 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 C1q: A subcomponent of complement C1, composed of six copies of three polypeptide chains (A, B, and C), each encoded by a separate gene (C1QA; C1QB; C1QC). This complex is arranged in nine subunits (six disulfide-linked dimers of A and B, and three disulfide-linked homodimers of C). C1q has binding sites for antibodies (the heavy chain of IMMUNOGLOBULIN G or IMMUNOGLOBULIN M). The interaction of C1q and immunoglobulin activates the two proenzymes COMPLEMENT C1R and COMPLEMENT C1S, thus initiating the cascade of COMPLEMENT ACTIVATION via the CLASSICAL COMPLEMENT PATHWAY.Complement 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 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 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 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).Lipodystrophy: A collection of heterogenous conditions resulting from defective LIPID METABOLISM and characterized by ADIPOSE TISSUE atrophy. Often there is redistribution of body fat resulting in peripheral fat wasting and central adiposity. They include generalized, localized, congenital, and acquired lipodystrophy.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, Classical Pathway: A serine protease that cleaves multiple COMPLEMENT 3 into COMPLEMENT 3A (anaphylatoxin) and COMPLEMENT 3B in the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. It is a complex of COMPLEMENT 4B and COMPLEMENT 2A (C4b2a).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 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 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).Chloroprene: Toxic, possibly carcinogenic, monomer of neoprene, a synthetic rubber; causes damage to skin, lungs, CNS, kidneys, liver, blood cells and fetuses. Synonym: 2-chlorobutadiene.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 C9: A 63-kDa serum glycoprotein encoded by gene C9. Monomeric C9 (mC9) binds the C5b-8 complex to form C5b-9 which catalyzes the polymerization of C9 forming C5b-p9 (MEMBRANE ATTACK COMPLEX) and transmembrane channels leading to lysis of the target cell. Patients with C9 deficiency suffer from recurrent bacterial infections.Receptors, Complement: Molecules on the surface of some B-lymphocytes and macrophages, that recognize and combine with the C3b, C3d, C1q, and C4b components of complement.Complement C1s: A 77-kDa subcomponent of complement C1, encoded by gene C1S, is a SERINE PROTEASE existing as a proenzyme (homodimer) in the intact complement C1 complex. Upon the binding of COMPLEMENT C1Q to antibodies, the activated COMPLEMENT C1R cleaves C1s into two chains, A (heavy) and B (light, the serine protease), linked by disulfide bonds yielding the active C1s. The activated C1s, in turn, cleaves COMPLEMENT C2 and COMPLEMENT C4 to form C4b2a (CLASSICAL C3 CONVERTASE).Complement Membrane Attack Complex: A product of COMPLEMENT ACTIVATION cascade, regardless of the pathways, that forms transmembrane channels causing disruption of the target CELL MEMBRANE and cell lysis. It is formed by the sequential assembly of terminal complement components (COMPLEMENT C5B; COMPLEMENT C6; COMPLEMENT C7; COMPLEMENT C8; and COMPLEMENT C9) into the target membrane. The resultant C5b-8-poly-C9 is the "membrane attack complex" or MAC.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.Glomerulonephritis: Inflammation of the renal glomeruli (KIDNEY GLOMERULUS) that can be classified by the type of glomerular injuries including antibody deposition, complement activation, cellular proliferation, and glomerulosclerosis. These structural and functional abnormalities usually lead to HEMATURIA; PROTEINURIA; HYPERTENSION; and RENAL INSUFFICIENCY.Complement C1r: A 80-kDa subcomponent of complement C1, existing as a SERINE PROTEASE proenzyme in the intact complement C1 complex. When COMPLEMENT C1Q is bound to antibodies, the changed tertiary structure causes autolytic activation of complement C1r which is cleaved into two chains, A (heavy) and B (light, the serine protease), connected by disulfide bonds. The activated C1r serine protease, in turn, activates COMPLEMENT C1S proenzyme by cleaving the Arg426-Ile427 bond. No fragment is released when either C1r or C1s is cleaved.Nephritis: Inflammation of any part of the KIDNEY.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 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 C8: A 150-kDa serum glycoprotein composed of three subunits with each encoded by a different gene (C8A; C8B; and C8G). This heterotrimer contains a disulfide-linked C8alpha-C8gamma heterodimer and a noncovalently associated C8beta chain. C8 is the next component to bind the C5-7 complex forming C5b-8 that binds COMPLEMENT C9 and acts as a catalyst in the polymerization of C9.Complement C1: The first complement component to act in the activation of CLASSICAL COMPLEMENT PATHWAY. It is a calcium-dependent trimolecular complex made up of three subcomponents: COMPLEMENT C1Q; COMPLEMENT C1R; and COMPLEMENT C1S at 1:2:2 ratios. When the intact C1 binds to at least two antibodies (involving C1q), C1r and C1s are sequentially activated, leading to subsequent steps in the cascade of COMPLEMENT ACTIVATION.Receptors, Complement 3b: Molecular sites on or in some B-lymphocytes and macrophages that recognize and combine with COMPLEMENT C3B. The primary structure of these receptors reveal that they contain transmembrane and cytoplasmic domains, with their extracellular portion composed entirely of thirty short consensus repeats each having 60 to 70 amino acids.Complement C5b: The larger fragment generated from the cleavage of C5 by C5 CONVERTASE that yields COMPLEMENT C5A and C5b (beta chain + alpha' chain, the residual alpha chain, bound by disulfide bond). C5b remains bound to the membrane and initiates the spontaneous assembly of the late complement components to form C5b-8-poly-C9, the MEMBRANE ATTACK COMPLEX.Complement C2a: The COOH-terminal fragment of COMPLEMENT 2, released by the action of activated COMPLEMENT C1S. It is a SERINE PROTEASE. C2a combines with COMPLEMENT C4B to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).Receptor, Anaphylatoxin C5a: A G-protein-coupled receptor that signals an increase in intracellular calcium in response to the potent ANAPHYLATOXIN peptide COMPLEMENT C5A.Autoantibodies: Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.Immunoglobulin G: The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B.Chondroitin ABC Lyase: An enzyme that catalyzes the eliminative degradation of polysaccharides containing 1,4-beta-D-hexosaminyl and 1,3-beta-D-glucuronosyl or 1,3-alpha-L-iduronosyl linkages to disaccharides containing 4-deoxy-beta-D-gluc-4-enuronosyl groups. (Enzyme Nomenclature, 1992)Complement C1 Inactivator Proteins: Serum proteins that inhibit, antagonize, or inactivate COMPLEMENT C1 or its subunits.Receptors, Complement 3d: Molecular sites on or in B-lymphocytes, follicular dendritic cells, lymphoid cells, and epithelial cells that recognize and combine with COMPLEMENT C3D. Human complement receptor 2 (CR2) serves as a receptor for both C3dg and the gp350/220 glycoprotein of HERPESVIRUS 4, HUMAN, and binds the monoclonal antibody OKB7, which blocks binding of both ligands to the receptor.Anaphylatoxins: Serum peptides derived from certain cleaved COMPLEMENT PROTEINS during COMPLEMENT ACTIVATION. They induce smooth MUSCLE CONTRACTION; mast cell HISTAMINE RELEASE; PLATELET AGGREGATION; and act as mediators of the local inflammatory process. The order of anaphylatoxin activity from the strongest to the weakest is C5a, C3a, C4a, and C5a des-arginine.Complement Fixation Tests: Serologic tests based on inactivation of complement by the antigen-antibody complex (stage 1). Binding of free complement can be visualized by addition of a second antigen-antibody system such as red cells and appropriate red cell antibody (hemolysin) requiring complement for its completion (stage 2). Failure of the red cells to lyse indicates that a specific antigen-antibody reaction has taken place in stage 1. If red cells lyse, free complement is present indicating no antigen-antibody reaction occurred in stage 1.Complement Factor I: A plasma serine proteinase that cleaves the alpha-chains of C3b and C4b in the presence of the cofactors COMPLEMENT FACTOR H and C4-binding protein, respectively. It is a 66-kDa glycoprotein that converts C3b to inactivated C3b (iC3b) followed by the release of two fragments, C3c (150-kDa) and C3dg (41-kDa). It was formerly called KAF, C3bINF, or enzyme 3b inactivator.Complement C4b-Binding Protein: A serum protein that regulates the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. It binds as a cofactor to COMPLEMENT FACTOR I which then hydrolyzes the COMPLEMENT C4B in the CLASSICAL PATHWAY C3 CONVERTASE (C4bC2a).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.

Antiproteinuric therapy while preventing the abnormal protein traffic in proximal tubule abrogates protein- and complement-dependent interstitial inflammation in experimental renal disease. (1/32)

In proteinuric glomerulopathies, the excess traffic of proteins into the renal tubule is a candidate trigger of interstitial inflammatory and immune events leading to progressive injury, and a key target for the renoprotective action of antiproteinuric drugs. Among proteins trafficked to the proximal tubule, the third component of complement (C3) can be activated locally and contribute to inflammation at sites of protein reabsorption. Experiments were performed in rats with renal mass reduction (RMR, 5/6 nephrectomy) with the following aims: (1) to study Ig (IgG) and complement deposition in proximal tubules, and interstitial macrophage infiltration and MHC class II expression at intervals after surgery by double immunofluorescence analysis; (2) to assess whether lisinopril (angiotensin-converting enzyme inhibitor [ACEi], 25 mg/L in the drinking water, from either day 1 or day 7) limited IgG and C3 accumulation and interstitial inflammation at day 30. In 7-d remnant kidneys, intracellular staining for both IgG and C3 was detectable in proximal tubules in focal areas; C3 was restricted to IgG-positive tubular cells, and there were no interstitial ED-1 macrophage and MHC II-positive cellular infiltrates. In 14-d and 30-d remnant kidneys, proximal tubular IgG and C3 staining was associated with the appearance of interstitial infiltrates that preferentially localized to areas of tubules positive for both proteins. RMR rats given ACEi had no or limited increases in levels of urinary protein excretion, tubular IgG, and C3 reactivity, and interstitial cellular infiltrates in kidneys at 30 d, even when ACEi was started from day 7 after surgery. These findings document that (1) in RMR, IgG and C3 accumulation in proximal tubular cells is followed by leukocyte infiltration and MHC II overexpression in the adjacent interstitium; (2) ACEi while preventing proteinuria limits both tubular accumulation of IgG and C3 and interstitial inflammation. The data suggest that ACE inhibition can be renoprotective by limiting the early abnormal protein traffic in proximal tubule and consequent deleterious effects of excess protein reabsorption, including the accumulation and local activation of complement as well as the induction of chemokines and endothelin genes known to promote interstitial inflammation and fibrosis.  (+info)

Identification of nephritic factor as an immunoglobulin. (2/32)

C3 nephritic factor (C3NeF) activity in sera from three patients with mesangiocapillary glomerulonephritis, one of whom had partial lipodystrophy, was found on chromatography to be associated with fractions containing IgG and no other detectable proteins. Immunoadsorption of IgG from these fractions with a highly purified anti-IgG removed the C3NeF, and the IgG, eluted after combination with the anti-IgG, retained C3NeF activity. In each case the isolated IgG with C3NeF activity was found to contain more than one subclass of IgG and both kappa and lambda chains, indicating that the immunoglobulin comprising C3NeF in these patients is heterogeneous and not monoclonal. The identification of C3NeF as an immunoglobulin suggests that it may be an autoantibody against antigenic determinants of complement components present in the C3 convertase of the alternative pathway.  (+info)

Requirements for the production of high-titre C3 nephritic factor (NEF) antibody in vitro. (3/32)

C3 nephritic factor (NEF) is an IgG autoantibody directed against neoantigenic determinants of the alternative C3 convertase (C3b.Bb). Structural and functional studies require important amounts of this antibody, which are difficult to obtain from patients' sera. We have developed a method for increasing NEF production in vitro. Epstein-Barr virus is a herpes virus which transforms B lymphocytes. Some authors were able to induce the production of NEF in vitro after infection with Epstein-Barr virus (EBV). These works were preformed without any previous cellular selection of B cells. We have performed a method of preselecting antigen-binding cells prior to EBV transformation. Non-preselected cells yielded 0.16 U/million cells in culture (U/M) of NEF antibody, whereas enriched cells for NEF antibody in eliminated 8 U/M (sheep erythrocytes coated with anti-IgG, A, M). Specific NEF synthesis can be increased, in peripheral blood mononuclear cells (PBMC) from patients by in vitro stimulation with the antigens recognized by NEF [C3b.Bb, 21,000 MW protein from patients' E membranes and 26,000 MW protein from sheep E membranes (ShE)]. The highest stimulation is induced by the C3b.Bb and by 26,000 MW protein, 21,000 MW protein had lowest stimulatory effect. In this work also we have shown that patients having NEF antibody in sera have an increase of the CD5-CD19 subset, when compared with the controls.  (+info)

Significance of C3 nephritic factor (C3NeF) in non-hypocomplementaemic serum with membranoproliferative glomerulonephritis (MPGN). (4/32)

C3NeF is an autoantibody of C3 convertase (C3bBb) and is often detected in the serum of hypocomplementaemic MPGN patients. Serum samples from 104 non-hypocomplementaemic MPGN patients (C3NeF) were studied. C3NeF, which cannot activate the alternative pathway, was found in the sera of 6 patients. We examined the C3NeF in purified IgG from five of the non-hypocomplementaemic serum samples (non-hypo C3NeF) and four hypocomplementaemic serum samples (hypocomplementaemic C3NeF) to determine why C3NeF does not induce C3 splitting and hypocomplementaemia. Purified IgG from non-hypo C3NeF stabilized EAC4b3bBb cells in a manner similar to IgG from hypocomplementaemic C3NeF in EDTA gelatin veronal buffer. However, the non-hypo C3NeF IgG did not stabilize C3 convertase (EAC4b3bBb cells) in the presence of control proteins (factors H and I), whereas the hypocomplementaemic C3NeF IgG did. The C3NeF in the hypocomplementaemic serum displayed two characteristics: (i) inhibition of intrinsic decay of Ce convertase (C3bBb); and (ii) inhibition of extrinsic decay by factors H and I. Although the C3NeF in the non-hypocomplementaemic sera did inhibit the intrinsic decay in a manner similar to the hypocomplementaemic C3NeF IgG, it did not inhibit the extrinsic decay. Due to the different characteristics of hypocomplementaemic C3NeF and non-hypo C3NeF in the serum samples, the non-hypo C3NeF did not activate C3. Therefore, we conclude that C3NeF exhibits a heterogeneity which is very important in relation to the pathogenesis of MPGN.  (+info)

Primary glomerulonephritis with isolated C3 deposits: a new entity which shares common genetic risk factors with haemolytic uraemic syndrome. (5/32)

INTRODUCTION: Abnormal control of the complement alternative pathway (CAP) (factor H, factor I and membrane cofactor protein (MCP) deficiencies) is a well established risk factor for the occurrence of haemolytic uraemic syndrome (HUS). In some instances, HUS may be associated with an unusual glomerulonephritis with isolated C3 deposits (glomerulonephritis C3). We determined whether HUS and glomerulonephritis C3 share common genetic susceptibility factors. METHODS: We identified 19 patients with glomerulonephritis C3. We measured levels of circulating complement components, performed assays for the detection of C3 nephritic factor (C3NeF) and screened factor H, factor I and MCP coding genes for the presence of mutations. RESULTS: Patients were divided in two groups based on renal pathology findings: group I (n = 13) had typical features of type I membranoproliferative glomerulonephritis (glomerulonephritis C3 with membranoproliferative glomerulonephritis (MPGN)) and group II (n = 6) was characterised by mesangial and epimembranous C3 deposits in the absence of mesangial proliferation (glomerulonephritis C3 without MPGN). Mutations in complement regulatory genes were detected in 4/6 patients with glomerulonephritis C3 without MPGN (heterozygous mutations in factor H gene (two patients) with low factor H antigenic level in one case, heterozygous mutations in factor I gene (two patients)) and in only 2/13 patients with glomerulonephritis C3 with MPGN (heterozygous mutations in factor H gene (one patient) and double heterozygous mutation in CD 46 gene (one patient)). In contrast, C3NeF was present in 5/13 patients with glomerulonephritis C3 with MPGN and in 2/6 patients with glomerulonephritis C3 without MPGN, one of whom had a factor H mutation. CONCLUSION: HUS and glomerulonephritis C3 without MPGN share common genetic risk factors. Constitutional or acquired dysregulation of the CAP is probably associated with a wide spectrum of diseases, ranging from HUS to glomerulonephritis C3 with MPGN.  (+info)

Membranoproliferative glomerulonephritis. (6/32)

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Proliferative glomerulonephritis secondary to dysfunction of the alternative pathway of complement. (7/32)

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Causes of alternative pathway dysregulation in dense deposit disease. (8/32)

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*List of MeSH codes (D12.776)

... complement c3 nephritic factor MeSH D12.776.377.715.548.114.323.390 - immunoconglutinins MeSH D12.776.377.715.548.114.323.480 ... complement factor h MeSH D12.776.377.715.182.338 - hemopexin MeSH D12.776.377.715.182.580 - plasminogen MeSH D12.776.377.715. ... vascular endothelial growth factor a MeSH D12.776.467.100.800.300 - vascular endothelial growth factor b MeSH D12.776.467.100. ... fibroblast growth factor 4 MeSH D12.776.624.664.700.112 - fibroblast growth factor 6 MeSH D12.776.624.664.700.114 - fms-like ...

*Barraquer-Simons syndrome

... low C3 serum complement levels, and the presence of a C3 nephritic factor. C3 nephritic factor is a serum immunoglobulin G that ... C3 nephritic factor induces the lysis of adipocytes that secrete adipsin, a product identical to complement factor D. The ... Around 83% of APL patients had low complement 3 (C3) levels and the presence of polyclonal immunoglobulin C3 nephritic factor. ... due to complement activation and consumption of C3). Low C3 levels may impair complement-mediated phagocytosis and bacterial ...

*List of MeSH codes (D12.776.124)

... complement c1 inhibitor protein MeSH D12.776.124.486.274.920.287 -- complement c3 nephritic factor MeSH D12.776.124.486.274.920 ... complement factor h MeSH D12.776.124.486.274.920.325.210 -- complement factor i MeSH D12.776.124.486.274.920.662 -- complement ... complement factor d MeSH D12.776.124.486.274.900 -- complement factor b MeSH D12.776.124.486.274.920 -- complement inactivator ... complement c3 nephritic factor MeSH D12.776.124.486.485.114.323.390 -- immunoconglutinins MeSH D12.776.124.486.485.114.323.480 ...

*Glomerulonephritis

The C3 Nephritic Factor autoantibody stabilizes C3-convertase, which may lead to an excessive activation of complement. Rapidly ... The nephritic syndrome is characterised by blood in the urine (especially Red blood cell casts with dysmorphic red blood cells ... These forms usually present with a triad of blood in the urine, decreased urine production, and hypertension, the nephritic ... Type 2 MPGN, also known as Dense Deposit Disease, is characterised by an excessive activation of the complement system. ...

*M. Amin Arnaout

He was the first to show that C3 nephritic factor is an autoantibody that activates the alternative complement pathway, a ... finding that suggested the potential of B cell or complement C5 depletion as adjunct therapies in certain forms of kidney ...

*Henoch-Schönlein purpura

... and immunofluorescence demonstrates IgA and C3 (a protein of the complement system) in the blood vessel wall. However, overall ... ISBN 1-4160-2999-0. Sais G, Vidaller A, Jucglà A, Servitje O, Condom E, Peyri J (1998). "Prognostic factors in leukocytoclastic ... About 20% of children that exhibit nephrotic or nephritic features experience long permanent renal impairment. The findings on ... One of the characteristics of IgA1 (and IgD) is the presence of an 18 amino acid-long "hinge region" between complement- ...

*Membranoproliferative glomerulonephritis

Membranoproliferative_GN at Nephropathology tutorial MP GN Pathophysiology discusses the nephritic auto-antibodies/factors. ... It is believed to be associated with the classical complement pathway. The preferred name is "dense deposit disease". Most ... A 2012 review considers DDD to be in a continuum with C3 glomerulonephritis, one reason the use of type I to type III ... February 2008). "Factor I is required for the development of membranoproliferative glomerulonephritis in factor H-deficient ...
Barraquer-Simons syndrome (or acquired partial lipodystrophy, cephalothoracic lipodystrophy, and progressive lipodystrophy)) is a rare form of lipodystrophy, which usually first affects the head, and then spreads to the thorax. It is named for Luis Barraquer Roviralta (1855-1928), a Spanish physician, and Arthur Simons (1879-1942), a German physician. Some evidence links it to LMNB2. The etiology of this condition has not been fully elucidated. Lipodystrophy is often associated with glomerulonephritis, low C3 serum complement levels, and the presence of a C3 nephritic factor. C3 nephritic factor is a serum immunoglobulin G that interacts with the C3bBb alternative pathway convertase to activate C3. C3 nephritic factor induces the lysis of adipocytes that secrete adipsin, a product identical to complement factor D. The distribution of the lipoatrophy is postulated to be dictated by the variable amounts of adipsin secreted by the adipocytes at different locations. Human PTRF mutations may cause ...
Membranoproliferative glomerulonephritis (MPGN) is a relatively-rare, immune-mediated glomerular disease. There is no accepted therapy and all current therapies are inadequate. Current therapeutic options include immunosuppression with corticosteroids alone or in combination with alkylating agents, antiplatelet therapy with aspirin and/or dipyridamole and/or warfarin, and angiotensin converting enzyme inhibitors and/or angiotensin receptor blockers. As with other glomerular diseases the amount of protein in the urine correlates well with the long-term prognosis. Thus, this parameter has been used in previous studies, and will be used in this study, as the primary indicator of therapeutic efficacy. We propose a pilot study to test the hypothesis that selective B lymphocyte depletion will result in disappearance of pathogenic antibodies and induce remission of proteinuria in patients with idiopathic membranoproliferative glomerulonephritis. Our population will be 10 adults with MPGN involving ...
Membranoproliferative Glomerulonephritis type II {1:Abrera-Abeleda et al. (2006)} summarized features of MPGN relevant to the complement cascade. MPGN type II, also known as dense deposit disease, causes chronic renal dysfunction that progresses to end-stage renal disease in about half of patients within 10 years of diagnosis. MPGN types I and III are variants of immune complex-mediated disease; MPGN II, in contrast, has no known association with immune complexes ({2:Appel et al., 2005}). MPGN II accounts for less than 20% of cases of MPGN in children and only a fractional percentage of cases in adults. Both sexes are affected equally, with the diagnosis usually made in children between the ages of 5 and 15 years who present with nonspecific findings such as hematuria, proteinuria, acute nephritic syndrome, or nephrotic syndrome. More than 80% of patients with MPGN II are positive for serum C3 nephritic factor (C3NeF), an autoantibody directed against C3bBb, the convertase of the alternative ...
I have been |b|diagnosed to have idiopathic membranoproliferative glomerulonephritis (MPGN)|/b| Type 1. I am taking one drug daily (Renitec- an ACE-inhibitor). What are your recommendations on my condition? Will this eventually lead to renal failure, even if I take the drug religiously? My 24-hour urine protein count is aroung 1.8 to 2g. Is this manageable? Is there a possibility that my protein count will normalize? What are the critical signs/factors that I should watch out for? Do I need to take any special diet?
Membranoproliferative glomerulonephritis (MPGN), also known as mesangiocapillary glomerulonephritis, is a pattern of glomerular injury viewed by light microscopy. Its name is derived from the characteristic histologic changes including hypercellulari
Overactivation of the alternative pathway of the complement system is associated with the renal diseases atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G). C3 nephritic factors (C3NeF) play an important role in C3G pathogenesis by stabilizing the key enzymatic complex of complement, the C3 convertase. However, the reliability of assays detecting these autoantibodies is limited. Therefore, in this study, we validated and optimized a prototype hemolytic method for robust detection and characterization of factors causing convertase overactivity in large patient cohorts. The assay assesses convertase activity directly in the physiological milieu of serum and therefore is not restricted to detection of stabilizing autoantibodies such as C3NeF but may also reveal genetic variants resulting in prolonged convertase activity. We first defined clear cutoff values based on convertase activity in healthy controls. Next, we evaluated 27 C3G patient samples and found 16 positive for prolonged
4. The complement based MPGN usually have capillary wall staining for C3 deposits, then look at the EM --, if sausage shaped deposits think dense deposit disease and if not then C3 glomerular disease. Both of these are a result of dysregulation of the alternative pathway of complement ...
Dense deposit disease (DDD) is a condition that primarily affects kidney function. Signs and symptoms usually start between the ages of 5 and 15 but may also begin in adulthood. The major features of DDD are due to kidney malfunction, and often include proteinuria; hematuria; reduced amounts of urine; low levels of protein in the blood; and swelling in many areas of the body. About half of affected people develop end-stage renal disease (ESRD) within 10 years after symptoms start. DDD can have genetic or non-genetic causes. It can be caused by mutations in the C3 and CFH genes; it may develop as a result of both genetic risk factors and environmental triggers; or it can result from the presence of autoantibodies that block the activity of proteins needed for the bodys immune response. Most cases are sporadic (occurring by chance in people with no history of the disorder in their family ...
Study subjects will be asked to take Sulodexide twice a day. The Sulodexide will be taken in addition to the regular medications the subject is on. There will be no change in these other medications. The subject will also be asked to have blood tests each month to follow kidney function. The frequency of these tests is the normal/standard frequency for persons with MPGN II/DDD and is neither increased nor decreased because of participation in this study. The study will occur over 6 months for each subject ...
General The IgG fraction is prepared by chromatographic separation of the IgGs of standard antisera. This procedure removes most of the non-specific proteins
Kidney transplant case. Niels Marcussen Hans Dieperink Odense University Hospital. Risc factors for the graft. Male_1961. Nephrotic syndrome 2004 MGUS Membranoproliferative glomerulonephritis, with kappa-chains deposits Peritoneal dialysis 2006 Renal transplant 16SEP2008 Slideshow 5977816...
A recent article published by AJKD reports 3 patients with nephrotic syndrome, two of whom also had hematuria. Kidney biopsy revealed mesangioproliferative glomerulonephritis in one case, membranoproliferative glomerulonephritis in the second case, and dense deposit disease in the third. In all cases, predominant C4d staining was observed in the glomeruli under immunofluorescence. The term C4…
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Inflammatory pseudotumour is a rare condition that can affect various organs. The clinical and histologic appearance of the pseudotumour may mimic haematological, lymphoproliferative, paraneoplastic or malignant processes. A previously healthy 39-year-old man presented with nephrotic syndrome. He had a history of headaches, nausea and swollen ankles. Computed tomography of the abdomen revealed a 6-cm mass in the spleen. Following a renal biopsy, a diagnosis of membranoproliferative glomerulonephritis (MPGN) type I was made. Splenectomy was performed and the examination revealed a mixed population of lymphocytes with predominantly T-cells, B-cells and lymphoplasmacytoid cells. Immunostaining confirmed that the small cells were mostly T-cells positive for all T-cell markers including CD2, CD3, CD4, CD5, CD7 and CD8. A diagnosis of inflammatory pseudotumour was established. The removal of the spleen was followed by remission of glomerulonephritis, but it was complicated by a subphrenic abscess and ...
Kidney inflammation diseases, such as glomerulonephritis, membranoproliferative glomerulonephritis (MPGN), IgA nephropathy, also known as Bergers disease, and lupus nephritis can lead to chronic kidney disease and kidney failure.
We have made a unique observation that, despite similar metabolic and cardiovascular outcomes related to juvenile obesity, adverse cellular responses within the kidney were shown only in males. Obesity in males was therefore characterized with an increase in kidney mass in conjunction with enlarged glomerular area and nucleated cell number together with raised Caspase-3 abundance. These possible signs of hypercellularity25 and reduced apoptosis have been linked to the development of glomerular proliferative nephritis in humans.26 We have thus found an early response that is compatible with the development of either membranoproliferative glomerulonephritis or focal segmental glomerulerosclerosis in normotensive obese individuals. Coincidently, these adaptations were accompanied by a pronounced effect on markers of metabolic and cellular stress and components of the inflammatory cascade in the cortex of male kidneys only. This could thus contribute to the established sex disparity in the ...
Dr. Arshad Ali (AA), from Emory University School of Medicine in Atlanta, Georgia, discusses his abstract for the National Kidney Foundations 2015 Spring Clinical Meetings (SCM15), Proliferative C4 Dense Deposition Disease Concurrent with Acute Thrombotic Microangiopathy in an Adult Patient with Acute Renal Failure, with Dr. Kenar Jhaveri, AJKD Blog Editor. AJKDblog: Why dont you…
Mesangiocapillary glomerulonephritis (MCGN)-which is synonymous with membranoproliferative glomerulonephritis-is diagnosed when renal biopsy reveals glomeruli with a characteristic lobular appearance. Immunohistology and electron microscopy allow further subdivision into three patterns, types I, II (also called dense deposit disease), and III. Clinical presentation is with proteinuria (sometimes nephrotic syndrome) and/or haematuria; hypertension and/or impairment of excretory kidney function may be associated....
MPGN type 2 is an autoimmune disease, in which the immune system attacks filters (glomeruli) in kidneys mistakenly. When the outside harmful substances such s lupus virus, hepatitis B virus invade into body as antigens, the antibodies in body fail to defeat them immediately for autoimmune disorder. As a result, the antigens and antibodies bind to each other and form immune complexes in blood and flow into kidneys through circulation ...
Membranoproliferative glomerulonephritis (MPGN) is inflammation of the filtering units of the kidney (glomerulonephritis or GN) that occurs with activation of the complement system of immunity. The Complement System The complement system of proteins provides immunity through 2 pathways. In the classical pathway (blue half of cartoon), an antigen-antibody forms an immune complex that activates C1.…
A 42-year-old woman with a known diagnosis of acquired partial lipodystrophy (PLD) presented to the ophthalmic clinic with blurring and distortion of vision in the left eye. On general inspection, she had classic lipoatrophy in the cephalothoracic distribution. Ophthalmic examination showed right vision 6/5 and left 6/18. She had marked bilateral drusen and retinal pigment … ...
Partial lipodystrophy with nephrotic syndrome.: A patient with nephrotic syndrome in association with partial lipodystrophy is reported. The features of partial
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Factor H deficiencyFactor H deficiency

More than 80% of patients with MPGN II are positive for serum C3 nephritic factor (C3NeF), an autoantibody directed against ... Laboratory features usually include decreased serum levels of factor H, complement component C3 ({120700}), and a decrease in ... Complement factor H (CFH) is a negative regulator of the alternative pathway of complement, and properdin is the sole positive ... Complement factor H-related protein 1 deficiency and factor H antibodies in pediatric patients with atypical hemolytic uremic ...
more infohttp://diseaseinfosearch.org/Factor+H+deficiency/8366

Evaluation and treatment of membranoproliferative glomerulonephritisEvaluation and treatment of membranoproliferative glomerulonephritis

Hypocomplementemic proliferative glomerulonephritis with C3 nephritic-factor-like activity in multiple myeloma. Nephron 1989; ... Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. ... C3 deposition glomerulopathy due to a functional factor H defect. Kidney Int 2009; 75:1230. ... These issues will be reviewed here with the exception of MPGN due to complement-mediated disease (C3 glomerulopathies, ...
more infohttps://www.uptodate.com/contents/evaluation-and-treatment-of-membranoproliferative-glomerulonephritis

C3 nephritic factor can be associated with membranous glomerulonephritis.  - PubMed - NCBIC3 nephritic factor can be associated with membranous glomerulonephritis. - PubMed - NCBI

Commonly associated with C3NeF are low C3 levels, decreased total haemolytic complement (CH50) and normal C4 levels. C3NeF ... C3 nephritic factor can be associated with membranous glomerulonephritis.. Niel O1, Dallocchio A, Thouret MC, Guigonis V, ... C3 nephritic factor (C3NeF) has been described in association with membranoproliferative glomerulonephritis and is involved in ... C3NeF is an immunoglobulin G (IgG) autoantibody which binds to the complement component 3 (C3) convertase C3bBb, thereby ...
more infohttps://www.ncbi.nlm.nih.gov/pubmed/25395361

Wie Doet Wat Database 3.0 | NVKCWie Doet Wat Database 3.0 | NVKC

Complement C3 nephritic factor [aanwezigheid] in serum of plasma. serum. Complement C3bBbp [eenheden/volume] in serum of plasma ... Complement factor H [massa/volume] in serum of plasma. EDTA plasma. Complement factor I [massa/volume] in serum of plasma. EDTA ... Complement factor B [massa/volume] in serum or plasma. EDTA plasma. Complement factor Bb [massa/volume] in serum of plasma. ... Complement C3d [massa/​volume] in serum of plasma. EDTA plasma. Complement C4d [massa/volume] in plasma m.b.v. immunoassay. ...
more infohttps://www.nvkc.nl/professional/wie-doet-wat-database?action=search&laboratory=A014&searchtext=Radboudumc

C3 Nephritic Factor | Diagnostic Testing | Clinical LaboratoryC3 Nephritic Factor | Diagnostic Testing | Clinical Laboratory

Complement Lab. Synonyms. Complement (C3 Nephritic Factor);2-D Immunoelectrophoresis (C3 Nephritic Factor) ... This is not a direct measurement of antibody quality, but only a qualitative (indirect) measure of its activity in causing C3 ...
more infohttps://www.nationaljewish.org/for-professionals/diagnostic-testing/adx/tests/c3-nephritic-factor

Maria Antonietta Villa - Research Output
     - Italian Ministry of HealthMaria Antonietta Villa - Research Output - Italian Ministry of Health

Selective C3 deficiency due to C3 nephritic factor in an apparently healthy girl. Tedesco, F., Tovo, P. A., Tamaro, G., ... Two types of dysfunctional eighth component of complement (C8) molecules in C8 deficiency in man. Reconstitution of normal C8 ...
more infohttps://moh-it.pure.elsevier.com/en/persons/maria-antonietta-villa/publications/?type=%2Fdk%2Fatira%2Fpure%2Fresearchoutput%2Fresearchoutputtypes%2Fcontributiontojournal%2Farticle

Code System ConceptCode System Concept

Complement C3 nephritic factor Current Synonym true false 3744850016 C3 - complement component 3 nephritic factor Current ... C3 - Complement component 3 nephritic factor Current Synonym true false 1220653012 Complement component 3 nephritic factor ... C3 NeF - complement component 3 nephritic factor Current Synonym true false 3744853019 C3 - complement component 3 convertase ... C3 NeF - Complement component 3 nephritic factor Current Synonym true false 1220652019 ...
more infohttps://phinvads.cdc.gov/vads/ViewCodeSystemConcept.action?oid=2.16.840.1.113883.6.96&code=123001009

Mayo Clinic/Renal Pathology Society Consensus Report on Pathologic Classification, Diagnosis, and Reporting of GN | American...Mayo Clinic/Renal Pathology Society Consensus Report on Pathologic Classification, Diagnosis, and Reporting of GN | American...

C3 nephritic factor and persistently low complement C3 and C5. Pediatr Nephrol 6: 239-243, 1992pmid:1616831. ... 40 C3 glomerulopathy is associated with abnormalities in regulation of the alternative pathway of complement. C3 glomerulopathy ... Granular C3 is often present along the monoclonal Ig.. If the intensity of staining for C3 exceeds the intensity of staining ... C3 glomerulopathy is characterized by the presence of dominant C3 deposits in the glomeruli with minimal or no Ig deposits on ...
more infohttps://jasn.asnjournals.org/content/27/5/1278

Diagnosis and treatment of lipodystrophy: a step-by-step approach | SpringerLinkDiagnosis and treatment of lipodystrophy: a step-by-step approach | SpringerLink

Patients tend to have low serum C3 complement and leptin levels and detectable C3 nephritic factor [6]. ... Activation of the classical complement pathway and low C4 complement levels have been associated with low leptin and ... The distribution of fat loss, age of onset, certain phenotypic traits, and family history are determining factors in diagnosing ... Diet and exercise are important factors in managing lipodystrophy comorbidities. Practice guidelines recommend that patients ...
more infohttps://link.springer.com/article/10.1007%2Fs40618-018-0887-z

PED Flashcards by Dimitrios Drekolias | BrainscapePED Flashcards by Dimitrios Drekolias | Brainscape

A/w accelerated complement activation and C3 nephritic factor ==> Hypothesized to cause lysis of adipose tissue. ...
more infohttps://www.brainscape.com/flashcards/ped-6562480/packs/10385697

List of MeSH codes (D12.776) - WikipediaList of MeSH codes (D12.776) - Wikipedia

... complement c3 nephritic factor MeSH D12.776.377.715.548.114.323.390 - immunoconglutinins MeSH D12.776.377.715.548.114.323.480 ... complement factor h MeSH D12.776.377.715.182.338 - hemopexin MeSH D12.776.377.715.182.580 - plasminogen MeSH D12.776.377.715. ... vascular endothelial growth factor a MeSH D12.776.467.100.800.300 - vascular endothelial growth factor b MeSH D12.776.467.100. ... fibroblast growth factor 4 MeSH D12.776.624.664.700.112 - fibroblast growth factor 6 MeSH D12.776.624.664.700.114 - fms-like ...
more infohttps://en.wikipedia.org/wiki/List_of_MeSH_codes_(D12.776)

C4 nephritic factor in a patient with chronic glomerulonephritis.C4 nephritic factor in a patient with chronic glomerulonephritis.

... whose C3 level was quite low (less than 1% of normal). C4NeF prevented the intrinsic decay of C4b2a enzyme and prolonged the ... C4 nephritic factor (C4NeF) was found in the serum of a patient with chronic glomerulonephritis, ... Complement C3 / metabolism. Complement C4 / metabolism. Electrophoresis, Polyacrylamide Gel. Female. Glomerulonephritis / ... C4 nephritic factor (C4NeF) was found in the serum of a patient with chronic glomerulonephritis, whose C3 level was quite low ( ...
more infohttp://www.biomedsearch.com/nih/C4-nephritic-factor-in-patient/3298656.html

Barraquer-Simons syndrome - WikipediaBarraquer-Simons syndrome - Wikipedia

... low C3 serum complement levels, and the presence of a C3 nephritic factor. C3 nephritic factor is a serum immunoglobulin G that ... C3 nephritic factor induces the lysis of adipocytes that secrete adipsin, a product identical to complement factor D. The ... Around 83% of APL patients had low complement 3 (C3) levels and the presence of polyclonal immunoglobulin C3 nephritic factor. ... due to complement activation and consumption of C3). Low C3 levels may impair complement-mediated phagocytosis and bacterial ...
more infohttps://en.wikipedia.org/wiki/Barraquer%E2%80%93Simons_syndrome

Progressive Lipodystrophy: Background, Pathophysiology, EtiologyProgressive Lipodystrophy: Background, Pathophysiology, Etiology

... low C3 serum complement levels, and the presence of a C3 nephritic factor. C3 nephritic factor is a serum immunoglobulin G that ... C3 nephritic factor induces the lysis of adipocytes that secrete adipsin, a product identical to complement factor D. The ... Adipocytes synthesize factor D, the limiting component of the alternative complement pathway, which cleaves C3-bound factor B ... Adipocytes synthesize factor D, the limiting component of the alternative complement pathway, which cleaves C3-bound factor B ...
more infohttps://emedicine.medscape.com/article/1082489-overview

C3 Nephritic Factor | Molecular Otolaryngology and Renal Research LaboratoriesC3 Nephritic Factor | Molecular Otolaryngology and Renal Research Laboratories

C3 nephritic factor and persistently low complement C3 and C5. Pediatr Nephrol. 1992 May; 6(3):239-43.. PubMed ID: 1616831 ... C3 nephritic factors (C3Nefs), C5 nephritic factors (C5Nefs) and nephritic factor activity (nef activity) are defined as IgG ... C3 Nephritic Factor/C5 Nephritic Factor/Nef Activity Assay. Dense Deposit Disease (DDD, aka Membranoproliferative ... Koch FJ, et al.: Test for C3 nephritic factor activity by immunofixation electrophoresis. Am J Clin Pathol. 1981 Jul, 76(1):63- ...
more infohttps://morl.lab.uiowa.edu/clinical-diagnostics/complement-assays/tests-included/c3-nephritic-factor

Progressive LipodystrophyProgressive Lipodystrophy

... low C3 serum complement levels, and the presence of a C3 nephritic factor. C3 nephritic factor is a serum immunoglobulin G that ... C3 nephritic factor induces the lysis of adipocytes that secrete adipsin, a product identical to complement factor D. The ... Hypocomplementemia is characterized by a low C3 complement level, a normal C4 level, and the presence of C3 nephritic factor. ... Adipocytes synthesize factor D, the limiting component of the alternative complement pathway, which cleaves C3-bound factor B ...
more infohttp://misc.medscape.com/pi/iphone/medscapeapp/html/A1082489-business.html

Complement, not Compliment | PascaleLanes Stream of ThoughtComplement, not Compliment | PascaleLane's Stream of Thought

... that occurs with activation of the complement system of immunity. The Complement System The complement system of proteins ... and complements: C3, C4, and C3 nephritic factor. The latter, an antibody to the activated form of C3 that can activate C5, is ... After a couple of steps it activates C3. Ultimately C5 and other complements come together, resulting in a membrane attack ... If C4 is low, then the classical pathway has been stimulated; C3 may be suppressed as well. If C4 remains normal with a low C3 ...
more infohttps://pascalelane.wordpress.com/2011/12/30/complement-not-compliment/

Dense Deposit Disease: Clinicopathologic Study of 32 Pediatric and Adult Patients | American Society of NephrologyDense Deposit Disease: Clinicopathologic Study of 32 Pediatric and Adult Patients | American Society of Nephrology

C3 nephritic factor, tested in nine patients, was positive in six of six children but in only one of three adults (P = 0.027). ... Variations in the complement regulatory genes factor H (CFH) and factor H related 5 (CFHR5) are associated with ... C3 nephritic factor was present in all six children and one of three adults tested (P = 0.027). The single patient (an adult) ... Importantly, all children in our cohort had a depressed serum complement C3 compared with only 42% of adults (P = 0.001). ...
more infohttps://cjasn.asnjournals.org/content/4/1/22?ijkey=f7e6dfb29a9cae237708651f2e8b433b6d92835a&keytype2=tf_ipsecsha

Immunology Flashcards by  | BrainscapeImmunology Flashcards by | Brainscape

Nephritic factors: autoantibodies targeted at complement components. Stabilise C3 convertase leading to consumption of C3 ... This leads to increased risk of autoimmunity Also complement activation increases clearance of immune complexes by erythrocytes ... Formation of antigen-antibody complexes activates which complement pathway? Starts with which protein? ... C4b binds to the pathogen and starts the process forming C3 convertase. ...
more infohttps://www.brainscape.com/flashcards/immunology-4870435/packs/7066822

Genetic Atypical Hemolytic-Uremic Syndrome - GeneReviews® - NCBI BookshelfGenetic Atypical Hemolytic-Uremic Syndrome - GeneReviews® - NCBI Bookshelf

C3G is associated with alternative pathway complement activation usually caused by C3 nephritic factors, IgG autoantibodies ... C3. 19p13. ​.3. Complement C3. C3 database. C3base: Mutation registry for C3 deficiency. C3. C3. ... Complement factor B. CFB database. CFB. CFB. CFH. 1q31. ​.3. Complement factor H. CFHbase: Mutation registry for Factor H ... C3 encodes complement component C3. Mainly produced by the liver, C3 is the pivotal component of the complement system. The ...
more infohttps://www.ncbi.nlm.nih.gov/books/NBK1367/

Complement gene variants determine the risk of immunoglobulin-associated MPGN and C3 glomerulopathy and predict long-term renal...Complement gene variants determine the risk of immunoglobulin-associated MPGN and C3 glomerulopathy and predict long-term renal...

Complement C3 Nephritic Factor/genetics. *Complement Pathway, Alternative/genetics*. *Female. *Fluorescent Antibody Technique ... Complement gene variants determine the risk of immunoglobulin-associated MPGN and C3 glomerulopathy and predict long-term renal ... and C3 glomerulopathy (C3G). In this study we aimed: (1) to evaluate the complement genetic and biochemical profile in patients ... patients without complement gene mutations or C3NeFs--autoantibodies that stabilize the alternative pathway C3 convertase--have ...
more infohttps://phgkb.cdc.gov/PHGKB/phgHome.action?action=forward&dbsource=huge&id=125618

Lipodystrophy, including LipoatrophyLipodystrophy, including Lipoatrophy

... and autoimmune disorders.Presence of C3 nephritic factor. C3 nephritic factor casues activation of the alternative complement ... This is associated with increased C3 nephritic factor levels, which is responsible for decreased C3 levels. ... A lack of knowledge regarding specific etiopathogenic factors, as well as the opportunism of some professionals and the media, ... The FDA has approved tesamorelin (synthetic analogue of growth hormone-relasing factor) acetate injection as the first and only ...
more infohttp://www.psychiatryadvisor.com/dermatology/lipodystrophy-including-lipoatrophy/article/589225/

Pediatric Nephritis: Practice Essentials, Etiology, EpidemiologyPediatric Nephritis: Practice Essentials, Etiology, Epidemiology

C3 nephritic factor associated with C3 glomerulopathy in children. Pediatr Nephrol. 2014 Jan. 29 (1):85-94. [Medline]. ... Defining the complement biomarker profile of C3 glomerulopathy. Clin J Am Soc Nephrol. 2014 Nov 7. 9 (11):1876-82. [Medline]. ... Nephritic syndrome may occur in people of all races. The race of the child is not generally helpful in determining the primary ... C3 glomerulopathy: clinicopathologic features and predictors of outcome. Clin J Am Soc Nephrol. 2014 Jan. 9 (1):46-53. [Medline ...
more infohttps://emedicine.medscape.com/article/982811-overview

PRIME PubMed | facial lipoatrophy syndrome journal articles from PubMedPRIME PubMed | facial lipoatrophy syndrome journal articles from PubMed

... laboratories showing absence of C3 nephritic factor and normal complement levels. The patient was treated with hyaluronic acid ...
more infohttps://www.unboundmedicine.com/medline/research/facial%20lipoatrophy%20syndrome

Anti-complement Therapy Of Renal Diseases | 17519Anti-complement Therapy Of Renal Diseases | 17519

T he role of complement activation as a primary etiology and a secondary exacerbater of kidney disease have long been known. ... C3 nephritic factor stabilizing C3 convertase, and C4 nephritic factor stabilizing C4 convertase. Two renal disorders have been ... There are 2 proteins (Factor B, C3) that are integral complement component in which gain of function mutations lead to ... There is a predominance of C3 staining by immunofluoresence in the glomeruli. Usually evidence of complement activation can be ...
more infohttps://www.omicsonline.org/proceedings/anticomplement-therapy-of-renal-diseases-17519.html
  • The treatment of MPGN depends upon the underlying cause since most patients have either a circulating immune complex disease or dysregulation of the alternative complement pathway. (uptodate.com)
  • Factor H (H) is a crucial fluid phase regulator of the AP, as it is an essential cofactor for factor I (C3b inactivator) in the proteolytic inactivation of C3b and C3(H 2 O) ( 6 , 7 ). (jimmunol.org)
  • Dense deposit disease (DDD) is a glomerular disease defined at the electron microscopic level by a transformation of the lamina densa of the glomerular basement membrane by ribbon-like, highly electron-dense material, which by immunofluorescence stains predominantly for C3. (asnjournals.org)
  • This is not a direct measurement of antibody quality, but only a qualitative (indirect) measure of its activity in causing C3 conversion. (nationaljewish.org)
  • The latter, an antibody to the activated form of C3 that can activate C5, is positive in 60-70% of patients with type 2 disease, but only 20% of other types. (wordpress.com)
  • The recent development of therapeutic anti-complement antibody preparations has enabled the clinician to capitalize on these scientific advances by treating these disorders in a targeted fashion. (omicsonline.org)
  • Eculizumab is a humanized anti-complement monoclonal antibody that has been utilized in the treatment of complements mediated diseases, including: Paroxysmal Nocturnal Hemoglobinuria (PNH), aHUS, C3G, and catastrophic anti-phospholipid syndrome. (omicsonline.org)
  • Drugs that specifically target complement activation (eculizumab) offer novel options for research. (wordpress.com)
  • Plasma exchange and eculizumab prophylaxis may prevent disease recurrences in those with mutation of circulating factors ( CFH, C3 , CFB , and CFI ). (nih.gov)
  • Other studies demonstrate components of the complement system depositing within the glomeruli. (wordpress.com)
  • There is a predominance of C3 staining by immunofluoresence in the glomeruli. (omicsonline.org)
  • Uremic bleeding is multifactorial , involving dysfunctional Von Willebrand factor, accumulation of many uremic toxins, particularly L-arginine, increased levels of cyclic AMP and cyclic GMP (cGMP) both of which reduce levels of thromboxane A2 (TxA2) and ADP, anemia, which causes platelets to travel midstream through the blood vessels, farther away from the endothelium. (blogspot.com)
  • T he role of complement activation as a primary etiology and a secondary exacerbater of kidney disease have long been known. (omicsonline.org)
  • Cryoprecipitate contains factor VIII, vWF, and fibrinogen, likely increasing the proportion of functional clotting factors in uremic patients' plasma. (blogspot.com)
  • The diagnosis of DDD was based on the ultrastructural finding of a transformation of glomerular basement membranes by ribbon-like, highly electron-dense material and predominant immunofluorescence staining for C3. (asnjournals.org)
  • In addition, the patient's serum contained C3-cleaving activity, probably C4b2a, C4NeF complex, which may have lowered the level of C3 in the patient's serum. (biomedsearch.com)
  • By binding to the short consensus repeat domain 3 of factor H, the dimer LOI blocked one of three interaction sites between H and C3b and thus inhibited the activity of H and induced an uncontrolled activation of the AP. (jimmunol.org)
  • Among the evolving risk factors for cardiovascular disease, could you comment on the role of physical activity or inactivity? (amazonaws.com)
  • Why the glomerulus is preferentially affected is unknown, although the physical stresses of glomerular filtration are likely to play a role in local complement activation. (asnjournals.org)