Complement c3 nephritic factor. Medical search

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.

A glycoprotein that is central in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C3 can be cleaved into COMPLEMENT C3A and COMPLEMENT C3B, spontaneously at low level or by C3 CONVERTASE at high level. The smaller fragment C3a is an ANAPHYLATOXIN and mediator of local inflammatory process. The larger fragment C3b binds with C3 convertase to form C5 convertase.

A glycoprotein that is important in the activation of CLASSICAL COMPLEMENT PATHWAY. C4 is cleaved by the activated COMPLEMENT C1S into COMPLEMENT C4A and COMPLEMENT C4B.

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.

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.

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.

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.

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).

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.

Enzymes that activate one or more COMPLEMENT PROTEINS in the complement system leading to the formation of the COMPLEMENT MEMBRANE ATTACK COMPLEX, an important response in host defense. They are enzymes in the various COMPLEMENT ACTIVATION pathways.

A 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.

Chronic glomerulonephritis characterized histologically by proliferation of MESANGIAL CELLS, increase in the MESANGIAL EXTRACELLULAR MATRIX, and a thickening of the glomerular capillary walls. This may appear as a primary disorder or secondary to other diseases including infections and autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Various subtypes are classified by their abnormal ultrastructures and immune deposits. Hypocomplementemia is a characteristic feature of all types of MPGN.

A 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.

The smaller fragment generated from the cleavage of complement C3 by C3 CONVERTASE. C3a, a 77-amino acid peptide, is a mediator of local inflammatory process. It induces smooth MUSCLE CONTRACTION, and HISTAMINE RELEASE from MAST CELLS and LEUKOCYTES. C3a is considered an anaphylatoxin along with COMPLEMENT C4A; COMPLEMENT C5A; and COMPLEMENT C5A, DES-ARGININE.

A 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.

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 activation initiated by the interaction of microbial ANTIGENS with COMPLEMENT C3B. When COMPLEMENT FACTOR B binds to the membrane-bound C3b, COMPLEMENT FACTOR D cleaves it to form alternative C3 CONVERTASE (C3BBB) which, stabilized by COMPLEMENT FACTOR P, is able to cleave multiple COMPLEMENT C3 to form alternative C5 CONVERTASE (C3BBB3B) leading to cleavage of COMPLEMENT C5 and the assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.

The 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 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.

The large fragment formed when COMPLEMENT C4 is cleaved by COMPLEMENT C1S. The membrane-bound C4b binds COMPLEMENT C2A, a SERINE PROTEASE, to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).

A 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.

The larger fragment generated from the cleavage of COMPLEMENT C3 by C3 CONVERTASE. It is a constituent of the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb), and COMPLEMENT C5 CONVERTASES in both the classical (C4b2a3b) and the alternative (C3bBb3b) pathway. C3b participates in IMMUNE ADHERENCE REACTION and enhances PHAGOCYTOSIS. It can be inactivated (iC3b) or cleaved by various proteases to yield fragments such as COMPLEMENT C3C; COMPLEMENT C3D; C3e; C3f; and C3g.

A serine protease that cleaves multiple COMPLEMENT 3 into COMPLEMENT 3A (anaphylatoxin) and COMPLEMENT 3B in the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. It is a complex of COMPLEMENT 4B and COMPLEMENT 2A (C4b2a).

A 105-kDa serum glycoprotein with significant homology to the other late complement components, C7-C9. It is a polypeptide chain cross-linked by 32 disulfide bonds. C6 is the next complement component to bind to the membrane-bound COMPLEMENT C5B in the assembly of MEMBRANE ATTACK COMPLEX. It is encoded by gene C6.

A 206-amino-acid fragment in the alpha chain (672-1663) of C3b. It is generated when C3b is inactivated (iC3b) and its alpha chain is cleaved by COMPLEMENT FACTOR I into C3c (749-954), and C3dg (955-1303) in the presence COMPLEMENT FACTOR H.

An important soluble regulator of the alternative pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It is a 139-kDa glycoprotein expressed by the liver and secreted into the blood. It binds to COMPLEMENT C3B and makes iC3b (inactivated complement 3b) susceptible to cleavage by COMPLEMENT FACTOR I. Complement factor H also inhibits the association of C3b with COMPLEMENT FACTOR B to form the C3bB proenzyme, and promotes the dissociation of Bb from the C3bBb complex (COMPLEMENT C3 CONVERTASE, ALTERNATIVE PATHWAY).

A 302-amino-acid fragment in the alpha chain (672-1663) of C3b. It is generated when C3b is inactivated (iC3b) and its alpha chain is cleaved by COMPLEMENT FACTOR I into C3c, and C3dg (955-1303) in the presence COMPLEMENT FACTOR H. Serum proteases further degrade C3dg into C3d (1002-1303) and C3g (955-1001).

A component of the CLASSICAL COMPLEMENT PATHWAY. C2 is cleaved by activated COMPLEMENT C1S into COMPLEMENT C2B and COMPLEMENT C2A. C2a, the COOH-terminal fragment containing a SERINE PROTEASE, combines with COMPLEMENT C4B to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).

A serum protein which is important in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY. This enzyme cleaves the COMPLEMENT C3B-bound COMPLEMENT FACTOR B to form C3bBb which is ALTERNATIVE PATHWAY C3 CONVERTASE.

A 63-kDa serum glycoprotein encoded by gene C9. Monomeric C9 (mC9) binds the C5b-8 complex to form C5b-9 which catalyzes the polymerization of C9 forming C5b-p9 (MEMBRANE ATTACK COMPLEX) and transmembrane channels leading to lysis of the target cell. Patients with C9 deficiency suffer from recurrent bacterial infections.

Molecules on the surface of some B-lymphocytes and macrophages, that recognize and combine with the C3b, C3d, C1q, and C4b components of complement.

A 77-kDa subcomponent of complement C1, encoded by gene C1S, is a SERINE PROTEASE existing as a proenzyme (homodimer) in the intact complement C1 complex. Upon the binding of COMPLEMENT C1Q to antibodies, the activated COMPLEMENT C1R cleaves C1s into two chains, A (heavy) and B (light, the serine protease), linked by disulfide bonds yielding the active C1s. The activated C1s, in turn, cleaves COMPLEMENT C2 and COMPLEMENT C4 to form C4b2a (CLASSICAL C3 CONVERTASE).

A product of COMPLEMENT ACTIVATION cascade, regardless of the pathways, that forms transmembrane channels causing disruption of the target CELL MEMBRANE and cell lysis. It is formed by the sequential assembly of terminal complement components (COMPLEMENT C5B; COMPLEMENT C6; COMPLEMENT C7; COMPLEMENT C8; and COMPLEMENT C9) into the target membrane. The resultant C5b-8-poly-C9 is the "membrane attack complex" or MAC.

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.

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.

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.

Inflammation of any part of the KIDNEY.

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.

A glycine-rich, heat-labile serum glycoprotein that contains a component of the C3 CONVERTASE ALTERNATE PATHWAY (C3bBb). Bb, a serine protease, is generated when factor B is cleaved by COMPLEMENT FACTOR D into Ba and Bb.

A 150-kDa serum glycoprotein composed of three subunits with each encoded by a different gene (C8A; C8B; and C8G). This heterotrimer contains a disulfide-linked C8alpha-C8gamma heterodimer and a noncovalently associated C8beta chain. C8 is the next component to bind the C5-7 complex forming C5b-8 that binds COMPLEMENT C9 and acts as a catalyst in the polymerization of C9.

The first complement component to act in the activation of CLASSICAL COMPLEMENT PATHWAY. It is a calcium-dependent trimolecular complex made up of three subcomponents: COMPLEMENT C1Q; COMPLEMENT C1R; and COMPLEMENT C1S at 1:2:2 ratios. When the intact C1 binds to at least two antibodies (involving C1q), C1r and C1s are sequentially activated, leading to subsequent steps in the cascade of COMPLEMENT ACTIVATION.

Molecular sites on or in some B-lymphocytes and macrophages that recognize and combine with COMPLEMENT C3B. The primary structure of these receptors reveal that they contain transmembrane and cytoplasmic domains, with their extracellular portion composed entirely of thirty short consensus repeats each having 60 to 70 amino acids.

The larger fragment generated from the cleavage of C5 by C5 CONVERTASE that yields COMPLEMENT C5A and C5b (beta chain + alpha' chain, the residual alpha chain, bound by disulfide bond). C5b remains bound to the membrane and initiates the spontaneous assembly of the late complement components to form C5b-8-poly-C9, the MEMBRANE ATTACK COMPLEX.

The COOH-terminal fragment of COMPLEMENT 2, released by the action of activated COMPLEMENT C1S. It is a SERINE PROTEASE. C2a combines with COMPLEMENT C4B to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).

A G-protein-coupled receptor that signals an increase in intracellular calcium in response to the potent ANAPHYLATOXIN peptide COMPLEMENT C5A.

Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.

The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B.

Compounds that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host.

Serum proteins that inhibit, antagonize, or inactivate COMPLEMENT C1 or its subunits.

Molecular sites on or in B-lymphocytes, follicular dendritic cells, lymphoid cells, and epithelial cells that recognize and combine with COMPLEMENT C3D. Human complement receptor 2 (CR2) serves as a receptor for both C3dg and the gp350/220 glycoprotein of HERPESVIRUS 4, HUMAN, and binds the monoclonal antibody OKB7, which blocks binding of both ligands to the receptor.

Serum peptides derived from certain cleaved COMPLEMENT PROTEINS during COMPLEMENT ACTIVATION. They induce smooth MUSCLE CONTRACTION; mast cell HISTAMINE RELEASE; PLATELET AGGREGATION; and act as mediators of the local inflammatory process. The order of anaphylatoxin activity from the strongest to the weakest is C5a, C3a, C4a, and C5a des-arginine.

Serologic tests based on inactivation of complement by the antigen-antibody complex (stage 1). Binding of free complement can be visualized by addition of a second antigen-antibody system such as red cells and appropriate red cell antibody (hemolysin) requiring complement for its completion (stage 2). Failure of the red cells to lyse indicates that a specific antigen-antibody reaction has taken place in stage 1. If red cells lyse, free complement is present indicating no antigen-antibody reaction occurred in stage 1.

A plasma serine proteinase that cleaves the alpha-chains of C3b and C4b in the presence of the cofactors COMPLEMENT FACTOR H and C4-binding protein, respectively. It is a 66-kDa glycoprotein that converts C3b to inactivated C3b (iC3b) followed by the release of two fragments, C3c (150-kDa) and C3dg (41-kDa). It was formerly called KAF, C3bINF, or enzyme 3b inactivator.

A serum protein that regulates the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. It binds as a cofactor to COMPLEMENT FACTOR I which then hydrolyzes the COMPLEMENT C4B in the CLASSICAL PATHWAY C3 CONVERTASE (C4bC2a).

Endogenous proteins that inhibit or inactivate COMPLEMENT C3B. They include COMPLEMENT FACTOR H and COMPLEMENT FACTOR I (C3b/C4b inactivator). They cleave or promote the cleavage of C3b into inactive fragments, and thus are important in the down-regulation of COMPLEMENT ACTIVATION and its cytolytic sequence.

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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The C3 Nephritic Factor autoantibody stabilizes C3-convertase, which may lead to an excessive activation of complement.: 553 ... "What is C3 Glomerulopathy?". National Renal Complement Therapeutics Centre. Retrieved 2021-02-04. COUSER, W (1 May 1999). " ... "Revisiting the role of factor H in age-related macular degeneration: Insights from complement-mediated renal disease and rare ... Hyperlipidemia is thought to be a result of the increased activity of the liver.: 549 The nephritic syndrome is characterised ...

https://en.wikipedia.org/wiki/Glomerulonephritis

... 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 ...

https://en.wikipedia.org/wiki/Barraquer–Simons_syndrome

... 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 ...

https://en.wikipedia.org/wiki/List_of_MeSH_codes_(D12.776)

Serum complement (C3 and C4) - Complement factors bind to antibodies to form immune complexes and a decreased serum complement ... Nephritic syndrome, like nephrotic syndrome, may involve low level of albumin in the blood due to the protein albumin moving ... Because nephritic syndrome is a syndrome and not a disease, the prognosis depends on the underlying cause. Generally, the ... The pathophysiology of nephritic syndrome is dependent on the underlying disease process, which can vary depending on what ...

https://en.wikipedia.org/wiki/Nephritic_syndrome

He was the first to show that C3 nephritic factor is an autoantibody to the complement C3bBb convertase that activates the ... alternative complement pathway, a finding that suggested the potential of B cell or complement C5 depletion as adjunct ...

https://en.wikipedia.org/wiki/M._Amin_Arnaout

... 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 ...

https://en.wikipedia.org/wiki/List_of_MeSH_codes_(D12.776.124)

Some HLA alleles have been suspected along with complement phenotypes as being genetic factors. Non-aggressive Berger's disease ... Immunofluorescence shows mesangial deposition of IgA often with C3 and properdin and smaller amounts of other immunoglobulins ( ... Nephritic syndrome Acute kidney failure (either as a complication of the frank hematuria, when it usually recovers or due to ... Hence the decision on which patients to treat should be based on the prognostic factors and the risk of progression. Also, IgA ...

https://en.wikipedia.org/wiki/IgA_nephropathy

... and immunofluorescence demonstrates IgA and C3 (a protein of the complement system) in the blood vessel wall. However, overall ... ISBN 978-1-4160-2999-1. Sais G, Vidaller A, Jucglà A, Servitje O, Condom E, Peyri J (1998). "Prognostic factors in ... 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- ...

https://en.wikipedia.org/wiki/Henoch–Schönlein_purpura

DDD is associated with deposition of complement C3 within the glomeruli with little or no staining for immunoglobulin. The ... Membranoproliferative_GN at Nephropathology tutorial MP GN Pathophysiology discusses the nephritic auto-antibodies/factors ( ... Also found with Sjögren syndrome, rheumatoid arthritis, inherited complement deficiencies (esp C3 deficiency), scleroderma, ... February 2008). "Factor I is required for the development of membranoproliferative glomerulonephritis in factor H-deficient ...

https://en.wikipedia.org/wiki/Membranoproliferative_glomerulonephritis

C3 nephritic factor associated with C3 glomerulopathy in children. Pediatr Nephrol. 2014 Jan. 29 (1):85-94. [QxMD MEDLINE Link] ... Defining the complement biomarker profile of C3 glomerulopathy. Clin J Am Soc Nephrol. 2014 Nov 7. 9 (11):1876-82. [QxMD ... What Factors Might Improve Screening for Hypertensive Disorders of Pregnancy? 0.25 CME / CE / ABIM MOC Credits Clinical Review ... Nephritic syndrome may occur in people of all races. The race of the child is not generally helpful in determining the primary ...

http://emedicine.medscape.com/article/982811-overview

C3 nephritic factor and persistently low complement C3 and C5. Pediatr Nephrol 6: 239- 243, 1992. ... In 12 (42.8%) cases, C3 levels were low; in 10 (35.7%) cases, it was within the normal range; and in six cases (21.5%), C3 ... Their mean serum creatinine was 1.69 mg/dl, with an average proteinuria of 2.98 g/d. The mean C3 level was 52.6 mg/dl (normal ... Rheumatoid factor activity was present in six cases (studies were not done in five cases). Serum immunofixation electrophoresis ...

https://cjasn.asnjournals.org/content/5/5/770

In most patients, loss of complement regulation is caused by C3 nephritic factor, an autoantibody directed against the C3 ... The development of molecular diagnostic tools and new therapies directed at controlling the AP of the complement cascade either ... convertase of the AP, but in some patients, mutations in the factor H gene have been identified. For the latter patients, ...

https://pubmed.ncbi.nlm.nih.gov/15800116/

Serum levels of complement components and of C3 nephritic factor (C3NeF) were measured serially in two patients with membrano- ... designated C3 lytic nephritic factor (C3LyNeF), which will cleave C3 to form the breakdown products, β1A and alpha 2D. The ... Finally, the lack of effect of nephrectomy on C3, alpha 2D, and C3NeF levels indicate that the site of C3 breakdown was ... In both patients, low levels of C3 and high levels of preformed alpha 2D, a C3 breakdown product, were present before ...

https://www.jci.org/articles/view/106524/cite

C4 Nephritic Factor? I Still Dont Understand C3 Glomerulopathy!. October 13, 2017 // 0 Comments ... complement. The Serendipitous Complement of IgA Nephropathy: Are We Futuristic Yet? January 20, 2021 // 0 Comments ... The complement system lies at the interface of innate and adaptive immunity. Perhaps its most remarkable feature is that it ... NephMadness 2019: Complement Inhibition in Post-Transplant Thrombotic Microangiopathy. April 4, 2019 // 0 Comments ...

https://ajkdblog.org/tag/complement/

C 3 Nephritic Factor. C3 Nephritic Factor. Complement 3 Nephritic Factor. Tree number(s):. D12.776.124.486.274.920.287. D12.776 ... Complement C3 Nephritic Factor Entry term(s). C 3 Nephritic Factor C3 Nephritic Factor Complement 3 Nephritic Factor ... Complement 3 (1975-1977). Public MeSH Note:. 2006; see COMPLEMENT 3 NEPHRITIC FACTOR 1991-2005, see COMPLEMENT INACTIVATORS ... Complement C3 Nephritic Factor - Preferred Concept UI. M0004930. Scope note. An IgG autoantibody against the ALTERNATIVE ...

https://decs.bvsalud.org/en/ths/resource/?id=22147

Complement C3 nephritic factor Active Synonym false false 3744850016 C3 - complement component 3 nephritic factor Active ... C3 - Complement component 3 nephritic factor Active Synonym false false 1220653012 Complement component 3 nephritic factor ... C3 NeF - complement component 3 nephritic factor Active Synonym false false 3744853019 C3 - complement component 3 convertase ... C3 NeF - Complement component 3 nephritic factor Active Synonym false false 1220652019 ...

https://phinvads.cdc.gov/vads/ViewCodeSystemConcept.action?oid=2.16.840.1.113883.6.96&code=123001009

Positivity to other complement autoantibodies (anti-C1q, anti-C3) was also linked to the presence of nephritic factors. ... C4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy. ... and C3 nephritic factor, whereas for Cluster 2-which is not reliable because of the small number of cases-strong immunoglobulin ... and higher C3 nephritic factor (C3NeF) prevalence at time of diagnosis compared to C4NeF negative patients. Patients positive ...

https://pesquisa.bvsalud.org/brasil/?lang=pt&q=au:Zieg,+Jakub

C3 Convertases, Complement use Complement C3-C5 Convertases C3 Nephritic Factor use Complement C3 Nephritic Factor ... C3 Convertase Activator use Complement Factor D C3 Convertase, Alternative Pathway use Complement C3 Convertase, Alternative ... C3 Convertase (C3bBb) use Complement C3 Convertase, Alternative Pathway C3 Convertase (C4b2a) use Complement C3 Convertase, ... C5 Convertase, Classical use Complement C5 Convertase, Classical Pathway C5 Convertases, Complement use Complement C3-C5 ...

https://decs.bvs.br/I/DeCS2017_Alfab-C.htm

Description: A competitive ELISA for quantitative measurement of Rabbit C3 Nephritic Factor C3 in samples from blood, plasma, ... Description: A sandwich quantitative ELISA assay kit for detection of Guinea pig Complement Component 3 (C3) in samples from ... Description: A competitive ELISA for quantitative measurement of Rabbit C3 Nephritic Factor C3 in samples from blood, plasma, ... Description: A sandwich quantitative ELISA assay kit for detection of Guinea pig Complement Component 3 (C3) in samples from ...

https://www.biolisp.org/rabbit-antibody-un-c3/

... two cases had positive C3-nephritic factor and one had anti-complement factor-H autoantibodies. Complement factor H gene ... Risk factors that contribute to the development include diabetes and female sex. Herein, we report EPN in a 59-year-old male ... The demographic and transplant details were noted, and data were analyzed for any possible risk factor. Results: A total of ... Utility of plasma exchange in early recurrent C3 glomerulopathy. p. 122. Ashwani Kumar, Joyita Bharati, Ritambhra Nada, ...

https://ijtonline.in/showBackIssue.asp?issn=2212-0017

C3). Terminal complement components C3, C5, C8, and C9 may be low or within the reference range, and nephritic factor of the ... C4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy. ... C3 glomerulonephritis. Immunofluorescence microscopy in C3 glomerulonephritis reveals extensive C3 deposition along the ... C3 levels are low in 70-80% of patients with MPGN type II. Early and terminal complement components are within the reference ...

https://emedicine.staging.medscape.com/article/240056-workup

C3 nephritic factor, complement factor I heterozygous mutation (I398L), and anti-factor H autoantibodies (4,500 AU/ml). A ... C3 nephritic factor, complement factor I heterozygous mutation (I398L), and anti-factor H autoantibodies (4,500\u00a0AU/ml). A ... C3 nephritic factor, complement factor I heterozygous mutation (I398L), and anti-factor H autoantibodies (4,500 AU/ml). A ... C3 nephritic factor associated with C3 glomerulopathy in children in PEDIATRIC NEPHROLOGY ...

https://scigraph.springernature.com/pub.10.1007/s00467-015-3061-2

In patients with new-onset nephritis syndrome and a low C3 level, Chauvet et al report that the presence of anti-factor B ... Lamba P, Nam KH, Contractor J, Kim A. Nephritic Syndrome. Prim Care. 2020 Dec. 47 (4):615-629. [QxMD MEDLINE Link]. ... Complement Levels. Differentiation of low and normal serum complement levels may allow the physician to narrow the differential ... Anti-Factor B Antibodies and Acute Postinfectious GN in Children. J Am Soc Nephrol. 2020 Apr. 31 (4):829-840. [QxMD MEDLINE ...

https://www.medscape.com/answers/239278-168247/what-is-the-role-of-serum-complement-measurement-in-the-workup-of-acute-glomerulonephritis-gn

https://decs2020.bvsalud.org/I/DeCS2016_Alfab-C.htm

https://decs2019.bvsalud.org/I/DeCS2017_Alfab-C.htm

https://decs2017.bvsalud.org/I/DeCS2017_Alfab-C.htm

https://decs2018.bvsalud.org/I/DeCS2018_Alfab-C.htm

https://decs2020.bvsalud.org/I/DeCS2017_Alfab-C.htm

1006 elisa kit for alternate complement pathway assay quantitative with 3 standards 1007 elisa kit for anti c3 nephritic factor ... 1136 human complement factor d elisa kit 1137 human complement factor h elisa kit 1138 human complement pan specific c3 reagent ... factor assays reagent for ca 50 ( factor ii deficient plasma, , factor assays reagent for ca 50 ( factor v deficient plasma, , ... factor assays reagent for ca 50 ( factor viii deficient plasma, , factor assays reagent for ca 50 ( factor ix deficient plasma ...

http://tendersindelhi.com/SearchTender?Keyword=glacial+acetic+acid

Complement C3 Nephritic Factor [D12.776.124.486.485.114.323.300] * Immunoconglutinins [D12.776.124.486.485.114.323.390] ... Immunologic Factors. Registry Number. 0. NLM Classification #. QW 575.5.A8. See Also. Autoimmune Diseases. Autoimmunity. Date ... Complement C3 Nephritic Factor [D12.776.124.790.651.114.323.300] * Immunoconglutinins [D12.776.124.790.651.114.323.390] ... Complement C3 Nephritic Factor [D12.776.377.715.548.114.323.300] * Immunoconglutinins [D12.776.377.715.548.114.323.390] ...

https://meshb-prev.nlm.nih.gov/record/ui?ui=D001323

76 often due to the presence of an immunoglobulin termed C3 nephritic factor that binds to the AP C3 convertase and delays its ... ANCA-induced neutrophil activation and initiation of the AP complement system remains to be elucidated, and whether anti- ... A few neoplastic cells co-express gonadotropins and the transcription factor steroidogenic factor 1, together with growth ... Besides gender and diabetes, late CKD stage and malnutrition-inflammation were risk factors see more for UTI. Key words: ...

https://atpase-receptor.com/index.php/2019/01/

Presence of C3 nephritic factor within 6 months of screening, based on central lab results or medical history. ... C3G IC-MPGN C3 Glomerulopathy C3 Glomerulonephritis Complement 3 Glomerulopathy Complement 3 Glomerulopathy (C3G) Complement 3 ... Serum C3 below the LLN during screening.. *Presence of an active urine sediment during screening, as evidenced by hematuria ... Phase III Study Assessing the Efficacy and Safety of Pegcetacoplan in Patients With C3 Glomerulopathy or Immune-Complex ...

https://clinicaltrials.gov/ct2/show/NCT05067127?term=pegcetacoplan&cond=c3g&draw=2&rank=2

Complement 3. Complement C3. Complement 3 Nephritic Factor. Complement C3 Nephritic Factor. ... Complement Inactivators. Complement Inactivator Proteins. DNA-Binding Protein, Cyclic AMP-Responsive. Cyclic AMP Response ... Complement 1s. Complement C1s. Glyceraldehyde 3 Phosphate-Dehydrogenase (NADP+)(Phosphorylating). Glyceraldehyde-3-Phosphate ... Complement. Complement System Proteins. Complement 1. Complement C1. Complement 1 Inactivators. Complement C1 Inactivator ...

https://decs2012.bvsalud.org/I/rep_i2006.htm

https://decs2008.bvsalud.org/I/rep_i2006.htm

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https://decs2011.bvsalud.org/I/rep_i2006.htm

https://decs2016.bvsalud.org/I/rep_i2006.htm

I Still Don't Understand C3 Glomerulopathy! (ajkdblog.org)
BackgroundC3 glomerulonephritis (C3GN) is a rare form of glomerulopathy that is characterized by predominant C3 deposits. (springernature.com)
In patients with new-onset nephritis syndrome and a low C3 level, Chauvet et al report that the presence of anti-factor B autoantibodies may help distinguish new-onset PSGN from hypocomplementemic C3 glomerulopathy. (medscape.com)
This is a Phase 3 study to assess the efficacy and safety of twice-weekly subcutaneous (SC) doses of pegcetacoplan compared to placebo in patients with C3 glomerulopathy (C3G) or immune-complex membranoproliferative glomerulonephritis (IC-MPGN) on the basis of a reduction in proteinuria. (clinicaltrials.gov)
INTRODUCTION: C3 glomerulopathy (C3G) is an ultrarare, chronic and progressive nephropathy mediated by dysregulation of the alternative pathway of complement (AP), with poor prognosis and limited treatment options. (imperial.ac.uk)
INTRODUCTION: C3 glomerulopathy (C3G) is a rare, progressive kidney disease resulting from dysregulation of the alternative pathway (AP) of complement. (imperial.ac.uk)

In most patients, loss of complement regulation is caused by C3 nephritic factor, an autoantibody directed against the C3 convertase of the AP, but in some patients, mutations in the factor H gene have been identified. (nih.gov)
An IgG autoantibody against the ALTERNATIVE PATHWAY C3 CONVERTASE, found in serum of patients with MESANGIOCAPILLARY GLOMERULONEPHRITIS . (bvsalud.org)

Serum levels of complement components and of C3 nephritic factor (C3NeF) were measured serially in two patients with membrano-proliferative glomerulonephritis who were subjected to bilateral nephrectomy and maintained by peritoneal dialysis for 2 wk before renal transplantation. (jci.org)
The complement profiles of types I-III membranoproliferative glomerulonephritis membranoproliferative glomerulonephritis (MPGN) are summarized below. (medscape.com)
In addition, normal complement levels suggest kidney diseases such as immune complex disease, idiopathic rapidly progressive glomerulonephritis, and immunoglobulin G (IgG) or immunoglobulin A (IgA) nephropathy. (medscape.com)

The Serendipitous Complement of IgA Nephropathy: Are We Futuristic Yet? (ajkdblog.org)
Complement Pathways in Membranous Nephropathy: Complex and Multifactorial. (crmrsni-nice.fr)

MPGN type II, also known as dense-deposit disease, is not due to immune complex deposition but results from the dysregulation of the alternative pathway of the complement cascade and secondary persistent complement activation ( 4 ). (asnjournals.org)
The pathophysiologic basis of MPGN II is associated with the uncontrolled systemic activation of the alternative pathway (AP) of the complement cascade. (nih.gov)
His first kidney biopsy confirmed this diagnosis, but complement investigations identified three alternative pathway dysregulation factors: C3 nephritic factor, complement factor I heterozygous mutation (I398L), and anti-factor H autoantibodies (4,500 AU/ml). (springernature.com)

With transplantation, C3 levels rose towards normal and alpha 2D disappeared from the serum. (jci.org)
The serum of both patients contained detectable amounts of C3NeF, a factor which has been shown to react with a cofactor found in normal serum to form an enzyme, designated C3 lytic nephritic factor (C3LyNeF), which will cleave C3 to form the breakdown products, β1A and alpha 2D. (jci.org)
The Rabbit Antibody Un C3 reagent is RUO (Research Use Only) to test human serum or cell culture lab samples. (biolisp.org)
Description: A sandwich quantitative ELISA assay kit for detection of Guinea pig Complement Component 3 (C3) in samples from serum, plasma, tissue homogenates or other biological fluids. (biolisp.org)
Description: A competitive ELISA for quantitative measurement of Rabbit C3 Nephritic Factor C3 in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. (biolisp.org)
Differentiation of low and normal serum complement levels may allow the physician to narrow the differential diagnosis. (medscape.com)
Low serum complement levels suggest the following systemic diseases: cryoglobulinemia, systemic lupus erythematosus (SLE), bacterial endocarditis, and shunt nephritis. (medscape.com)
Normal serum complement levels suggest a visceral abscess, polyarteritis nodosa, Goodpasture syndrome, or Henoch-Schönlein purpura. (medscape.com)

The presence of C3NeF in detectable amounts in both patients suggest that C3LyNeF, formed by the reaction of C3NeF and cofactor, was responsible for the low C3 levels. (jci.org)
Finally, the lack of effect of nephrectomy on C3, alpha 2D, and C3NeF levels indicate that the site of C3 breakdown was extrarenal and that C3NeF and cofactor are at least in large part of extrarenal origin. (jci.org)
NFa (C3NeF) is present in more than 70% of patients, but factor H levels may be low. (medscape.com)

These complexes typically trigger activation of complement and a phase of acute injury in the glomerular capillaries and mesangium. (asnjournals.org)
Urinalysis and sediment examination are crucial in the evaluation of patients with acute nephritic syndrome. (medscape.com)

A second biopsy performed 11 months after initial presentation (nephrotic range proteinuria) showed a C3GN suggestive of isolated C3 deposits.Despite the use of intensive immunosuppressive therapy (rituximab, corticosteroids, mycophenolate), nephrotic-range proteinuria persisted and a third kidney biopsy showed the same C3GN pattern with more endocapillary proliferation. (springernature.com)
Indications for diagnostic biopsy include unexplained nephritic or nephrotic. (merckmanuals.com)
3.5 g/day) and edema qualifying for the nephrotic syndrome to a nephritic syndrome where hematuria and hypertension are more prominent while proteinuria is less pronounced. (statpearls.com)

Biomarkers at baseline were investigated in patients with C3G who participated in two phase 2 studies with the factor D (FD) inhibitor, danicopan. (imperial.ac.uk)

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. (bvsalud.org)
C3 levels are low in about half of the patients with MPGN type I. There is evidence of activation of the classic pathway of complement (ie, low C4, C2, C1q, B, C3). (medscape.com)
Systemic complement AP activation was evident by reduced median concentrations of C3 and C5, elevated sC5b-9, and normal C4, relative to reference ranges. (imperial.ac.uk)
Anti-PLA2R1 Antibodies Containing Sera Induce In Vitro Cytotoxicity Mediated by Complement Activation. (crmrsni-nice.fr)

We report a C3GN patient successfully treated with eculizumab.Case diagnosis/treatmentA 5-year-old boy who presented with proteinuria, hematuria, high ASO titers, and low C3 levels was initially diagnosed with post-streptococcal GN. (springernature.com)

which shows focal and segmental hyalinization of the glomeruli, often with immunostaining showing IgM and complement (C3) deposits in a nodular and coarse granular pattern. (merckmanuals.com)

Nephritic syndrome may occur in people of all races. (medscape.com)

The development of molecular diagnostic tools and new therapies directed at controlling the AP of the complement cascade either locally in the kidney or at the systemic level may lead to effective treatments for MPGN II. (nih.gov)

Targeted inhibition of proximal AP through factor D (FD) blockade represents a rational treatment approach. (imperial.ac.uk)

Because hypocomplementemia is a characteristic finding in all types of MPGN, obtain complement profiles in patients with suspected MPGN. (medscape.com)
C3 levels are low in 70-80% of patients with MPGN type II. (medscape.com)
C3 levels are decreased in 50% of patients with MPGN type III. (medscape.com)

Patients with a nephritic presentation typically have a decreased GFR. (medscape.com)

The second group comprised 12 secondary SS patients (sSS) positive for rheumatoid selleck factor, anti-nuclear antibodies, as shown in Table 1. (atpase-receptor.com)

In both patients, low levels of C3 and high levels of preformed alpha 2D, a C3 breakdown product, were present before nephrectomy and remained essentially unchanged during the anephric period. (jci.org)
In both patients, levels of C4 and C5 were either normal or elevated over the period of the study and bore no relationship to the C3 level. (jci.org)

The high levels of alpha 2D during the anephric period and the disappearance of this protein as C3 levels approach normal at the time of transplantation indicate that the low C3 levels were largely the result of C3 breakdown rather than diminished synthesis. (jci.org)

Terminal complement components C3, C5, C8, and C9 may be low or within the reference range, and nephritic factor of the amplification loop (NFc or C4NeF) or nephritic factor of the terminal pathway (NFt) may be present. (medscape.com)
Early and terminal complement components are within the reference range. (medscape.com)

Complement and clinical biomarkers, biopsy composite score, and activity and chronicity indices were assessed at baseline and analyzed by pairwise Spearman correlation analysis. (imperial.ac.uk)

Biacore analysis of four purified IgG samples confirmed resistance to decay and showed that properdin-independent nephritic factors increased convertase half-life over 50-fold, whereas properdin-dependent nephritic factors increased the half-life 10- to 20-fold and also increased activity of the C3 convertase up to 10-fold. (nih.gov)

On the basis of immunofluorescence (IF) the disorder is divided into C3 glomerulopathy (C3G) or immunoglobulin-mediated membranoproliferative glomerulonephritis. (nih.gov)
Through electron microscopy C3G is further divided into Dense deposit disease, with highly electrondense deposits in the glomerular basement membrane, and C3 glomerulonephritis, with mesangial, intramembranous, subendothelial and subepithelial deposits. (nih.gov)
Lipodystrophy is often associated with glomerulonephritis, low C3 serum complement levels, and the presence of a C3 nephritic factor. (medscape.com)
The definitive diagnosis of C3G requires a renal biopsy with specialized immunofluorescence and electron microscopy studies both for diagnosis and to distinguish between the two major subtypes of C3G: C3 glomerulonephritis (C3GN) and dense deposit disease (DDD). (nih.gov)
Understanding the role of alternative complement pathway dysregulation in membranoproliferative glomerulonephritis (MPGN) has led to a dramatic shift in its classification into two subgroups: immune complex-mediated MPGN and complement-mediated MPGN, consisting of dense deposit disease and C3 glomerulonephritis (C3GN). (biomedcentral.com)
The latter embraces dense deposit disease (DDD) and examples of MPGN type I and III in which immunofluorescence reveals exclusive or predominant C3 deposits, now termed C3 glomerulonephritis (C3GN) [ 1 - 7 ]. (biomedcentral.com)
Activation of the complement system through the classic pathway by immune complexes or direct cell-mediated injury in ANCA-associated glomerulonephritis results in the recruitment of inflammatory cellular infiltrates (eg, lymphocytes, macrophages, neutrophils), proliferation of the mesangial and endothelial cells, and necrosis. (medscape.com)
Therefore, urinalysis during active inflammation (or glomerulonephritis) characteristically shows an active urinary sediment, with RBCs or casts, WBCs or casts, and variable degrees of proteinuria (ie, nephritic pattern). (medscape.com)
[10] A 2012 review considers DDD to be in a continuum with C3 glomerulonephritis , [11] one reason the use of the type I to type III classification system is falling out of favour. (mdwiki.org)

Individuals with C3G typically present with hematuria, proteinuria, hematuria and proteinuria, acute nephritic syndrome or nephrotic syndrome, and low levels of the complement component C3. (nih.gov)
Nephrotic syndrome was confirmed (edema, proteinuria 4 - 18 g/day, hypoalbuminemia 21 - 28 g/L, typically changed proteinogram and lipidogram) with some additional elements of nephritic syndrome (microhematuria, hypertension). (biomedcentral.com)
Glomerular disease can cause proteinuria or hematuria, leading to nephrotic or nephritic syndromes, respectively. (clinicaladvisor.com)
Nephritic syndrome is characterized by red blood cells and casts on urine microscopy, hypertension, renal insufficiency, mild proteinuria and edema. (clinicaladvisor.com)
3 g urine protein/day), nephritic syndrome (proteinuria + hematuria), or isolated hematuria. (basicmedicalkey.com)
This was a 13-year-old girl presenting with fever, chest and abdominal pain, acute kidney injury, nephrotic-range proteinuria and low complement levels. (pubfacts.com)

C3 nephritic factors are autoantibodies that prolong the half-life or prevent regulation of the alternative pathway C3 convertase, resulting in uncontrolled complement activation. (nih.gov)

C3 glomerulopathy (C3G) is a complex ultra-rare complement-mediated renal disease caused by uncontrolled activation of the complement alternative pathway (AP) in the fluid phase (as opposed to cell surface) that is rarely inherited in a simple mendelian fashion. (nih.gov)
The first is driven by classical complement pathway activation, while the second is believed to be associated with complement alternative pathway (AP) dysregulation and is a new entity, C3 glomerulopathy [ 3 ]. (biomedcentral.com)
Predominant C3 deposits detected by immunofluorescence define C3 glomerulopathy but its original definition as "C3 only" appeared too stringent if the goal of the diagnosis is to identify all candidates for evaluation of complement AP dysregulation. (biomedcentral.com)

Adipocytes synthesize C3, factor B, and factor D (adipsin), which allows C3bBb to be formed locally, but which usually does not result in the activation of the terminal lytic part of the complement pathway (C5-9).The IgG antibody, C3Nef, prevents the alternative complement C3-convertase C3Bb from dissociative inactivation, resulting in adipocyte lysis. (medscape.com)
This special IgG autoantibody is called " nephritic factor" (or C3NeF), and is responsible for some of the cases of type 1 MPGN. (osmosis.org)
[ 4 ] Patients with MPGN are more likely to have low C3 levels and the presence of C3 nephritic factor (C3NeF). (medscape.com)
Activation of an alternate complement pathway, C3 hypocomplementemia with lysis of adipocytes induced by C3NeF, has been implicated. (medscape.com)

Over time, many of these immune complexes might find their way to the glomerulus, and they cause an activation of the classical complement pathway, leading to complement protein deposition right along side the immune complex deposits. (osmosis.org)
It is believed to be associated with the classical complement pathway . (mdwiki.org)

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. (medscape.com)

C3 nephritic factor is a serum immunoglobulin G that interacts with the C3bBb alternative pathway convertase to activate C3. (medscape.com)
Is either entirely negative or has focal and weak mesangial C3 or IgM immunoglobulin deposits. (basicmedicalkey.com)
DDD is associated with deposition of complement C3 within the glomeruli with little or no staining for immunoglobulin. (mdwiki.org)
The presence of C3 without significant immunoglobulin suggested to early investigators that DDD was due to abnormal activation of the complement alternative pathway (AP). (mdwiki.org)

The presence of autoantibodies (immunoglobulins) in the serum that react against C3 convertase (C3bBb). (nih.gov)
The high content of anti-idiotypes against autoantibodies in IVIG facilitates its ability to neutralize autoantibodies, as is shown in patients with acquired hemophilia due to autoantibodies against factor VIII . (medscape.com)

Glomerular disease may manifest with varying degrees of nephrotic and nephritic features. (clinicaladvisor.com)

We describe a clinical case of a 16 year-old boy with C3GN, mediated by complement AP dysregulation, which appeared to be triggered by immune complex-mediated MPGN. (biomedcentral.com)
Most cases are associated with the dysregulation of the alternative complement pathway . (mdwiki.org)

In persons with Kawasaki disease and dermatomyositis, IVIG is thought to inhibit the generation of membrane attack complexes (C5b-C9) and subsequent complement-mediated tissue damage by binding the activated components C3b and C4b, thus preventing their deposition on target surfaces. (medscape.com)
The results of in vitro C3 uptake studies evaluating the effect of IVIG on the clearance of pre-opsonized cells suggest that IVIG produces a kinetic depression of C3 uptake and modifies the process of complement fragment deposition on erythrocytes. (medscape.com)

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. (nih.gov)

A ubiquitously expressed complement receptor that binds COMPLEMENT C3B and COMPLEMENT C4B and serves as a cofactor for their inactivation. (childrensmercy.org)

The factors correlating with response at 6 months were older age at diagnosis, hypertension, activity, and chronicity indices and duration of symptoms prior to therapy. (indianjnephrol.org)

The activation of complement by direct contact with polysaccharides located on yeast cells, bacteria, or protozoans. (thefreedictionary.com)

Model of the adipocyte destruction in acquired partial lipodystrophy showing complement activation at the adipocyte surface resulting in adipocyte lysis. (medscape.com)

Clinicians and pathologists should be aware that, in some patients, an underlying genetic or acquired complement alternative pathway abnormality can be masked by an initial immune complex-mediated mechanism, which subsequently triggers an unbalanced excessive continual driving of complement terminal pathway activation and the development of C3GN. (biomedcentral.com)

In this paper, we review currently available evidence to support the hypothesis that the etiology of most forms of GN are likely infections, and review how the immune response to infectious agents, modified by both genetic and epigenetic factors, could lead to the autoimmune processes that we now know mediate GN ( Table 1 , Figures 1 and 2). (medscape.com)
The presence of immune complexes in the skin and kidney biopsies, as well as the profound decrease in the serum C3/C4 level coinciding with vasculitic and nephritic flares, suggested a dominant role of the autoimmune B-cell pathway in causing these manifestations. (medscape.com)

IgA antiphospholipid antibodies are an independent risk factor for thrombosis. (machaondiagnostics.com)

On the flip side, since C3 convertase usually only exists for a short time, there could also be inappropriate activation if there is an IgG antibody that actually binds to C3 convertase, which makes the C3 convertase more stable, causing it to exist for longer periods of time, which means it keeps on converting C3 to C3a and C3b. (osmosis.org)

If the family history is positive for renal disease, evaluation of apparently asymptomatic at-risk relatives can include molecular genetic testing (if the pathogenic variants in the family are known), urinalysis, and comprehensive analysis of the complement system. (nih.gov)

Forty-two of 48 nephritic factors tested prevented convertase decay by factor H, and most of these by decay accelerating factor (28) and complement receptor 1 (34). (nih.gov)

Representative properdin-independent nephritic factors had no effect on C5 cleavage and terminal pathway activity, while properdin-dependent nephritic factors enhanced activity. (nih.gov)
Complement inhibition with a terminal pathway blocker may alter disease course in some individuals. (nih.gov)

Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors. (rareneurologynews.com)

Specifically, with this pathway, C3 is converted to C3a and C3b by the enzyme C3 convertase. (osmosis.org)

To that end, we have multiple tests that offer you an in-depth look at your kidney function so you can better gauge your risk factors for chronic kidney disease (CKD). (ultalabtests.com)

[1] The underlying mechanism is believed to involve immune complexes building up in the kidneys and activating the complement system . (mdwiki.org)

angiogenesis factor a substance that causes the growth of new blood vessels, found in tissues with high metabolic requirements such as cancers and the retina. (thefreedictionary.com)

Adipocytes synthesize factor D, the limiting component of the alternative complement pathway, which cleaves C3-bound factor B to its active enzymatic form. (medscape.com)
factor B a complement component that participates in the alternative complement pathway. (thefreedictionary.com)
Hereditary angioedema (HAE) is a rare autosomal dominant condition caused by a deficiency or dysfunction of C1 esterase inhibitor (C1-INH) that normally blocks activation of C1, the first component of the complement cascade. (emjreviews.com)

Types include granulocyte , granulocyte-macrophage , and macrophage colony-stimulating factors . (thefreedictionary.com)

Close monitoring of renal function by a nephrologist with familiarity with the C3G disease spectrum, complete biannual assessment of the complement pathway, periodic eye examinations to evaluate the fundus. (nih.gov)
2. a preparation of factor VIII administered intravenously for the prevention or treatment of hemorrhage in patients with hemophilia A and the treatment of von Willebrand disease , hypofibrinogenemia , and coagulation factor XIII deficiency. (thefreedictionary.com)
The disproportionate increase in the burden of cardiovascular disease in patients with CKD may be significantly contributed by nontraditional risk factors. (indianjnephrol.org)

Another form of lipodystrophy that fits the classification of "acquired partial" not involving the complement pathway is associated with hematopoietic stem cell transplantation (HSCT) to treat leukemia or neuroblastoma. (medscape.com)

It's used to evaluate the level of kidney dysfunction in patients with known risk factors of CKD. (ultalabtests.com)

Diseases4

Chemicals and Drugs46

Analytical, Diagnostic and Therapeutic Techniques and Equipment2

Phenomena and Processes3

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

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

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

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

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

Membranoproliferative glomerulonephritis. (6/32)

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

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

Complement C3 Nephritic Factor

Complement C3

Complement C4

Complement C3-C5 Convertases

Complement Inactivator Proteins

Complement Activation

Complement C5

Complement System Proteins

Complement C4a

Complement Activating Enzymes

Complement C5 Convertase, Classical Pathway

Glomerulonephritis, Membranoproliferative

Properdin

Complement C3a

Complement Hemolytic Activity Assay

Complement C1q

Complement Pathway, Alternative

Complement C5a

Complement Pathway, Classical

Complement C4b

Lipodystrophy

Complement C3b

Complement C3 Convertase, Classical Pathway

Complement C6

Complement C3c

Complement Factor H

Complement C3d

Complement C2

Complement Factor D

Complement C9

Receptors, Complement

Complement C1s

Complement Membrane Attack Complex

Antigens, CD55

Glomerulonephritis

Complement C1r

Nephritis

Complement C7

Complement Factor B

Complement C8

Complement C1

Receptors, Complement 3b

Complement C5b

Complement C2a

Receptor, Anaphylatoxin C5a

Autoantibodies

Immunoglobulin G

Complement Inactivating Agents

Complement C1 Inactivator Proteins

Receptors, Complement 3d

Anaphylatoxins

Complement Fixation Tests

Complement Factor I

Complement C4b-Binding Protein

Complement C3b Inactivator Proteins

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

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

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

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

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

Membranoproliferative glomerulonephritis. (6/32)

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

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

Meningococcal meningitis associated with persistent hypocomplementaemia due to circulating C3 nephritic factor. - Experimental...

Molecular analysis of C3 allotypes in patients with nephritic factor

Metabolism of C3 and Glycine-Rich β Glycoprotein (GBG) in Hypocomplementaemia | Clinical Science | Portland Press

Barraquer-Simons syndrome - Wikipedia

Pilot Study of Rituximab for Membranoproliferative Glomerulonephritis - Full Text View - ClinicalTrials.gov

Factor H deficiency

What is the treatment of membranoproliferative glomerulonephritis (MPGN)?

Evaluation and treatment of membranoproliferative glomerulonephritis

Overactivity of alternative pathway convertases in patients with complement-mediated renal diseases

Nephron Power: MPGN revisited

Membranoproliferative glomerulonephritis with predominant IgG2 and IgG3 deposition in a patient with IgG4-related disease | BMC...

Membranoproliferative glomerulonephritis type 2

Sulodexide Treatment in Patients With Dense Deposit Disease - Full Text View - ClinicalTrials.gov

Response of hepatitis B induced membranoproliferative glomerulonephritis to HAART | Sexually Transmitted Infections

Life With Chronic Kidney Disease - Nephrotic Syndrome Info and Tips

Purified IgG fraction | New Scientific Company

A novel CFHR5 mutation associated with C3 glomerulonephritis in a Turkish girl | AVESİS

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