C3

Summary

Gene Symbol: C3
Description: complement C3
Alias: AHUS5, ARMD9, ASP, C3a, C3b, CPAMD1, HEL-S-62p, complement C3, C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1, C3a anaphylatoxin, acylation-stimulating protein cleavage product, complement component 3, complement component C3a, complement component C3b, epididymis secretory sperm binding protein Li 62p, prepro-C3
Species: human
Products:     C3

Top Publications

  1. Lambris J, Lao Z, Oglesby T, Atkinson J, Hack C, Becherer J. Dissection of CR1, factor H, membrane cofactor protein, and factor B binding and functional sites in the third complement component. J Immunol. 1996;156:4821-32 pubmed
    ..of iC3 by factor I and CR1, or H, CR1 and H bind to at least two sites on C3 and that the MCP binding site(s) on C3b are different from those for CR1...
  2. Mocco J, Wilson D, Komotar R, Sughrue M, Coates K, Sacco R, et al. Alterations in plasma complement levels after human ischemic stroke. Neurosurgery. 2006;59:28-33; discussion 28-33 pubmed
    ..We compared peripheral blood levels of complement factor 3a (C3a), 5a (C5a), and sC5b-9 drawn from 15 patients on poststroke Days 1, 2, 3, 7, 14, 21, and 28 to age-, race/ethnicity-..
  3. Servais A, Noel L, Roumenina L, Le Quintrec M, Ngo S, Dragon Durey M, et al. Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. Kidney Int. 2012;82:454-64 pubmed publisher
    Dense deposit disease and glomerulonephritis with isolated C3 deposits are glomerulopathies characterized by deposits of C3 within or along the glomerular basement membrane...
  4. Nishida N, Walz T, Springer T. Structural transitions of complement component C3 and its activation products. Proc Natl Acad Sci U S A. 2006;103:19737-42 pubmed
    ..In the cleavage product C3b, the position of the thioester domain moves approximately 100 Angstrom, which becomes covalently coupled to ..
  5. Premanandan C, Storozuk C, Clay C, Lairmore M, Schlesinger L, Phipps A. Complement protein C3 binding to Bacillus anthracis spores enhances phagocytosis by human macrophages. Microb Pathog. 2009;46:306-14 pubmed publisher
    ..anthracis spores by human monocyte-derived macrophages in the presence of nonimmune human serum. Here we show that C3b is bound to B. anthracis spores and is activated through the classical pathway by IgG bound to the spore surface...
  6. Bhattacharjee A, Lehtinen M, Kajander T, Goldman A, Jokiranta T. Both domain 19 and domain 20 of factor H are involved in binding to complement C3b and C3d. Mol Immunol. 2010;47:1686-91 pubmed publisher
    ..19 and 20 of FH are essential in target discrimination and are known to contain binding sites for the C3d part of C3b, heparin, and endothelial cells...
  7. Wan J, Fukuda N, Endo M, Tahira Y, Yao E, Matsuda H, et al. Complement 3 is involved in changing the phenotype of human glomerular mesangial cells. J Cell Physiol. 2007;213:495-501 pubmed
    ..HMCs also expressed C3a receptor mRNA and protein. Exogenous C3a stimulated DNA synthesis in HMCs in a dose-dependent manner...
  8. Rhodes B, Hunnangkul S, Morris D, Hsaio L, Graham D, Nitsch D, et al. The heritability and genetics of complement C3 expression in UK SLE families. Genes Immun. 2009;10:525-30 pubmed publisher
    As the central component of the complement system, C3 has sensory and effector functions bridging innate and adaptive immunity...
  9. Ferreira V, Herbert A, Cortes C, McKee K, Blaum B, Esswein S, et al. The binding of factor H to a complex of physiological polyanions and C3b on cells is impaired in atypical hemolytic uremic syndrome. J Immunol. 2009;182:7009-18 pubmed publisher
    ..Its two C-terminal domains (CCP 19-20) anchor fH to self-surfaces where it prevents C3b amplification in a process requiring its N-terminal four domains...

More Information

Publications102 found, 100 shown here

  1. Shaw C, Storek M, Young K, Kovacs J, Thurman J, Holers V, et al. Delineation of the complement receptor type 2-C3d complex by site-directed mutagenesis and molecular docking. J Mol Biol. 2010;404:697-710 pubmed publisher
    The interactions between the complement receptor type 2 (CR2) and the C3 complement fragments C3d, C3dg, and iC3b are essential for the initiation of a normal immune response...
  2. Saleh J, Summers L, Cianflone K, Fielding B, Sniderman A, Frayn K. Coordinated release of acylation stimulating protein (ASP) and triacylglycerol clearance by human adipose tissue in vivo in the postprandial period. J Lipid Res. 1998;39:884-91 pubmed
    The objective of this study was to determine whether Acylation Stimulating Protein (ASP) is generated in vivo by human adipose tissue during the postprandial period...
  3. Wiesmann C, Katschke K, Yin J, Helmy K, Steffek M, Fairbrother W, et al. Structure of C3b in complex with CRIg gives insights into regulation of complement activation. Nature. 2006;444:217-20 pubmed
    ..After exposure to pathogens, the third component of the complement system, C3, is cleaved to C3b which, after recruitment of factor B, initiates formation of the alternative pathway convertases...
  4. Phillips C, Kesse Guyot E, Ahluwalia N, McManus R, Hercberg S, Lairon D, et al. Dietary fat, abdominal obesity and smoking modulate the relationship between plasma complement component 3 concentrations and metabolic syndrome risk. Atherosclerosis. 2012;220:513-9 pubmed publisher
    ..b>Complement component 3 (C3) is a novel cardiometabolic risk factor...
  5. Matsuyama W, Nakagawa M, Takashima H, Muranaga F, Sano Y, Osame M. Molecular analysis of hereditary deficiency of the third component of complement (C3) in two sisters. Intern Med. 2001;40:1254-8 pubmed
    We report two sisters with hereditary deficiency of the third complement component (C3) and a homozygous mutation at C3303G (Tyr1081Stop) of the gene. They developed systemic lupus erythematosus-like symptoms during adolescence...
  6. Wu Y, Zhang J, Wen Y, Wang H, Zhang M, Cianflone K. Increased acylation-stimulating protein, C-reactive protein, and lipid levels in young women with polycystic ovary syndrome. Fertil Steril. 2009;91:213-9 pubmed publisher
    ..changes that are independent of body size in the lipogenic adipokine acylation-stimulating protein (ASP), complement C3 (ASP precursor), and C-reactive protein (CRP) in subjects with polycystic ovary syndrome (PCOS)...
  7. Yavuz S, Acarturk T. Acquired partial lipodystrophy with C3 hypocomplementemia and antiphospholipid and anticardiolipin antibodies. Pediatr Dermatol. 2010;27:504-8 pubmed publisher
    ..We present three boys with acquired partial lipodystrophy having C(3) hypocomplementemia. In addition, one of them had antiphospholipid and anticardiolipin antibodies. ..
  8. Botto M, Fong K, So A, Barlow R, Routier R, Morley B, et al. Homozygous hereditary C3 deficiency due to a partial gene deletion. Proc Natl Acad Sci U S A. 1992;89:4957-61 pubmed
    The molecular mechanism of C3 deficiency in an Afrikaans patient with recurrent pyogenic infections was studied. Restriction enzyme analysis showed a gene deletion of 800 base pairs (bp) mapping to the alpha chain of C3...
  9. Szakonyi G, Guthridge J, Li D, Young K, Holers V, Chen X. Structure of complement receptor 2 in complex with its C3d ligand. Science. 2001;292:1725-8 pubmed
    ..These results provide a detailed understanding of receptor-ligand interactions in this protein family and reveal potential target sites for molecular drug design. ..
  10. Despriet D, van Duijn C, Oostra B, Uitterlinden A, Hofman A, Wright A, et al. Complement component C3 and risk of age-related macular degeneration. Ophthalmology. 2009;116:474-480.e2 pubmed publisher
    To explore the association between polymorphisms in the complement component 3 (C3) gene and age-related macular degeneration (AMD), and to investigate the modifying effect of complement factor H (CFH) Y402H, LOC387715 A69S and smoking...
  11. Reynolds R, Hartnett M, Atkinson J, Giclas P, Rosner B, Seddon J. Plasma complement components and activation fragments: associations with age-related macular degeneration genotypes and phenotypes. Invest Ophthalmol Vis Sci. 2009;50:5818-27 pubmed publisher
    ..Plasma complement components (C3, CFB, CFI, CFH, and factor D) and activation fragments (Bb, C3a, C5a, iC3b, and SC5b-9) were analyzed...
  12. Spencer K, Olson L, Anderson B, Schnetz Boutaud N, Scott W, Gallins P, et al. C3 R102G polymorphism increases risk of age-related macular degeneration. Hum Mol Genet. 2008;17:1821-4 pubmed publisher
    ..factor B (CFB) genes has targeted the search for additional loci to the alternative complement cascade, of which C3 is a major component...
  13. Oran A, Isenman D. Identification of residues within the 727-767 segment of human complement component C3 important for its interaction with factor H and with complement receptor 1 (CR1, CD35). J Biol Chem. 1999;274:5120-30 pubmed
    ..employing blocking antibodies and synthetic peptides have implicated the 727-767 segment at the NH2 terminus of C3b alpha'-chain as contributing to the interactions with factor B, factor H, and CR1...
  14. Werfel T, Kirchhoff K, Wittmann M, Begemann G, Kapp A, Heidenreich F, et al. Activated human T lymphocytes express a functional C3a receptor. J Immunol. 2000;165:6599-605 pubmed
    The C3a molecule is an anaphylatoxin of the C system with a wide spectrum of proinflammatory effects predominantly on cells of myeloid origin...
  15. Maga T, Nishimura C, Weaver A, Frees K, Smith R. Mutations in alternative pathway complement proteins in American patients with atypical hemolytic uremic syndrome. Hum Mutat. 2010;31:E1445-60 pubmed publisher
    ..complement factor H-related 5 (CFHR5), complement factor I (CFI), CD46 (MCP), complement factor B (CFB), complement component 3 (C3) and thrombomodulin (THBD). Since aHUS is rare, mutation rate data in large patient cohorts are scarce...
  16. Olsson S, Stokowska A, Holmegaard L, Jood K, Blomstrand C, Pekna M, et al. Genetic variation in complement component C3 shows association with ischaemic stroke. Eur J Neurol. 2011;18:1272-4 pubmed publisher
    The aim of this study was to investigate whether genetic variation at the third complement component (C3) locus is associated with ischaemic stroke (IS)...
  17. Mogilenko D, Kudriavtsev I, Trulioff A, Shavva V, Dizhe E, Missyul B, et al. Modified low density lipoprotein stimulates complement C3 expression and secretion via liver X receptor and Toll-like receptor 4 activation in human macrophages. J Biol Chem. 2012;287:5954-68 pubmed publisher
    b>Complement C3 is a pivotal component of three cascades of complement activation. C3 is expressed in human atherosclerotic lesions and is involved in atherogenesis...
  18. Ylitalo K, Nuotio I, Viikari J, Auwerx J, Vidal H, Taskinen M. C3, hormone-sensitive lipase, and peroxisome proliferator-activated receptor gamma expression in adipose tissue of familial combined hyperlipidemia patients. Metabolism. 2002;51:664-70 pubmed
    This study aimed to assess the role of complement C3, hormone-sensitive lipase (HSL), and peroxisome proliferator-activated receptor gamma (PPARgamma) gene expression in familial combined hyperlipidemia (FCHL)...
  19. Licht C, Heinen S, Jozsi M, Löschmann I, Saunders R, Perkins S, et al. Deletion of Lys224 in regulatory domain 4 of Factor H reveals a novel pathomechanism for dense deposit disease (MPGN II). Kidney Int. 2006;70:42-50 pubmed
    ..severely reduced cofactor and decay-accelerating activity, as well as reduced binding to the central complement component C3b. However, cell-binding activity of the mutant protein was normal and comparable to wild-type Factor H...
  20. Seddon J, Reynolds R, Maller J, Fagerness J, Daly M, Rosner B. Prediction model for prevalence and incidence of advanced age-related macular degeneration based on genetic, demographic, and environmental variables. Invest Ophthalmol Vis Sci. 2009;50:2044-53 pubmed publisher
    ..3 (95% CI, 0.1-0.7) for C2 E318D; 0.3 (95% CI, 0.1-0.5) for CFB; and 3.6 (95% CI, 1.4-9.4) for C3 R102G, comparing the homozygous risk/protective genotypes to the referent genotypes...
  21. Tortajada A, Montes T, Martinez Barricarte R, Morgan B, Harris C, de Cordoba S. The disease-protective complement factor H allotypic variant Ile62 shows increased binding affinity for C3b and enhanced cofactor activity. Hum Mol Genet. 2009;18:3452-61 pubmed publisher
    ..surface plasmon resonance analyses, we show here that the protective fH-Ile(62) variant binds more efficiently to C3b than fH-Val(62) and competes better with factor B in proconvertase formation...
  22. Morgan H, Schmidt C, Guariento M, Blaum B, Gillespie D, Herbert A, et al. Structural basis for engagement by complement factor H of C3b on a self surface. Nat Struct Mol Biol. 2011;18:463-70 pubmed publisher
    Complement factor H (FH) attenuates C3b molecules tethered by their thioester domains to self surfaces and thereby protects host tissues...
  23. Endo M, Ohi H, Ohsawa I, Matsushita M, Fujita T. Glomerular deposition of mannose-binding lectin (MBL) indicates a novel mechanism of complement activation in IgA nephropathy. Nephrol Dial Transplant. 1998;13:1984-90 pubmed
    ..The deposited MBL/MASP-1 was observed to associate with C3b/C3c and C5b-9 but not with IgG, IgM, C1q, C4c, or properdin...
  24. Kalant D, MacLaren R, Cui W, Samanta R, Monk P, Laporte S, et al. C5L2 is a functional receptor for acylation-stimulating protein. J Biol Chem. 2005;280:23936-44 pubmed
    ..001, where basal = 100%) and glucose transport (168 +/- 21%, 10 microM ASP, p < 0.001). C3a similarly stimulates TGS (163 +/- 12%, p < 0.001), but C5a and C5a des-Arg have no effect...
  25. Zhang J, Wang C, Tang Y, Peng H, Ye Z, Li C, et al. Serum immunoglobulin A/C3 ratio predicts progression of immunoglobulin A nephropathy. Nephrology (Carlton). 2013;18:125-31 pubmed publisher
    The serum immunoglobulin A (IgA)/C3 ratio has been shown to be a good predictor of histological lesions and prognosis for patients with IgA nephropathy (IgAN) in Japanese. But its validity in the Chinese population is unclear...
  26. Murray I, Kohl J, Cianflone K. Acylation-stimulating protein (ASP): structure-function determinants of cell surface binding and triacylglycerol synthetic activity. Biochem J. 1999;342 ( Pt 1):41-8 pubmed
    Acylation-stimulating protein (ASP or C3adesArg) is a potent lipogenic factor in human and murine adipocytes and fibroblasts. The arginated form of ASP, i.e...
  27. Francis P, Hamon S, Ott J, Weleber R, Klein M. Polymorphisms in C2, CFB and C3 are associated with progression to advanced age related macular degeneration associated with visual loss. J Med Genet. 2009;46:300-7 pubmed publisher
    ..0001, odds ratio (OR) 0.35, 95% confidence interval (CI) 0.2 to 0.6), CFB (p = 0.0001, OR 0.35, 95% CI 0.2 to 0.6), C3 (p = 0.0001, OR 3.91, 95% CI 1.94 to 7.88), APOE (epsilon4, p = 0.01, OR 0.50, 95% CI 0.29 to 0.86) and VEGFA (p = 0...
  28. Haupt K, Reuter M, van den Elsen J, Burman J, Hälbich S, Richter J, et al. The Staphylococcus aureus protein Sbi acts as a complement inhibitor and forms a tripartite complex with host complement Factor H and C3b. PLoS Pathog. 2008;4:e1000250 pubmed publisher
    ..Factor H binds to Sbi in combination with C3b or C3d, and forms tripartite SbiratioC3ratioFactor H complexes...
  29. Schraufstatter I, DiScipio R, Zhao M, Khaldoyanidi S. C3a and C5a are chemotactic factors for human mesenchymal stem cells, which cause prolonged ERK1/2 phosphorylation. J Immunol. 2009;182:3827-36 pubmed publisher
    ..complement activation occurs whenever there is tissue damage, the effects of the complement activation products C3a and C5a on MSCs were examined...
  30. Van Greevenbroek M, Jacobs M, van der Kallen C, Blaak E, Jansen E, Schalkwijk C, et al. Human plasma complement C3 is independently associated with coronary heart disease, but only in heavy smokers (the CODAM study). Int J Cardiol. 2012;154:158-62 pubmed publisher
    b>Complement C3 is an emerging risk factor for coronary heart disease (CHD) and is particularly increased in the metabolic syndrome. A direct effect of smoking on structure and function of complement C3 has been suggested...
  31. Roumenina L, Frimat M, Miller E, Provot F, Dragon Durey M, Bordereau P, et al. A prevalent C3 mutation in aHUS patients causes a direct C3 convertase gain of function. Blood. 2012;119:4182-91 pubmed publisher
    ..Here, we report 14 sporadic aHUS patients carrying the same mutation, R139W, in the complement C3 gene...
  32. Lhotta K, Janecke A, Scheiring J, Petzlberger B, Giner T, Fally V, et al. A large family with a gain-of-function mutation of complement C3 predisposing to atypical hemolytic uremic syndrome, microhematuria, hypertension and chronic renal failure. Clin J Am Soc Nephrol. 2009;4:1356-62 pubmed publisher
    ..Recently, heterozygous gain-of-function mutations in the complement C3 gene have been found in patients with aHUS. A large family with a C3 R570Q mutation is described...
  33. Ueda Y, Nagasawa K, Tsukamoto H, Horiuchi T, Nishizaka H, Ikeda K, et al. Production of the third and fourth component of complement (C3, C4) by smooth muscle cells. Immunology. 1996;89:183-8 pubmed
    Production of the third and fourth components of complement (C3, C4) by smooth muscle cells was investigated by using normal human aortic smooth muscle cells (AoSMC), human smooth muscle cell line (G402) and vascular smooth muscle cells ..
  34. Cianflone K, Zakarian R, Couillard C, Delplanque B, Despres J, Sniderman A. Fasting acylation-stimulating protein is predictive of postprandial triglyceride clearance. J Lipid Res. 2004;45:124-31 pubmed
    Postprandial plasma triglyceride (ppTG) and NEFA clearance were stratified by plasma acylation-stimulating protein (ASP) and gender to determine the contribution of fasting ASP in a normal population (70 men; 71 women)...
  35. Wu J, Wu Y, Ricklin D, Janssen B, Lambris J, Gros P. Structure of complement fragment C3b-factor H and implications for host protection by complement regulators. Nat Immunol. 2009;10:728-33 pubmed publisher
    ..FH by solving the crystal structure of the first four domains of FH in complex with its target, complement fragment C3b. FH interacted with multiple domains of C3b, covering a large, extended surface area...
  36. Janssen B, Huizinga E, Raaijmakers H, Roos A, Daha M, Nilsson Ekdahl K, et al. Structures of complement component C3 provide insights into the function and evolution of immunity. Nature. 2005;437:505-11 pubmed
    ..Activation of component C3 (1,641 residues) is central to the three complement pathways and results in inflammation and elimination of self and ..
  37. Yates J, Sepp T, Matharu B, Khan J, Thurlby D, Shahid H, et al. Complement C3 variant and the risk of age-related macular degeneration. N Engl J Med. 2007;357:553-61 pubmed
    ..6 (95% CI, 1.6 to 4.1). The estimated population attributable risk for C3F was 22%. Complement C3 is important in the pathogenesis of age-related macular degeneration...
  38. Burman J, Leung E, Atkins K, O Seaghdha M, Lango L, Bernado P, et al. Interaction of human complement with Sbi, a staphylococcal immunoglobulin-binding protein: indications of a novel mechanism of complement evasion by Staphylococcus aureus. J Biol Chem. 2008;283:17579-93 pubmed publisher
    ..IV on its own (Sbi-IV), bind complement component C3 via contacts involving both the C3dg fragment and the C3a anaphylatoxin domain...
  39. Sartz L, Olin A, Kristoffersson A, Ståhl A, Johansson M, Westman K, et al. A novel C3 mutation causing increased formation of the C3 convertase in familial atypical hemolytic uremic syndrome. J Immunol. 2012;188:2030-7 pubmed publisher
    ..Patient sera exhibited more C3 cleavage and higher levels of C3a. The C3 mutation resulted in increased C3 binding to factor B and increased net formation of the C3 convertase, ..
  40. Cain S, Monk P. The orphan receptor C5L2 has high affinity binding sites for complement fragments C5a and C5a des-Arg(74). J Biol Chem. 2002;277:7165-9 pubmed
    ..C5L2 also has a moderate affinity for anaphylatoxin C3a, but cross-competition studies suggest that C3a binds to a distinct site from C5a...
  41. Reynolds R, Rosner B, Seddon J. Serum lipid biomarkers and hepatic lipase gene associations with age-related macular degeneration. Ophthalmology. 2010;117:1989-95 pubmed publisher
    ..Participants were genotyped for 8 variants associated with AMD: 2 CFH variants, C2, CFB, C3, CFI, the ARMS2/HTRA1 gene region, and LIPC...
  42. Sethi S, Fervenza F, Zhang Y, Nasr S, Leung N, Vrana J, et al. Proliferative glomerulonephritis secondary to dysfunction of the alternative pathway of complement. Clin J Am Soc Nephrol. 2011;6:1009-17 pubmed publisher
    ..A recently described entity referred to as GN with C3 deposition (GN-C3) bears many similarities to DDD...
  43. Dolmer K, Sottrup Jensen L. Disulfide bridges in human complement component C3b. FEBS Lett. 1993;315:85-90 pubmed
    The disulfide bridges of human complement component C3b, derived from C3 by removal of the 77-residue C3a, have been determined...
  44. Chen W, Stambolian D, Edwards A, Branham K, Othman M, Jakobsdottir J, et al. Genetic variants near TIMP3 and high-density lipoprotein-associated loci influence susceptibility to age-related macular degeneration. Proc Natl Acad Sci U S A. 2010;107:7401-6 pubmed publisher
    ..results validate AMD susceptibility loci near CFH (P < 10(-75)), ARMS2 (P < 10(-59)), C2/CFB (P < 10(-20)), C3 (P < 10(-9)), and CFI (P < 10(-6))...
  45. DiScipio R. Ultrastructures and interactions of complement factors H and I. J Immunol. 1992;149:2592-9 pubmed
    ..Only one end of factor H associates with C3b. When bound to C3b, factor H still shows considerable conformational flexibility...
  46. Rodriguez de Cordoba S, Harris C, Morgan B, Llorca O. Lessons from functional and structural analyses of disease-associated genetic variants in the complement alternative pathway. Biochim Biophys Acta. 2011;1812:12-22 pubmed publisher
    ..A critical step in complement activation is the formation of the C3 convertase of the alternative pathway (AP), a labile bimolecular complex formed by activated fragments of the C3 and ..
  47. Fremeaux Bacchi V, Miller E, Liszewski M, Strain L, Blouin J, Brown A, et al. Mutations in complement C3 predispose to development of atypical hemolytic uremic syndrome. Blood. 2008;112:4948-52 pubmed publisher
    ..We report here mutations in the central component of the complement cascade, C3, in association with aHUS...
  48. Pei X, Li X, Bao Y, Yu W, Yan Z, Qi H, et al. Association of c3 gene polymorphisms with neovascular age-related macular degeneration in a chinese population. Curr Eye Res. 2009;34:615-22 pubmed
    The purpose of this study was to determine whether the genetic polymorphisms of complement factor 3 (C3) are associated with neovascular age-related macular degeneration (AMD) in the Chinese population...
  49. Mazumdar B, Kim H, Meyer K, Bose S, Di Bisceglie A, Ray R, et al. Hepatitis C virus proteins inhibit C3 complement production. J Virol. 2012;86:2221-8 pubmed publisher
    The third component of human complement (C3) plays a central role in innate immune function as its activation is required to trigger classical as well as alternative complement pathways...
  50. Smith J, Cianflone K, Martin J, Poirier P, Broderick T, Noel M. Plasma adipokine and hormone changes in mountaineers on ascent to 5300 meters. Wilderness Environ Med. 2011;22:107-14 pubmed publisher
    ..05) with no change in leptin, complement C3 (C3), high sensitivity C-reactive protein (hsCRP) or cortisol levels throughout the mountain ascent from SL to ..
  51. Kim S, Koo H, Lim B, Oh H, Yoo D, Shin D, et al. Decreased circulating C3 levels and mesangial C3 deposition predict renal outcome in patients with IgA nephropathy. PLoS ONE. 2012;7:e40495 pubmed publisher
    Mesangial C3 deposition is frequently observed in patients with IgA nephropathy (IgAN). However, the role of complement in the pathogenesis or progression of IgAN is uncertain...
  52. Park K, Fridley B, Ryu E, Tosakulwong N, Edwards A. Complement component 3 (C3) haplotypes and risk of advanced age-related macular degeneration. Invest Ophthalmol Vis Sci. 2009;50:3386-93 pubmed publisher
    Nonsynonymous coding single nucleotide polymorphisms (nsSNPs) in complement component 3 (C3) alter the risk of age-related macular degeneration (AMD). This was a study of the effect of haplotypes across C3 on AMD risk...
  53. Kerns E, Rozansky D, Troxell M. Evolution of immunoglobulin deposition in C3-dominant membranoproliferative glomerulopathy. Pediatr Nephrol. 2013;28:2227-31 pubmed publisher
    ..proposed subcategory of glomerular disease with features including membranoproliferative glomerulonephritis (MPGN), C3-dominant immunofluorescent staining without appreciable immunoglobulin deposition, and electron-dense deposits...
  54. Morikis D, Lambris J. The electrostatic nature of C3d-complement receptor 2 association. J Immunol. 2004;172:7537-47 pubmed
  55. Medjeral Thomas N, O Shaughnessy M, O Regan J, Traynor C, Flanagan M, Wong L, et al. C3 glomerulopathy: clinicopathologic features and predictors of outcome. Clin J Am Soc Nephrol. 2014;9:46-53 pubmed publisher
    The term C3 glomerulopathy describes renal disorders characterized by the presence of glomerular deposits composed of C3 in the absence of significant amounts of Ig...
  56. Li J, Pircher P, Schulman I, Westin S. Regulation of complement C3 expression by the bile acid receptor FXR. J Biol Chem. 2005;280:7427-34 pubmed
    ..The analysis of the human complement C3 gene reveals the presence of functional FXR response elements in the proximal promoter of C3...
  57. Maller J, Fagerness J, Reynolds R, Neale B, Daly M, Seddon J. Variation in complement factor 3 is associated with risk of age-related macular degeneration. Nat Genet. 2007;39:1200-1 pubmed
    ..We identify a nonsynonymous coding change in complement factor 3 that is strongly associated with risk of age-related macular degeneration in a large case-control sample. ..
  58. St Pierre D, Cianflone K, Smith J, Coderre L, Karelis A, Imbeault P, et al. Change in plasma acylation stimulating protein during euglycaemic-hyperinsulinaemic clamp in overweight and obese postmenopausal women: a MONET study. Clin Endocrinol (Oxf). 2009;70:539-46 pubmed publisher
    Acylation-stimulating protein (ASP) has been shown to positively stimulate fatty acid esterification and glucose uptake in adipocytes. In vitro studies demonstrate that insulin stimulates ASP secretion from adipocytes...
  59. Cui W, Lapointe M, Gauvreau D, Kalant D, Cianflone K. Recombinant C3adesArg/acylation stimulating protein (ASP) is highly bioactive: a critical evaluation of C5L2 binding and 3T3-L1 adipocyte activation. Mol Immunol. 2009;46:3207-17 pubmed publisher
    C5L2 is a recently identified receptor for C5a/C5adesArg, C3a and C3adesArg (ASP). C5a/C5adesArg bind with high affinity, with no identified activation...
  60. Vik D, Amiguet P, Moffat G, Fey M, Amiguet Barras F, Wetsel R, et al. Structural features of the human C3 gene: intron/exon organization, transcriptional start site, and promoter region sequence. Biochemistry. 1991;30:1080-5 pubmed
    The third component of human complement (C3) is a key molecule in the activation of the complement cascade. C3 cDNA fragments were used to identify seven cosmid clones that covered all but 1 kilobase pair (kb) of the C3 gene...
  61. Moore I, Strain L, Pappworth I, Kavanagh D, Barlow P, Herbert A, et al. Association of factor H autoantibodies with deletions of CFHR1, CFHR3, CFHR4, and with mutations in CFH, CFI, CD46, and C3 in patients with atypical hemolytic uremic syndrome. Blood. 2010;115:379-87 pubmed publisher
    ..In 5 patients mutations were identified: 1 in CFH, 1 in CFI, 1 in CD46, and 2 in C3. The latter observation emphasizes that multiple concurrent factors may be necessary in individual patients for ..
  62. Athanasiou Y, Voskarides K, Gale D, Damianou L, Patsias C, Zavros M, et al. Familial C3 glomerulopathy associated with CFHR5 mutations: clinical characteristics of 91 patients in 16 pedigrees. Clin J Am Soc Nephrol. 2011;6:1436-46 pubmed publisher
    ..alternative complement pathway, where loss of function mutations lead to a glomerulopathy with isolated mesangial C3 deposits without immunoglobulins. Gale et al...
  63. Mueller Ortiz S, Wanger A, Norris S. Mycobacterial protein HbhA binds human complement component C3. Infect Immun. 2001;69:7501-11 pubmed
    ..Human complement component C3 has previously been shown to mediate attachment and phagocytosis of these bacteria by mononuclear phagocytes...
  64. Hunnangkul S, Nitsch D, Rhodes B, Chadha S, Roberton C, Pessôa Lopes P, et al. Familial clustering of non-nuclear autoantibodies and C3 and C4 complement components in systemic lupus erythematosus. Arthritis Rheum. 2008;58:1116-24 pubmed publisher
    ..of non-nuclear antibodies (anti-C1q and anticardiolipin antibodies [aCL]) and depletion of complement components C3 and C4, aggregate in families. In addition, we examined relationships between anti-C1q and C3 and C4 levels...
  65. Inoue H, Mashimo Y, Funamizu M, Shimojo N, Hasegawa K, Hirota T, et al. Association study of the C3 gene with adult and childhood asthma. J Hum Genet. 2008;53:728-38 pubmed publisher
    ..It has been shown that complement-C3-deficient mice have reduced inflammation of asthmatic airways...
  66. Sethi S, Fervenza F, Zhang Y, Zand L, Vrana J, Nasr S, et al. C3 glomerulonephritis: clinicopathological findings, complement abnormalities, glomerular proteomic profile, treatment, and follow-up. Kidney Int. 2012;82:465-73 pubmed
    b>C3 glomerulonephritis (C3GN) is a recently described disorder that typically results from abnormalities in the alternative pathway (AP) of complement...
  67. Scholl H, Charbel Issa P, Walier M, Janzer S, Pollok Kopp B, Börncke F, et al. Systemic complement activation in age-related macular degeneration. PLoS ONE. 2008;3:e2593 pubmed publisher
    ..Subjects were analyzed for single nucleotide polymorphisms in factor H (CFH), factor B-C2 (BF-C2) and complement C3 (C3) genes which were previously found to be associated with AMD...
  68. Liu X, Zhao P, Tang S, Lu F, Hu J, Lei C, et al. Association study of complement factor H, C2, CFB, and C3 and age-related macular degeneration in a Han Chinese population. Retina. 2010;30:1177-84 pubmed publisher
    Genes in the complement pathway, including complement factor H (CFH), C2/BF, and C3, have been reported to be associated with age-related macular degeneration (AMD)...
  69. Reca R, Mastellos D, Majka M, Marquez L, Ratajczak J, Franchini S, et al. Functional receptor for C3a anaphylatoxin is expressed by normal hematopoietic stem/progenitor cells, and C3a enhances their homing-related responses to SDF-1. Blood. 2003;101:3784-93 pubmed
    ..human CD34(+) cells and lineage-differentiated hematopoietic progenitors express the complement anaphylatoxin C3a receptor (C3aR) and respond to C3a...
  70. Kalant D, Cain S, Maslowska M, Sniderman A, Cianflone K, Monk P. The chemoattractant receptor-like protein C5L2 binds the C3a des-Arg77/acylation-stimulating protein. J Biol Chem. 2003;278:11123-9 pubmed
    ..receptor C5L2 has recently been described as a high affinity binding protein for complement fragments C5a and C3a that, unlike the previously described C5a receptor (CD88), couples only weakly to G(i)-like G proteins (Cain, S. A...
  71. Zerbib J, Richard F, Puche N, Leveziel N, Cohen S, Korobelnik J, et al. R102G polymorphism of the C3 gene associated with exudative age-related macular degeneration in a French population. Mol Vis. 2010;16:1324-30 pubmed
    ..Our purpose was to analyze the role of the R102G polymorphism of the complement component (C3) gene in a French population, in a case-control study...
  72. Janssen B, Christodoulidou A, McCarthy A, Lambris J, Gros P. Structure of C3b reveals conformational changes that underlie complement activity. Nature. 2006;444:213-6 pubmed
    ..Central to the complement system is the activated form of C3, called C3b, which attaches covalently to target surfaces to amplify complement response, label cells for phagocytosis and ..
  73. Fritzinger D, Hew B, Thorne M, Pangburn M, Janssen B, Gros P, et al. Functional characterization of human C3/cobra venom factor hybrid proteins for therapeutic complement depletion. Dev Comp Immunol. 2009;33:105-16 pubmed publisher
    Cobra venom factor (CVF) is a structural and functional analog of complement C3 isolated from cobra venom. Both CVF and C3b can bind factor B and subsequently form the bimolecular C3/C5 convertases CVF,Bb or C3b,Bb, respectively...
  74. Onat A, Hergenc G, Can G, Kaya Z, Yuksel H. Serum complement C3: a determinant of cardiometabolic risk, additive to the metabolic syndrome, in middle-aged population. Metabolism. 2010;59:628-34 pubmed publisher
    We studied whether serum complement C3 (C3) is an independent determinant of incident cardiometabolic risk (coronary heart disease [CHD], metabolic syndrome [MetS], and type 2 diabetes mellitus)...
  75. Gilbert H, Eaton J, Hannan J, Holers V, Perkins S. Solution structure of the complex between CR2 SCR 1-2 and C3d of human complement: an X-ray scattering and sedimentation modelling study. J Mol Biol. 2005;346:859-73 pubmed
    Complement receptor type 2 (CR2, CD21) forms a tight complex with C3d, a fragment of C3, the major complement component...
  76. Winters M, Spellman D, Lambris J. Solvent accessibility of native and hydrolyzed human complement protein 3 analyzed by hydrogen/deuterium exchange and mass spectrometry. J Immunol. 2005;174:3469-74 pubmed
    Complement protein C3 is a 187-kDa (1641-aa) protein that plays a key role in complement activation and immune responses. Its hydrolyzed form, C3(H2O), is responsible for the initiation of the activation of alternative complement pathway...
  77. Ricklin D, Ricklin Lichtsteiner S, Markiewski M, Geisbrecht B, Lambris J. Cutting edge: members of the Staphylococcus aureus extracellular fibrinogen-binding protein family inhibit the interaction of C3d with complement receptor 2. J Immunol. 2008;181:7463-7 pubmed
    ..fibrinogen-binding protein (Efb) and the Efb homologous protein (Ehp) have previously been demonstrated to bind to C3 and inhibit complement activation and amplification...
  78. Maier M, Peng Y, Jiang L, Seabrook T, Carroll M, Lemere C. Complement C3 deficiency leads to accelerated amyloid beta plaque deposition and neurodegeneration and modulation of the microglia/macrophage phenotype in amyloid precursor protein transgenic mice. J Neurosci. 2008;28:6333-41 pubmed publisher
    ..To further address the role of C3 in AD pathology, we generated a complement C3-deficient amyloid precursor protein (APP) transgenic AD mouse model (APP;C3(-/-))...
  79. Scholl H, Fleckenstein M, Fritsche L, Schmitz Valckenberg S, Göbel A, Adrion C, et al. CFH, C3 and ARMS2 are significant risk loci for susceptibility but not for disease progression of geographic atrophy due to AMD. PLoS ONE. 2009;4:e7418 pubmed publisher
    ..Variants in the genes encoding complement factor H (CFH), complement component 3 (C3) and age-related maculopathy susceptibility 2 (ARMS2) have repeatedly been shown to confer significant ..
  80. Ghannam A, Pernollet M, Fauquert J, Monnier N, Ponard D, Villiers M, et al. Human C3 deficiency associated with impairments in dendritic cell differentiation, memory B cells, and regulatory T cells. J Immunol. 2008;181:5158-66 pubmed
    Primary C3 deficiency, a rare autosomal inherited disease (OMIM 120700), was identified in a 2-year-old male suffering from recurrent pyogenic infections from early infancy with undetectable total complement hemolytic activity (CH50) and ..
  81. Yanagisawa S, Kondo N, Miki A, Matsumiya W, Kusuhara S, Tsukahara Y, et al. A common complement C3 variant is associated with protection against wet age-related macular degeneration in a Japanese population. PLoS ONE. 2011;6:e28847 pubmed publisher
    Genetic variants in the complement component 3 gene (C3) have been shown to be associated with age-related macular degeneration (AMD) in Caucasian populations of European descent...
  82. Nozaki M, Raisler B, Sakurai E, Sarma J, Barnum S, Lambris J, et al. Drusen complement components C3a and C5a promote choroidal neovascularization. Proc Natl Acad Sci U S A. 2006;103:2328-33 pubmed
    ..Here we provide evidence that bioactive fragments of these complement components (C3a and C5a) are present in drusen of patients with AMD, and that C3a and C5a induce VEGF expression in vitro and in ..
  83. Sahu A, Isaacs S, Soulika A, Lambris J. Interaction of vaccinia virus complement control protein with human complement proteins: factor I-mediated degradation of C3b to iC3b1 inactivates the alternative complement pathway. J Immunol. 1998;160:5596-604 pubmed
    ..Recombinant VCP bound to C3 and the proteolytically cleaved form of C3 (C3b), but not to the 135,300-m.w. fragment of C3 generated using elastase (C3c) and the 35,000-m.w...
  84. Bergeron Sawitzke J, Gold B, Olsh A, Schlotterbeck S, Lemon K, Visvanathan K, et al. Multilocus analysis of age-related macular degeneration. Eur J Hum Genet. 2009;17:1190-9 pubmed publisher
    ..Subjects were genotyped for single nucleotide polymorphisms (SNPs) from CFH, complement component 2 (C2), complement component 3 (C3), complement factor B (CFB), age-related maculopathy susceptibility (ARMS2), HtrA serine peptidase 1 (..
  85. Neale B, Fagerness J, Reynolds R, Sobrin L, Parker M, Raychaudhuri S, et al. Genome-wide association study of advanced age-related macular degeneration identifies a role of the hepatic lipase gene (LIPC). Proc Natl Acad Sci U S A. 2010;107:7395-400 pubmed publisher
    ..Results implicate different biologic pathways than previously reported and provide new avenues for prevention and treatment of AMD. ..
  86. Botto M, Fong K, So A, Koch C, Walport M. Molecular basis of polymorphisms of human complement component C3. J Exp Med. 1990;172:1011-7 pubmed
    b>C3 exhibits two common allotypic variants that may be separated by gel electrophoresis and are called C3 fast (C3 F) and C3 slow (C3 S)...
  87. Haas M, Rahman M, Racusen L, Kraus E, Bagnasco S, Segev D, et al. C4d and C3d staining in biopsies of ABO- and HLA-incompatible renal allografts: correlation with histologic findings. Am J Transplant. 2006;6:1829-40 pubmed
  88. Zhou J, Jang Y, Kim S, Sparrow J. Complement activation by photooxidation products of A2E, a lipofuscin constituent of the retinal pigment epithelium. Proc Natl Acad Sci U S A. 2006;103:16182-7 pubmed
    ..Accordingly, we found that the C3 split products inactivated C3b (iC3b) and C3a were elevated in serum, overlying ARPE-19 cells that had accumulated A2E and were irradiated to induce A2E ..
  89. Hecker L, Edwards A, Ryu E, Tosakulwong N, Baratz K, Brown W, et al. Genetic control of the alternative pathway of complement in humans and age-related macular degeneration. Hum Mol Genet. 2010;19:209-15 pubmed publisher
    ..Genetic variation across the genes encoding complement factor H (CFH), factor B (CFB) and component 3 (C3) was determined...
  90. Heurich M, Martinez Barricarte R, Francis N, Roberts D, Rodriguez de Cordoba S, Morgan B, et al. Common polymorphisms in C3, factor B, and factor H collaborate to determine systemic complement activity and disease risk. Proc Natl Acad Sci U S A. 2011;108:8761-6 pubmed publisher
    ..fB binding kinetics and convertase stability were identical, but native and recombinant fH bound more strongly to C3b(102R) (K(D) C3b(102R): 1.0 ?M; C3b(102G): 1.4 ?M; P < 0.0001)...
  91. Nagar B, Jones R, Diefenbach R, Isenman D, Rini J. X-ray crystal structure of C3d: a C3 fragment and ligand for complement receptor 2. Science. 1998;280:1277-81 pubmed
    Activation and covalent attachment of complement component C3 to pathogens is the key step in complement-mediated host defense...
  92. Happonen K, Saxne T, Geborek P, Andersson M, Bengtsson A, Hesselstrand R, et al. Serum COMP-C3b complexes in rheumatic diseases and relation to anti-TNF-? treatment. Arthritis Res Ther. 2012;14:R15 pubmed publisher
    ..We recently showed that COMP activates complement via the alternative pathway and that COMP-C3b complexes are present in sera of RA patients, but not in healthy controls...