C6

Summary

Gene Symbol: C6
Description: complement C6
Alias: complement component C6, complement component 6
Species: human
Products:     C6

Top Publications

  1. Parham K, Roberts A, Thomas A, Wurzner R, Henderson H, Potter P, et al. Prevalence of mutations leading to complete C6 deficiency (C6Q0) in the Western Cape, South Africa and detection of novel mutations leading to C6Q0 in an Irish family. Mol Immunol. 2007;44:2756-60 pubmed
    b>Complement component C6 is one of five terminal complement components incorporated into the membrane attack complex...
  2. Eid N, Hussein A, Elzein A, Mohamed H, Rockett K, Kwiatkowski D, et al. Candidate malaria susceptibility/protective SNPs in hospital and population-based studies: the effect of sub-structuring. Malar J. 2010;9:119 pubmed publisher
  3. Aleshin A, DiScipio R, Stec B, Liddington R. Crystal structure of C5b-6 suggests structural basis for priming assembly of the membrane attack complex. J Biol Chem. 2012;287:19642-52 pubmed publisher
    ..The first step in MAC assembly is cleavage of C5 to generate metastable C5b, which forms a stable complex with C6, termed C5b-6...
  4. Lengweiler S, Schaller J, DiScipio R, Rickli E. Elucidation of the disulfide-bonding pattern in the factor I modules of the sixth component (C6) of human complement. Biochim Biophys Acta. 1997;1342:13-8 pubmed
    b>Complement component C6 is known to contain two factor I modules in tandem at its C-terminus. To localize the disulfide bridges in those domains, native C6 was cleaved with trypsin, followed by subtilisin...
  5. Chakravarti D, Muller Eberhard H. Biochemical characterization of the human complement protein C6. Association with alpha-thrombin-like enzyme and absence of serine protease activity in cytolytically active C6. J Biol Chem. 1988;263:18306-12 pubmed
    Complement protein C6 has been proposed by others to be a serine protease whose activity is obligatory for complement-directed cell lysis...
  6. Hadders M, Bubeck D, Roversi P, Hakobyan S, Forneris F, Morgan B, et al. Assembly and regulation of the membrane attack complex based on structures of C5b6 and sC5b9. Cell Rep. 2012;1:200-7 pubmed publisher
    ..b>C6 captures this conformation, which is preserved in the larger sC5b9 assembly...
  7. Dewald G, Nöthen M, Cichon S. Polymorphism of human complement component C6: an amino acid substitution (Glu/Ala) within the second thrombospondin repeat differentiates between the two common allotypes C6 A and C6 B. Biochem Biophys Res Commun. 1993;194:458-64 pubmed
    Component C6 of the human complement system exhibits a genetic polymorphism in all populations tested so far...
  8. DiScipio R. Formation and structure of the C5b-7 complex of the lytic pathway of complement. J Biol Chem. 1992;267:17087-94 pubmed
    ..cytolytic intermediary complex, C5b-7, were studied with the aim of determining the interactive regions of C5, C6, and C7...
  9. Fosbrink M, Cudrici C, Tegla C, Soloviova K, Ito T, Vlaicu S, et al. Response gene to complement 32 is required for C5b-9 induced cell cycle activation in endothelial cells. Exp Mol Pathol. 2009;86:87-94 pubmed publisher
    ..All these data together suggest that cell cycle induction by C5b-9 in AEC is RGC-32 dependent and this is in part through regulation of Akt and growth factor release. ..

More Information

Publications32

  1. Aleshin A, Schraufstatter I, Stec B, Bankston L, Liddington R, DiScipio R. Structure of complement C6 suggests a mechanism for initiation and unidirectional, sequential assembly of membrane attack complex (MAC). J Biol Chem. 2012;287:10210-22 pubmed publisher
    ..complex (MAC) is formed by the sequential assembly of C5b with four homologous proteins as follows: one copy each of C6, C7, and C8 and 12-14 copies of C9. Together these form a lytic pore in bacterial membranes...
  2. Gancz D, Donin N, Fishelson Z. Involvement of the c-jun N-terminal kinases JNK1 and JNK2 in complement-mediated cell death. Mol Immunol. 2009;47:310-7 pubmed publisher
    ..Our results demonstrate that complement C5b-9 induce a JNK/Bid-dependent and JNK-independent necrotic cell death. Both JNK1 and JNK2 have cytotoxic potential, however JNK2 is the primary signal transducer. ..
  3. Hobart M, Fernie B, DiScipio R, Lachmann P. A physical map of the C6 and C7 complement component gene region on chromosome 5p13. Hum Mol Genet. 1993;2:1035-6 pubmed
  4. Gonzalez S, Lopez Larrea C. Characterization of the human C6 promoter: requirement of the CCAAT enhancer binding protein binding site for C6 gene promoter activity. J Immunol. 1996;157:2282-90 pubmed
    The sixth complement component (C6) is a late-acting complement protein that participates in the assembly of the membrane attack complex. C6 and most of the complement proteins are mainly synthesized in the liver...
  5. Wu W, Yang H, Feng Y, Zhang P, Li S, Wang X, et al. Polymorphisms in complement genes and risk of preeclampsia in Taiyuan, China. Inflamm Res. 2016;65:837-45 pubmed publisher
    ..We examined 51 SNPs in the C3, C5, C6, MASP1, MBL2 and CD55 genes and the risk of preeclampsia and its clinical subtypes in a nested case-control study of ..
  6. DiScipio R, Hugli T. The molecular architecture of human complement component C6. J Biol Chem. 1989;264:16197-206 pubmed
    The molecular architecture of human complement component C6 was elucidated at several levels of structural organization...
  7. Muller Eberhard H. Molecular organization and function of the complement system. Annu Rev Biochem. 1988;57:321-47 pubmed
  8. Chakravarti D, Chakravarti B, Parra C, Muller Eberhard H. Structural homology of complement protein C6 with other channel-forming proteins of complement. Proc Natl Acad Sci U S A. 1989;86:2799-803 pubmed
    The amino acid sequence of the amino-terminal half of the complement protein C6 has been found to show overall structural homology with the homologous regions of the channel-forming proteins C7, C8 alpha, C8 beta, and C9...
  9. Aslibekyan S, An P, Frazier Wood A, Kabagambe E, Irvin M, Straka R, et al. Preliminary evidence of genetic determinants of adiponectin response to fenofibrate in the Genetics of Lipid Lowering Drugs and Diet Network. Nutr Metab Cardiovasc Dis. 2013;23:987-94 pubmed publisher
    ..Our findings provide support for fine-mapping of the 12q24 region to investigate the shared biological mechanisms underlying levels of circulating adiponectin and susceptibility to metabolic disease. ..
  10. Bulla R, Bossi F, Agostinis C, Radillo O, Colombo F, De Seta F, et al. Complement production by trophoblast cells at the feto-maternal interface. J Reprod Immunol. 2009;82:119-25 pubmed publisher
    ..Both cell types secrete C4 and C3, and HTR8/SVneo trophoblast cells secrete C6 in a measurable amount...
  11. Orren A, Owen E, Henderson H, van der Merwe L, Leisegang F, Stassen C, et al. Complete deficiency of the sixth complement component (C6Q0), susceptibility to Neisseria meningitidis infections and analysis of the frequencies of C6Q0 gene defects in South Africans. Clin Exp Immunol. 2012;167:459-71 pubmed publisher
    Complete complement component 6 deficiency (C6Q0) is a co-dominant genetic disease presenting as increased susceptibility to invasive Neisseria meningitidis infections...
  12. Coto E, Martinez Naves E, Dominguez O, DiScipio R, Urra J, Lopez Larrea C. DNA polymorphisms and linkage relationship of the human complement component C6, C7, and C9 genes. Immunogenetics. 1991;33:184-7 pubmed
    In this report we describe the linkage between genes encoding human complement components C6, C7, and C9. Polymorphisms have been described at the DNA level for the C7 and C9 genes...
  13. Soejima M, Tachida H, Tsuneoka M, Takenaka O, Kimura H, Koda Y. Nucleotide sequence analyses of human complement 6 (C6) gene suggest balancing selection. Ann Hum Genet. 2005;69:239-52 pubmed publisher
    The sixth complement component (C6) has a common charge polymorphism, C6A and C6B, with similar gene frequencies in all major populations. In addition, C6B2 is also found in Japanese populations at a frequency of about 6%...
  14. Hobart M, Fernie B, Fijen K, Orren A. The molecular basis of C6 deficiency in the western Cape, South Africa. Hum Genet. 1998;103:506-12 pubmed
    Deficiency of the sixth component of human complement (C6) has been reported in a number of families from the western Cape, South Africa...
  15. Pasaje C, Bae J, Park B, Cheong H, Jang A, Uh S, et al. Association analysis of C6 genetic variations and aspirin hypersensitivity in Korean asthmatic patients. Hum Immunol. 2011;72:973-8 pubmed publisher
    ..The complement component (C6) is a constituent of a biochemical cascade that has been implicated in airway epithelial damage and nasal polyposis, ..
  16. Hofsteenge J, Blommers M, Hess D, Furmanek A, Miroshnichenko O. The four terminal components of the complement system are C-mannosylated on multiple tryptophan residues. J Biol Chem. 1999;274:32786-94 pubmed
    ..C-mannosylation, and to learn more about its mechanism, the terminal components of the human complement system (C6, C7, C8,and C9), which contain multiple and complex recognition motifs, were examined...
  17. Shi H, Williams J, Guo L, Stampoulis D, Francesca Cordeiro M, Moss S. Exposure to the complement C5b-9 complex sensitizes 661W photoreceptor cells to both apoptosis and necroptosis. Apoptosis. 2015;20:433-43 pubmed publisher
    ..Our observations provide new insight into the potential role of the complement system in photoreceptor loss, with implications for the molecular aetiology of retinal disease. ..
  18. Haefliger J, Tschopp J, Vial N, Jenne D. Complete primary structure and functional characterization of the sixth component of the human complement system. Identification of the C5b-binding domain in complement C6. J Biol Chem. 1989;264:18041-51 pubmed
    Complement C6 is one of five plasma proteins that are incorporated into the lytic terminal complement complex on lipid membranes (C5b-9m) upon activation of the complement cascade...
  19. Molleran Lee S, Villanueva J, Sumegi J, Zhang K, Kogawa K, Davis J, et al. Characterisation of diverse PRF1 mutations leading to decreased natural killer cell activity in North American families with haemophagocytic lymphohistiocytosis. J Med Genet. 2004;41:137-44 pubmed
  20. Zhu Z, Totemchokchyakarn K, Atkinson T, Volanakis J. Molecular defects leading to human complement component C6 deficiency in an African-American family. Clin Exp Immunol. 1998;111:91-6 pubmed
    b>Complement component C6 deficiency (C6D) was diagnosed in a 16-year-old African-American male with meningococcal meningitis...
  21. Hobart M, Fernie B, DiScipio R. Structure of the human C6 gene. Biochemistry. 1993;32:6198-205 pubmed
    The terminal components of the complement system (C6-C9) are related proteins, differing in size and complexity. They seem to be typical mosaic proteins, composed of modules which are homologous with parts of other proteins...
  22. Hetland G, Johnson E, Falk R, Eskeland T. Synthesis of complement components C5, C6, C7, C8 and C9 in vitro by human monocytes and assembly of the terminal complement complex. Scand J Immunol. 1986;24:421-8 pubmed
    ..There was a significantly binding of monoclonal anti-C3c antibodies, polyclonal anti-C5, anti-C6, anti-C7, anti-C8, and anti-C9 antibodies, and of a monoclonal antibody against a neoantigen of polymerized C9 to ..
  23. Wang Z, Liao J, Wu S, Li C, Fan J, Peng Z. Recipient C6 rs9200 genotype is associated with hepatocellular carcinoma recurrence after orthotopic liver transplantation in a Han Chinese population. Cancer Gene Ther. 2016;23:157-61 pubmed publisher
    ..The sixth complement component (C6) is a late-acting complement protein that participates in the assembly of the membrane attack complex, which has an ..