complement c7

Summary

Summary: 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.

Top Publications

  1. Vázquez Bermúdez M, Barroso S, Walter K, Alvarez A, Alarcon A, Lopez Trascasa M, et al. Complement component C7 deficiency in a Spanish family. Clin Exp Immunol. 2003;133:240-6 pubmed
    ..The missense mutation G357R would make an interesting topic of analysis with regard to meningococcal disease susceptibility in the Spanish population. ..
  2. Schlesinger M, Nave Z, Levy Y, Slater P, Fishelson Z. Prevalence of hereditary properdin, C7 and C8 deficiencies in patients with meningococcal infections. Clin Exp Immunol. 1990;81:423-7 pubmed
    ..This and other findings indicate that the type of complement abnormality found in association with meningococcal infections varies with the ethnic origin of the patient. ..
  3. DiScipio R, Chakravarti D, Muller Eberhard H, Fey G. The structure of human complement component C7 and the C5b-7 complex. J Biol Chem. 1988;263:549-60 pubmed
    ..Thus, C7 plays a major role in bringing about the hydrophilic-amphiphilic transition during the formation of the membrane attack complex, and it serves as a membrane anchor for the C5b-7 complex. ..
  4. Hobart M, Fernie B, DiScipio R. Structure of the human C7 gene and comparison with the C6, C8A, C8B, and C9 genes. J Immunol. 1995;154:5188-94 pubmed
    ..With the exception of exon 1, all intron/exon boundaries are preserved with respect to phase when compared with C6. ..
  5. Nishizaka H, Horiuchi T, Zhu Z, Fukumori Y, Volanakis J. Genetic bases of human complement C7 deficiency. J Immunol. 1996;157:4239-43 pubmed
    b>Complement C7 deficiency (C7D) is associated frequently with recurrent bacterial infections, especially meningitis caused by Neisseria meningitidis. We report in this work the molecular bases of C7D in two unrelated Japanese males...
  6. Tschopp J, Chonn A, Hertig S, French L. Clusterin, the human apolipoprotein and complement inhibitor, binds to complement C7, C8 beta, and the b domain of C9. J Immunol. 1993;151:2159-65 pubmed
    ..These results show that clusterin exerts its inhibitory effect by interacting with a structural motif common to C7, C8 alpha, and C9b. ..
  7. Rameix Welti M, Regnier C, Bienaime F, Blouin J, Schifferli J, Fridman W, et al. Hereditary complement C7 deficiency in nine families: subtotal C7 deficiency revisited. Eur J Immunol. 2007;37:1377-85 pubmed
    ..Our results provide evidence that R499S represents a loss-of-function polymorphism of C7 due to a defective folding of the protein. ..
  8. Kuijpers T, Nguyen M, Hopman C, Nieuwenhuys E, Dewald G, Lankester A, et al. Complement factor 7 gene mutations in relation to meningococcal infection and clinical recurrence of meningococcal disease. Mol Immunol. 2010;47:671-7 pubmed publisher
    ..The results endorse the lack of clinical recurrences once antibodies are present, whereas in C7Q0 patients the anti-meningococcal antibodies may not suffice to protect from recurrent meningococcal infection. ..
  9. Behar D, Schlesinger M, Halle D, Ben Ami H, Edoute Y, Shahar E, et al. C7 complement deficiency in an Israeli Arab village. Am J Med Genet. 2002;110:25-9 pubmed
    ..The implications of this finding are discussed in the context of family pedigree, the protective effect of complement deficiency, and the clinical outcome. ..

More Information

Publications62

  1. Wurzner R, Witzel Schlömp K, Tokunaga K, Fernie B, Hobart M, Orren A. Reference typing report for complement components C6, C7 and C9 including mutations leading to deficiencies. Exp Clin Immunogenet. 1998;15:268-85 pubmed
    ..The results of the protein typing for C6 are published in the following paper of this issue. ..
  2. Fernie B, Orren A, Sheehan G, Schlesinger M, Hobart M. Molecular bases of C7 deficiency: three different defects. J Immunol. 1997;159:1019-26 pubmed
    ..However, one individual is heterozygous for markers at adjacent C6 loci, showing that there has been an intergenic recombination and suggesting that the deficiency mutation is of appreciable antiquity. ..
  3. Fernie B, Wurzner R, Orren A, Morgan B, Potter P, Platonov A, et al. Molecular bases of combined subtotal deficiencies of C6 and C7: their effects in combination with other C6 and C7 deficiencies. J Immunol. 1996;157:3648-57 pubmed
    ..Each molecular defect has also been found in isolation and has the expected effect. ..
  4. 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
    ..However, surface-bound IgG does initiate the classical pathway of complement activation, which is active in the lung, resulting in deposition of the opsonin C3b on the spore surface. ..
  5. Corvini M, Randolph C, Aronin S. Complement C7 deficiency presenting as recurrent aseptic meningitis. Ann Allergy Asthma Immunol. 2004;93:200-5 pubmed
    ..The ability to detect the bacterial cause of meningitis in such patients is organism dependent and may be influenced by factors such as cerebrospinal fluid bacterial concentration and previous antibiotic drug exposure. ..
  6. Reinartz J, Hansch G, Kramer M. Complement component C7 is a plasminogen-binding protein. J Immunol. 1995;154:844-50 pubmed
    ..Based on these findings, an interaction is proposed between the complement system and the plasminogen activator system; a mechanism that may focus plasmin activity to structures that have been tagged by Ab and complement deposition. ..
  7. Binks M, Sriprakash K. Characterization of a complement-binding protein, DRS, from strains of Streptococcus pyogenes containing the emm12 and emm55 genes. Infect Immun. 2004;72:3981-6 pubmed
    ..Despite this, the recombinant DRS preparation showed no significant effect on complement function, as determined by lysis of sensitized sheep erythrocytes. Furthermore, the presence of DRS is not inhibitory to SIC activity. ..
  8. Nishimukai H, Nishimura K, Orimoto C, Okiura T, Fujii K, Fukumori Y, et al. Single nucleotide polymorphisms in the human complement C6 and C7 genes. Leg Med (Tokyo). 2003;5 Suppl 1:S198-200 pubmed
    ..920 and *A=0.080. SNPs in the C7 gene are as follows: T382C in exon 4, G1166C and A1258C in exon 9, and G[+10]A in intron 13. Nt382C and nt1258C would be responsible for C7-5 (=C7-3) and C7-4 allotypes, respectively. ..
  9. Wickramaarachchi W, Whang I, Kim E, Lim B, Jeong H, De Zoysa M, et al. Genomic characterization and transcriptional evidence for the involvement of complement component 7 in immune response of rock bream (Oplegnathus fasciatus). Dev Comp Immunol. 2013;41:44-9 pubmed publisher
    ..tarda infection. These results suggested that Rb-C7 is lytic pathway gene in complement system and its transcriptional regulation may be an important immune response in pathogenic defense mechanism of rock bream. ..
  10. Fernie B, Hobart M. Five new polymorphisms in the complement C7 gene and their association with C7 deficiency. Exp Clin Immunogenet. 1999;16:150-61 pubmed
  11. Gabrielsson B, Johansson J, Lönn M, Jernas M, Olbers T, Peltonen M, et al. High expression of complement components in omental adipose tissue in obese men. Obes Res. 2003;11:699-708 pubmed
  12. Langeggen H, Pausa M, Johnson E, Casarsa C, Tedesco F. The endothelium is an extrahepatic site of synthesis of the seventh component of the complement system. Clin Exp Immunol. 2000;121:69-76 pubmed
    ..By contrast, interferon-gamma had only a marginal effect on the production of C3, but markedly down-regulated the synthesis of C7 as assessed both by ELISA and RT-PCR. ..
  13. Sassi F, Bardi R, Neji T, Ayed K, Ben Dridi M. [Immunological study in sickle cell disease patients: importance of the complement system]. Tunis Med. 2003;81:195-9 pubmed
    ..Numeration of Band T cells revealed slight decrease in proportion of CD3 and CD4 at one patient associated with an increase in B cells, but normal or increased absolute numbers of all cells population. ..
  14. Goundis D, Reid K. Properdin, the terminal complement components, thrombospondin and the circumsporozoite protein of malaria parasites contain similar sequence motifs. Nature. 1988;335:82-5 pubmed
    ..These similarities may provide insight into the mechanisms by which parasites avoid host defences mediated by complement. ..
  15. Thai C, Ogata R. Recombinant C345C and factor I modules of complement components C5 and C7 inhibit C7 incorporation into the complement membrane attack complex. J Immunol. 2005;174:6227-32 pubmed
    ..These results demonstrate that the interaction between the C345C domain of C5 and the FIMs of C7, which mediates reversible binding of C5 to C7 in solution, also plays an essential role in MAC formation and complement lytic activity. ..
  16. 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
  17. Barroso S, Sanchez B, Alvarez A, Lopez Trascasa M, Lanuza A, Luque R, et al. Complement component C7 deficiency in two Spanish families. Immunology. 2004;113:518-23 pubmed
    ..On the other hand, certain C7 defects appear to be prevalent in individuals from certain populations or living in defined geographical areas. ..
  18. Blanchin S, Estienne V, Durand Gorde J, Carayon P, Ruf J. Complement activation by direct C4 binding to thyroperoxidase in Hashimoto's thyroiditis. Endocrinology. 2003;144:5422-9 pubmed
    ..Investigating this complement pathway, therefore, would provide an excellent means of reaching a better understanding of the etiology of other degenerative diseases. ..
  19. Nemeth T, Virtic O, Sitaru C, Mocsai A. The Syk Tyrosine Kinase Is Required for Skin Inflammation in an In Vivo Mouse Model of Epidermolysis Bullosa Acquisita. J Invest Dermatol. 2017;137:2131-2139 pubmed publisher
  20. Pausa M, Pellis V, Cinco M, Giulianini P, Presani G, Perticarari S, et al. Serum-resistant strains of Borrelia burgdorferi evade complement-mediated killing by expressing a CD59-like complement inhibitory molecule. J Immunol. 2003;170:3214-22 pubmed
    ..Western blot analysis revealed preferential binding of the C inhibitory molecule to C9 and weak interaction with C8 beta. ..
  21. Bhandari M, Kawamoto M, Thomas A, Barreto S, Schloithe A, Carati C, et al. Galanin receptor antagonist m35 but not m40 or c7 ameliorates cerulein-induced acute pancreatitis in mice. Pancreatology. 2010;10:682-8 pubmed publisher
    ..Complex galanin pathways may be involved in cerulein-induced AP. M35 and galantide are potential therapeutic peptides for the treatment of AP and further evaluation should be considered. and IAP. ..
  22. Høgåsen A, Wurzner R, Abrahamsen T, Dierich M. Human polymorphonuclear leukocytes store large amounts of terminal complement components C7 and C6, which may be released on stimulation. J Immunol. 1995;154:4734-40 pubmed
    ..This suggests a new role for the PMN as a C membrane attack modulator. ..
  23. Shen Y, Zhang J, Xu X, Fu J, Li J. Expression of complement component C7 and involvement in innate immune responses to bacteria in grass carp. Fish Shellfish Immunol. 2012;33:448-54 pubmed publisher
    ..Significant changes in C7 transcript expression (>20-fold) were detected following Aeromonas hydrophila infection, indicating C7 involvement in innate immune responses to bacteria in teleost fish. ..
  24. Keiser P, Broderick M. Meningococcal polysaccharide vaccine failure in a patient with C7 deficiency and a decreased anti-capsular antibody response. Hum Vaccin Immunother. 2012;8:582-6 pubmed publisher
  25. Debard A, Lamy B, Monneret G, Mira J, Pachot A, Kleijer M, et al. FcgammaRIIIb and complement component C7 codeficiency in a patient with recurrence of fulminant meningococcal septic shock. Clin Infect Dis. 2005;40:1679-83 pubmed
    ..We found a total deficiency of FcgammaRIIIb on neutrophils, which could partially explain the unusually severe clinical presentation. ..
  26. Papanastasiou A, Zarkadis I. Gene duplication of the seventh component of complement in rainbow trout. Immunogenetics. 2005;57:703-8 pubmed publisher
    ..Although duplication of complement genes is a common observation in teleost fish, this is the first report of two gene isotypes of a terminal membrane attack complex/perforin complement component in any organism...
  27. Phelan M, Thai C, Herbert A, Bella J, Uhrin D, Ogata R, et al. 1H, 15N and 13C resonance assignment of the pair of Factor-I like modules of the complement protein C7. Biomol NMR Assign. 2009;3:49-52 pubmed publisher
    ..The chemical shifts have been deposited in the BioMagResBank; Accession No. 15996. ..
  28. Liu G, Zhang J, Chen X. Molecular and functional characterization of a CD59 analogue from large yellow croaker Pseudosciana crocea. Mol Immunol. 2007;44:3661-71 pubmed publisher
    ..These results represent the first functional identification of a CD59 analogue in teleost fish, strongly suggesting the presence of regulatory mechanism for terminal complement pathway in teleost fish...
  29. Horiuchi T, Nishizaka H, Tsukamoto H, Harashima S, Sawabe T, Morita C, et al. An NcoI polymorphism in the human complement component 7 (C7) gene. J Hum Genet. 1999;44:270-1 pubmed
    ..660 and 0.340. This NcoI polymorphism would be useful to perform a DNA marker haplotype study in patients with deficiencies of the complement genes, such as C6, C7, C9, which are located closely on chromosome 5p13. ..
  30. Triebel T, Grillhösl B, Kacani L, Lell C, Fuchs A, Speth C, et al. Importance of the terminal complement components for immune defence against Candida. Int J Med Microbiol. 2003;292:527-36 pubmed
  31. Naughton M, Walport M, Wurzner R, Carter M, Alexander G, Goldman J, et al. Organ-specific contribution to circulating C7 levels by the bone marrow and liver in humans. Eur J Immunol. 1996;26:2108-12 pubmed
    ..These findings provide further support for the concept that locally secreted complement proteins have an important role in inflammation. ..
  32. Muller Eberhard H. Complement. Annu Rev Biochem. 1975;44:697-724 pubmed
  33. Lian L, Ching A, Chong Z, Chua K. Complement components 2 and 7 (C2 and C7) gene polymorphisms are not major risk factors for SLE susceptibility in the Malaysian population. Rheumatol Int. 2012;32:3665-8 pubmed publisher
    ..These overall results suggest a lack of strong association with the C2 and C7 gene polymorphisms to the susceptibility of SLE in the Malaysian population. ..
  34. DiScipio R, Schraufstatter I. Magnetic bead based assays for complement component C5. J Immunol Methods. 2017;450:50-57 pubmed publisher
    ..In contrast only β-glucans from two species of macrofungi were able to interfere with interaction of C5 with the FIMs of C7. ..
  35. Mishra M, Byrd M, Sergeant S, Azad A, Parsek M, MCPHAIL L, et al. Pseudomonas aeruginosa Psl polysaccharide reduces neutrophil phagocytosis and the oxidative response by limiting complement-mediated opsonization. Cell Microbiol. 2012;14:95-106 pubmed publisher
    ..This provides a survival advantage in vivo. Since phagocytes are critical in early recognition and control of infection, therapies aimed at Psl could improve the quality of life for patients colonized with P. aeruginosa...
  36. Raitses Gurevich M, Fishelson Z. Construction and characterization of recombinant human C9 or C7 linked to single chain Fv directed to CD25. Mol Immunol. 2013;55:400-8 pubmed publisher
    ..an anti-Tac (CD25) monoclonal antibody fused at its C terminus either to complement protein C9 (scFv-C9) or to complement C7 (scFv-C7) and tagged with six histidines at the C terminal end...
  37. Chiang Y, Shyur S, Huang L, Wen T, Lin M, Yang H, et al. Deficiency of the seventh component of complement in a Taiwanese boy. J Formos Med Assoc. 2006;105:770-4 pubmed
    ..Awareness of the possibility of late complement deficiency is important as they comprise a small percentage of patients who present with disseminated meningococcal disease or other serious infections caused by encapsulated organisms. ..
  38. Corbo R, Tartaglia M, Scacchi R, Rickards O, Dash S, Sharma S, et al. A survey of six genetic markers on the populations of Punjab and Rajasthan (India). Gene Geogr. 1991;5:113-21 pubmed
    ..366 +/- 0.037; PGM1*2 = 0.247 +/- 0.017; PGD*C = 0.041 +/- 0.008. These data may contribute to evaluate the extent of the Mongoloid genetic admixture into the Caucasoid gene pool of the Punjab and Rajasthan Hindu population. ..
  39. Bramham J, Thai C, Soares D, Uhrin D, Ogata R, Barlow P. Functional insights from the structure of the multifunctional C345C domain of C5 of complement. J Biol Chem. 2005;280:10636-45 pubmed
    ..Modeling of homologous domains from complement proteins C3 and C4, which do not participate in membrane attack complex assembly, suggests that this provisionally identified C6/C7-interacting face is indeed specific to C5. ..
  40. Dewald G, Nöthen M, Rüther K. A common Ser/Thr polymorphism in the perforin-homologous region of human complement component C7. Hum Hered. 1994;44:301-4 pubmed
    ..The two alleles are both very common, probably in all major races. ..
  41. Agah A, Montalto M, Kiesecker C, Morrissey M, Grover M, Whoolery K, et al. Isolation, characterization, and cloning of porcine complement component C7. J Immunol. 2000;165:1059-65 pubmed
    ..These data demonstrate that porcine and human C7 are highly conserved, sharing structural and functional characteristics. ..
  42. Witzel Schlömp K, Spath P, Hobart M, Fernie B, Rittner C, Kaufmann T, et al. The human complement C9 gene: identification of two mutations causing deficiency and revision of the gene structure. J Immunol. 1997;158:5043-9 pubmed
    ..No additional introns were detected in exons 6 and 10. Furthermore, DNA marker studies were conducted using known polymorphisms of the C6, C7, and C9 genes, confirming the linkage of the observed C9 mutations with defined haplotypes. ..
  43. van Oorschot R, Birmingham V, Porter P, Kammerer C, Vandeberg J. Linkage between complement components 6 and 7 and glutamic pyruvate transaminase in the marsupial Monodelphis domestica. Biochem Genet. 1993;31:215-22 pubmed
    ..The newly described C6 and C7 polymorphisms provide additional power for developing a linkage map for M. domestica and for localizing genes that confer susceptibility to diseases for which this species is used as a model. ..
  44. Wurzner R, Rance N, Potter P, Hendricks M, Lachmann P, Orren A. C7 M/N protein polymorphism typing applied to inherited deficiencies of human complement proteins C6 and C7. Clin Exp Immunol. 1992;89:485-9 pubmed
    ..C6/C7 haplotype analysis of South African C6-deficient (C6Q0) subjects revealed a strong allelic association of C6*Q0 and C7*M. ..
  45. Shen Y, Zhang J, Fu J, Xu X, Li J, Wang R, et al. A 425 T>C polymorphism in complement C7 association with resistance to Aeromonas hydrophila in grass carp. Genet Mol Res. 2016;15: pubmed publisher
    ..hydrophila in grass carp. These findings suggest that the 425 C>T polymorphism in C7 gene may be a significant molecular marker for resistance to A. hydrophila in grass carp. ..
  46. Scheetz T, Fingert J, Wang K, Kuehn M, Knudtson K, Alward W, et al. A genome-wide association study for primary open angle glaucoma and macular degeneration reveals novel Loci. PLoS ONE. 2013;8:e58657 pubmed publisher
    ..However, several yielded putative associations requiring validation. Our data suggest that POAG is more genetically complex than AMD, with no common risk alleles of large effect. ..
  47. Welsh K, Lewis C, Boyd S, Braun M, Actor J. Complement factor C7 contributes to lung immunopathology caused by Mycobacterium tuberculosis. Clin Dev Immunol. 2012;2012:429675 pubmed publisher
    ..Mice deficient in complement C7 and wild-type C57BL/6 mice were aerosol challenged with MTB Erdman and assessed for bacterial burden, ..
  48. Løvoll M, Kilvik T, Boshra H, Bøgwald J, Sunyer J, Dalmo R. Maternal transfer of complement components C3-1, C3-3, C3-4, C4, C5, C7, Bf, and Df to offspring in rainbow trout (Oncorhynchus mykiss). Immunogenetics. 2006;58:168-79 pubmed
    ..This study is the first to address the ontogeny of several complement components and represents the first evidence that maternal transfer of complement components, other than C3, occurs in teleost fish. ..
  49. Schirinzi R, Lantin J, Fremeaux Bacchi V, Schifferli J, Trendelenburg M. [Combined-heterozygous deficiency of complement C7 in a patient with recurrent meningitis]. Med Klin (Munich). 2006;101:655-8 pubmed
    ..e., C5-C9. In the presented study the first case of a patient with combined-heterozygous deficiency of complement C7 is described. The defect led to a strongly reduced but still measurable production of C7...
  50. Kallio S, Jakkula E, Purcell S, Suvela M, Koivisto K, Tienari P, et al. Use of a genetic isolate to identify rare disease variants: C7 on 5p associated with MS. Hum Mol Genet. 2009;18:1670-83 pubmed publisher
    ..These results suggest that the MS predisposing locus on 5p is more complex than assumed and exemplify power of population isolates in the identification of rare disease alleles. ..
  51. Fernie B, Hobart M. Complement C7 deficiency: seven further molecular defects and their associated marker haplotypes. Hum Genet. 1998;103:513-9 pubmed
    ..They are distributed along the C7 gene, but predominantly towards the 3' end. All were found in compound heterozygous individuals. The C6/C7 marker haplotypes associated with most C7 defects are tabulated. ..
  52. Oka R, Sasagawa T, Ninomiya I, Miwa K, Tanii H, Saijoh K. Reduction in the local expression of complement component 6 (C6) and 7 (C7) mRNAs in oesophageal carcinoma. Eur J Cancer. 2001;37:1158-65 pubmed
    ..Our findings suggest a possible relationship between oesophageal tumorigenesis and reduced expression of C6 and C7 mRNAs, which is probably caused by a change in gene expression regulation and not by genetic loss of the locus. ..
  53. Orren A, Hobart M, Nash H, Lachmann P. Close linkage between mouse genes determining the two forms of complement component C6 and component C7, and cis action of a C6 regulatory gene. Immunogenetics. 1985;21:591-9 pubmed
    ..Sera from a number of inbred strains were also screened for a complement C7 polymorphism by means of isoelectric focusing and functional overlay...