C8B

Summary

Gene Symbol: C8B
Description: complement C8 beta chain
Alias: C82, complement component C8 beta chain, complement component 8 subunit beta, complement component 8, beta polypeptide
Species: human
Products:     C8B

Top Publications

  1. Brannen C, Sodetz J. Incorporation of human complement C8 into the membrane attack complex is mediated by a binding site located within the C8beta MACPF domain. Mol Immunol. 2007;44:960-5 pubmed
    ..Furthermore, results indicate the principal binding sites for C8alpha and C5b-7 are located within this domain, and that C8beta binding specificity is not determined by the N- and C-terminal modules. ..
  2. Slade D, Chiswell B, Sodetz J. Functional studies of the MACPF domain of human complement protein C8alpha reveal sites for simultaneous binding of C8beta, C8gamma, and C9. Biochemistry. 2006;45:5290-6 pubmed
    ..Such a result suggests that this segment of C8alpha and corresponding segments of the other MAC family members are independently folded domains. ..
  3. Musingarimi P, Plumb M, Sodetz J. Interaction between the C8 alpha-gamma and C8 beta subunits of human complement C8: role of the C8 beta N-terminal thrombospondin type 1 module and membrane attack complex/perforin domain. Biochemistry. 2002;41:11255-60 pubmed
    ..Results from activity assays suggest that residues within this segment also mediate binding and incorporation of C8 into the MAC. ..
  4. Ng S, Rao A, Howard O, Sodetz J. The eighth component of human complement: evidence that it is an oligomeric serum protein assembled from products of three different genes. Biochemistry. 1987;26:5229-33 pubmed
  5. Föcking M, Sabherwal S, Cates H, Scaife C, Dicker P, Hryniewiecka M, et al. Complement pathway changes at age 12 are associated with psychotic experiences at age 18 in a longitudinal population-based study: evidence for a role of stress. Mol Psychiatry. 2019;: pubmed publisher
    ..subcomponents were significantly upregulated following correction for multiple comparisons (VTN↑, C1RL↑, C8B↑, C8A↑, CFH↑, and C5↑)...
  6. Tseng M, Tsai J, Tsai I, Huang S, Huang J, Fan W, et al. Whole-exome sequencing detects mutations in pediatric patients with atypical hemolytic uremic syndrome in Taiwan. Clin Chim Acta. 2019;494:143-150 pubmed publisher
    ..to be implicated in aHUS (CFH, CFI, CD46, CFHR5, and DGKE), while 4 and 5 mutations were detected on complement- (C8B, C9, and MASP1) and coagulation-associated (VWF and CD36) genes, respectively...
  7. Dellepiane R, Dell Era L, Pavesi P, Macor P, Giordano M, De Maso L, et al. Invasive meningococcal disease in three siblings with hereditary deficiency of the 8(th) component of complement: evidence for the importance of an early diagnosis. Orphanet J Rare Dis. 2016;11:64 pubmed publisher
    ..Serum complement factors were measured by haemolytic assays and immunoblotting. Sequence DNA analysis of the C8B gene was performed...
  8. Goshima M, Sekiguchi R, Matsushita M, Nonaka M. The complement system of elasmobranches revealed by liver transcriptome analysis of a hammerhead shark, Sphyrna zygaena. Dev Comp Immunol. 2016;61:13-24 pubmed publisher
    ..C1r, one C1s, one MASP-1/-3, one MASP-2, two factor B/C2, one C3, three C4, one C5, one C6, one C7, one C8A, three C8B, one C8G, one C9, two factor I and one S protein. No MBL, ficolin, C1qA or C1qC were found...
  9. 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. ..

More Information

Publications45

  1. 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. ..
  2. Parker C, Sodetz J. Role of the human C8 subunits in complement-mediated bacterial killing: evidence that C8 gamma is not essential. Mol Immunol. 2002;39:453-8 pubmed
    ..Furthermore, they provide the first direct evidence that C8 gamma is not required for complement-mediated killing of Gram-negative bacteria. ..
  3. Plumb M, Scibek J, Barber T, Dunlap R, Platteborze P, Sodetz J. Chimeric and truncated forms of human complement protein C8 alpha reveal binding sites for C8 beta and C8 gamma within the membrane attack complex/perforin region. Biochemistry. 1999;38:8478-84 pubmed
    ..They also suggest that the N-terminal modules in C8 alpha are important for C9 binding and/or expression of C8 activity. ..
  4. Alper C, Marcus D, Raum D, Petersen B, Spira T. Genetic polymorphism in C8 beta-chains. Evidence for two unlinked genetic loci for the eighth component of human complement (C8). J Clin Invest. 1983;72:1526-31 pubmed
    ..from unrelated Caucasians suggested autosomal codominant inheritance of three structural alleles at a single locus, C82: C82 degrees A (acidic), C82 degrees B (basic), and C82 degrees A1 (very acidic) with frequencies of 0.952, 0...
  5. Rogde S, Teisberg P, Olaisen B. C8A and C8B polymorphisms in Norwegians and Norwegian lapps. Hum Hered. 1989;39:81-8 pubmed
    ..for such alleles was not found in C8A (alpha-gamma complex), two observations of null allele segregation in C8B (beta chain) indicate a C8BQ*0 allele frequency of about 0.07...
  6. Muller Eberhard H. Molecular organization and function of the complement system. Annu Rev Biochem. 1988;57:321-47 pubmed
  7. Haefliger J, Tschopp J, Nardelli D, Wahli W, Kocher H, Tosi M, et al. Complementary DNA cloning of complement C8 beta and its sequence homology to C9. Biochemistry. 1987;26:3551-6 pubmed
    ..In particular, the cysteine-rich domains and membrane-inserting regions of C9 are well conserved. These findings are discussed in relation to a possible mechanism of membrane attack complex formation. ..
  8. Barba G, Kaufmann T, Schneider P, Rittner C, Brai M. Polymorphism of the complement C8A and -B genes in two families with C8 beta deficiency and neisserial infections. Clin Immunol Immunopathol. 1994;72:83-9 pubmed
    ..described PCR system, demonstrate the presence of a C-T exchange, which creates a stop codon in exon 9 of the C8B gene, as cause for the C8 beta deficiency...
  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. ..
  10. 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
    ..These results provide a structural framework for understanding MAC pore formation and regulation, processes important for fighting infections and preventing complement-mediated tissue damage. ..
  11. Nicholson A, Lepow I. Host defense against Neisseria meningitidis requires a complement-dependent bactericidal activity. Science. 1979;205:298-9 pubmed
    ..Thus, serum complement-dependent bactericidal activity may be especially critical for the host's defense against invasive Neisseria species. ..
  12. Bellavia D, Schneider P, Rittner C, Malfitano G, Kaufmann T, Brai M. Detection of heterozygous C8 beta deficiency by PCR in a healthy Italian population. Exp Clin Immunogenet. 1996;13:173-80 pubmed
    ..In this study we have screened a cohort of 527 Italian blood donors from western Sicily for the presence of C8B mutated allele...
  13. Hu Y, Malone J, Fagan A, Townsend R, Holtzman D. Comparative proteomic analysis of intra- and interindividual variation in human cerebrospinal fluid. Mol Cell Proteomics. 2005;4:2000-9 pubmed
    ..These results validate our method of identifying differences in proteomic profiles of CSF samples and have important implications for the design of CSF biomarker studies for AD and other central nervous system disorders. ..
  14. Rogde S, Olaisen B, Gedde Dahl T, Teisberg P. The C8A and C8B loci are closely linked on chromosome 1. Ann Hum Genet. 1986;50:139-44 pubmed
    ..demonstrated between the loci governing the polymorphisms of complement component C8 alpha-gamma (C8A) and beta (C8B). Both C8 loci were linked to the chromosome 1 marker loci PGM1 and Rh...
  15. Scheurer B, Rittner C, Schneider P. Expression of the human complement C8 subunits is independently regulated by interleukin 1 beta, interleukin 6, and interferon gamma. Immunopharmacology. 1997;38:167-75 pubmed
    ..In contrast, only IFN-gamma but not IL-6 and IL-1 beta had an increasing effect on the expression of C8 beta. Thus the total amount of assembled functionally active C8 appears to be limited by the rate of C8 beta expression. ..
  16. Rogde S, Mevag B, Teisberg P, Olaisen B, Sodetz J. A BamH1 RFLP of the C8B gene. Nucleic Acids Res. 1989;17:6760 pubmed
  17. Saucedo L, Ackermann L, Platonov A, Gewurz A, Rakita R, Densen P. Delineation of additional genetic bases for C8 beta deficiency. Prevalence of null alleles and predominance of C-->T transition in their genesis. J Immunol. 1995;155:5022-8 pubmed
    ..The basis for this apparent high frequency of C-->T transitions occurring in a relatively short stretch of DNA is uncertain. ..
  18. Kaufmann T, Rittner C, Schneider P. The human complement component C8B gene: structure and phylogenetic relationship. Hum Genet. 1993;92:69-75 pubmed
    ..C8A, C8B, and C8G. In serum, the beta-subunit is non-covalently bound to the disulfide-linked alpha-gamma subunit...
  19. Dewald G, Hemmer S, Nöthen M. Human complement component C8. Molecular basis of the beta-chain polymorphism. FEBS Lett. 1994;340:211-5 pubmed
    ..C8 exhibits a structural genetic polymorphism: using isoelectric focusing two major allotypes can be identified (C8B B ('basic') and C8B A ('acidic'))...
  20. Kaufman K, Snider J, Spurr N, Schwartz C, Sodetz J. Chromosomal assignment of genes encoding the alpha, beta, and gamma subunits of human complement protein C8: identification of a close physical linkage between the alpha and the beta loci. Genomics. 1989;5:475-80 pubmed
    ..These were governed by separate loci designated C8A and C8B for alpha-gamma and beta, respectively...
  21. Späth G, Ramadori G, Rittner C, Schneider P. Expression of the complement C8 genes during interleukin-6-mediated in vitro induction of the acute-phase response. Exp Clin Immunogenet. 1995;12:53-60 pubmed
    ..C8A and C8B are closely linked on chromosome 1p and C8G is located on chromosome 9q...
  22. Howard O, Rao A, Sodetz J. Complementary DNA and derived amino acid sequence of the beta subunit of human complement protein C8: identification of a close structural and ancestral relationship to the alpha subunit and C9. Biochemistry. 1987;26:3565-70 pubmed
    ..For beta and alpha, the overall homology is 33% on the basis of identity and 53% when conserved substitutions are allowed. For beta and C9, the values are 26% and 47%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS) ..
  23. Rogde S, Mevag B, Teisberg P, Gedde Dahl T, Tedesco F, Olaisen B. Genetic polymorphism of complement component C8. Hum Genet. 1985;70:211-6 pubmed
    ..Using these methods, we could detect both alpha-gamma (C81) and beta (C82) chain polymorphisms in the same gel...
  24. Plumb M, Sodetz J. An indel within the C8 alpha subunit of human complement C8 mediates intracellular binding of C8 gamma and formation of C8 alpha-gamma. Biochemistry. 2000;39:13078-83 pubmed
  25. Rogde S, Høiby E, Teisberg P, Olaisen B. Genetic aspects of complement component C8 in Norwegian meningococcal disease patients. Scand J Infect Dis. 1990;22:673-9 pubmed
    ..In the patient group, one C8B deficient individual was discovered; none in the control group. No case of C8A deficiency was encountered...
  26. Rogde S, Gedde Dahl T, Teisberg P, Jonassen R, Høyheim B, Olaisen B. Linkage and association studies with C8A and C8B RFLPs on chromosome 1. Ann Hum Genet. 1992;56:233-42 pubmed
    Linkage relations for the C8A and C8B BamHI RFLPs have been investigated. A peak lod score of 4.52 at recombination fraction zero was obtained between the two C8 genes. Combined with our previously obtained linkage data (Rogde et al...
  27. Densen P, McRill C. Differential functional expression of the C8 subunits. Primary role of C8 beta in assembly of intact C8. J Immunol. 1988;141:2674-9 pubmed
    ..Thus the availability of C8 beta determines the quantity of C8 produced. Further, these data suggest the possibility that the C8 structural genes may be differentially expressed despite their close genetic linkage. ..
  28. Kaufmann T, Hansch G, Rittner C, Spath P, Tedesco F, Schneider P. Genetic basis of human complement C8 beta deficiency. J Immunol. 1993;150:4943-7 pubmed
    ..is a serum protein consisting of three chains (alpha, beta, and gamma) and encoded by three different genes, C8A, C8B, and C8G. C8A and C8B are closely linked on chromosome 1p, whereas C8G is located on chromosome 9q...
  29. Bubeck D, Roversi P, Donev R, Morgan B, Llorca O, Lea S. Structure of human complement C8, a precursor to membrane attack. J Mol Biol. 2011;405:325-30 pubmed publisher
    ..Here, we demonstrate that both the C8? protrusion and the C8?-MACPF region that inserts into the membrane upon activation are accessible. ..
  30. Lovelace L, Cooper C, Sodetz J, Lebioda L. Structure of human C8 protein provides mechanistic insight into membrane pore formation by complement. J Biol Chem. 2011;286:17585-92 pubmed publisher
    ..This leads to a model of the MAC that explains how C8-C9 and C9-C9 interactions could facilitate refolding and insertion of putative MACPF transmembrane β-hairpins to form a circular pore. ..
  31. Kotnik V, Luznik Bufon T, Schneider P, Kirschfink M. Molecular, genetic, and functional analysis of homozygous C8 beta-chain deficiency in two siblings. Immunopharmacology. 1997;38:215-21 pubmed
    ..Genetic analysis at the genomic DNA level demonstrated the common C-T mutation in exon 9 of the C8B gene...
  32. Xu Y, Yu Y, Zhang X, Huang Z, Li H, Dong S, et al. Molecular characterization and expression analysis of complement component 3 in dojo loach (Misgurnus anguillicaudatus). Fish Shellfish Immunol. 2018;72:484-493 pubmed publisher
    ..in significant up-regulated expression of other complement-relevant genes in gill, liver and skin, such as C4, C5, C8b, especially at 24 h and 36 h...
  33. 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
    ..Since this module has been found in a large number of other proteins, the results suggest further candidates for C-mannosylation. ..
  34. Steckel E, York R, Monahan J, Sodetz J. The eighth component of human complement. Purification and physicochemical characterization of its unusual subunit structure. J Biol Chem. 1980;255:11997-2005 pubmed
    ..These results indicate that C8 is an atypical serum protein with regard to both its subunit structure and denaturation characteristics. ..
  35. Stewart J, Kolb W, Sodetz J. Evidence that C5b recognizes and mediates C8 incorporation into the cytolytic complex of complement. J Immunol. 1987;139:1960-4 pubmed
    ..They also reveal that C5b contains a specific site for interaction with beta. ..
  36. Raum D, Spence M, Balavitch D, Tideman S, Merritt A, Taggart R, et al. Genetic control of the eighth component of complement. J Clin Invest. 1979;64:858-65 pubmed
    ..Two alleles, C8A and C8B, have been identified in orientals, with gene frequencies of 0.655 and 0.345...