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C6  Protein, Antibody, ELISA Kit, cDNA Clone

発現宿主: Human Cells  
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12426-H08H-200
12426-H08H-100
200 µg 
100 µg 
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C6 Related Area

C6 サマリー & タンパク質情報

C6 背景

遺伝子の概要: C6 is a component of complement cascade. It is part of the membrane attack complex which can insert into the cell membrane and cause cell to lyse. People with C6 deficiency are prone to bacterial infection. Two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2008]
General information above from NCBI
触媒活性: ATP + H(2)O = ADP + phosphate.
サブユニット構造: Component of the membrane attack complex (MAC). MAC assembly is initiated by proteolytic cleavage of C5 into C5a and C5b. C5b binds sequentially C6, C7, C8 and 12-14 copies of the pore-forming subunit C9. {ECO:0000269|PubMed:22267737}.
細胞内位置: Secreted.
組織特異性: Detected in heart, brain, liver, pancreas, placenta and lung.
翻訳後: All cysteine residues are assumed to be cross-linked to one another. Individual modules containing an even number of conserved cysteine residues are supposed to have disulfide linkages only within the same module.
疾患関連性: DISEASE: Complement component 6 deficiency (C6D) [MIM:612446]: A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis. {ECO:0000269|PubMed:15565285}. Note=Disease susceptibility is associated with variations affecting the gene represented in this entry.
シーケンスの類似性: Belongs to the complement C6/C7/C8/C9 family. {ECO:0000305}.; Contains 1 EGF-like domain. {ECO:0000305}.; Contains 2 Kazal-like domains. {ECO:0000255|PROSITE-ProRule:PRU00798}.; Contains 1 LDL-receptor class A domain. {ECO:0000255|PROSITE-ProRule:PRU00124}.; Contains 1 MACPF domain. {ECO:0000255|PROSITE-ProRule:PRU00745}.; Contains 2 Sushi (CCP/SCR) domains. {ECO:0000255|PROSITE-ProRule:PRU00302}.; Contains 3 TSP type-1 domains. {ECO:0000255|PROSITE-ProRule:PRU00210}.
General information above from UniProt

C6 代替名

complement component C6, [human]
AW111623, [mus-musculus]

C6 関連研究

  • Lengweiler S, et al. (1997) Elucidation of the disulfide-bonding pattern in the factor I modules of the sixth component (C6) of human complement. Biochim Biophys Acta. 1342 (1): 13-8.
  • Chakravarti DN, et al. (1989) Structural homology of complement protein C6 with other channel-forming proteins of complement. Proc Natl Acad Sci U S A. 86 (8): 2799-803.
  • DiScipio RG, et al. (1989) The molecular architecture of human complement component C6. J Biol Chem. 264 (27): 16197-206.
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