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C6  Protein, Antibody, ELISA Kit, cDNA Clone

表達宿主: Human Cells  
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12426-H08H-200
12426-H08H-100
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100 µg 
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C6 相关研究领域

C6 相關信號通路

C6 概述&蛋白信息

C6 研究背景

基因概述: 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
催化活性: ATP + H(2)O = ADP + phosphate.
亞單位結構: 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}.
亞細胞定位: Secreted.
組織特異性: Detected in heart, brain, liver, pancreas, placenta and lung.
翻譯後修飾: 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.
相關疾病 : 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.
相似的序列: 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 別稱

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

C6 相關文獻

  • 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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