C6  Protein, Antibody, ELISA Kit, cDNA Clone

발현 숙주: Human Cells  
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12426-H08H-200
12426-H08H-100
200 µg 
100 µg 
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C6 Related Area

C6 요약 및 단백질 정보

C6 배경

유전자 요약: 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
촉매 활성: ATP + H(2)O = ADP + phosphate.
하부단위 구조: 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}.
세포하 위치: Secreted.
조직 특이성: Detected in heart, brain, liver, pancreas, placenta and lung.
번역 후: 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.
질병과의 관련성: 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.
염기서열 유사성: 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 대체 이름

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

C6 관련 연구

  • 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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