2017-5-4
英文名称 Anti-C7
中文名称 补体C7抗体
别 名 C7; CO7_HUMAN; complement component 7; Complement component C7.
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Function : Constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells. C7 serves as a membrane anchor.
Subunit : Monomer or dimer; as a C5b-7 complex it can also form multimeric rosettes. MAC assembly is initiated by protelytic cleavage of C5 into C5a and C5b. C5b binds sequentially C6, C7, C8 and multiple copies of the pore-forming subunit C9.
Subcellular Location : Secreted.
Post-translational modifications : C7 has 28 disulfide bridges.
O- and C-glycosylated. O-glycosylated with core 1 or possibly core 8 glycans.
DISEASE : Defects in C7 are a cause of complement component 7 deficiency (C7D) [MIM:610102]. A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis.
Similarity : Belongs to the complement C6/C7/C8/C9 family.
Contains 1 EGF-like domain.
Contains 1 LDL-receptor class A domain.
Contains 1 MACPF domain.
Contains 2 Sushi (CCP/SCR) domains.
Contains 2 TSP type-1 domains.
Database links : UniProtKB/Swiss-Prot: P10643.2
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