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- W2912461913 abstract "C9 is a single-chain plasma glycoprotein. It is a member of the membrane attack complex/perforin-like (MACPF)/CDC superfamily. The sole function of C9 is to form the cytolytic membrane attack complex, an important antibacterial defense. The membrane-bound MAC precursor complex, comprising C5b, C6, C7 and C8, recruits C9 from the fluid phase and facilitates its unfolding and insertion into the membrane, aligned with the C8 α-chain. Unfolding of the first C9 exposes binding sites for the next C9 molecule which binds, unfolds, inserts and aligns alongside the previous molecule. This sequential recruitment then continues until the ring structure of the MAC is complete. C9 is present in human plasma at a concentration of 46–66 mg/L (mean ± sd from local control cohort) and in cerebrospinal fluid (0.2 mg/L mean in local control cohort). C9 is a moderate acute-phase reactant, and levels increase 1.5- to 2-fold in response to acute inflammatory triggers. C9 deficiency is common in Japan with a homozygous frequency of ∼1:1000 (carrier frequency of ∼6%). It is the most common inherited immunodeficiency in this population." @default.
- W2912461913 created "2019-02-21" @default.
- W2912461913 creator A5018121418 @default.
- W2912461913 date "2018-01-01" @default.
- W2912461913 modified "2023-09-25" @default.
- W2912461913 title "C9" @default.
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- W2912461913 doi "https://doi.org/10.1016/b978-0-12-810420-0.00022-5" @default.
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