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complement factor B  단백질

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complement factor B 요약 및 단백질 정보

complement factor B 배경

촉매 활성: Cleavage of Arg-|-Ser bond in complement component C3 alpha-chain to yield C3a and C3b, and Arg-|-Xaa bond in complement component C5 alpha-chain to yield C5a and C5b.
하부단위 구조: Monomer. {ECO:0000269|PubMed:19574954}.
도메인: The unliganded VWA domain has an inactive 'locked' conformation whereby the scissile Arg-259|Lys-260 bond is protected from proteolytic activation. {ECO:0000269|PubMed:17310251}.
세포하 위치: Secreted.
질병과의 관련성: DISEASE: Hemolytic uremic syndrome atypical 4 (AHUS4) [MIM:612924]: An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease. {ECO:0000269|PubMed:17182750, ECO:0000269|PubMed:20513133}. Note=Disease susceptibility is associated with variations affecting the gene represented in this entry. Susceptibility to the development of atypical hemolytic uremic syndrome can be conferred by mutations in various components of or regulatory factors in the complement cascade system. Other genes may play a role in modifying the phenotype.; DISEASE: Complement factor B deficiency (CFBD) [MIM:615561]: An immunologic disorder characterized by increased susceptibility to bacterial infections, particularly Neisseria infections, due to a defect in the alternative complement pathway. {ECO:0000269|PubMed:24152280}. Note=The disease is caused by mutations affecting the gene represented in this entry.
염기서열 유사성: Belongs to the peptidase S1 family. {ECO:0000255|PROSITE-ProRule:PRU00274}.; Contains 1 peptidase S1 domain. {ECO:0000255|PROSITE-ProRule:PRU00274}.; Contains 3 Sushi (CCP/SCR) domains. {ECO:0000255|PROSITE-ProRule:PRU00302}.; Contains 1 VWFA domain. {ECO:0000255|PROSITE-ProRule:PRU00219}.
General information above from UniProt

complement factor B 대체 이름

ARMD14,BF,FB,BFD,GBG,CFAB,CFBD,PBF2,AHUS4,FBI12,H2-Bf, [homo-sapiens]
B,Bf,C2,Fb,H2-Bf,AI195813,AI255840, [mus-musculus]

complement factor B 관련 연구

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