The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
追加情報WB: Use at a concentration of 1 µg/ml. Predicted molecular weight: 65 kDa (unprocessed precursor).
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
機能Responsible for cleaving the alpha-chains of C4b and C3b in the presence of the cofactors C4-binding protein and factor H respectively.
関連疾患Defects in CFI are a cause of susceptibility to hemolytic uremic syndrome atypical type 3 (AHUS3) [MIM:612923]. 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. Note=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. Defects in CFI are the cause of complement factor I deficiency (CFI deficiency) [MIM:610984]. CFI deficiency is an autosomal recessive condition associated with a propensity to pyogenic infections.
配列類似性Belongs to the peptidase S1 family. Contains 1 Kazal-like domain. Contains 2 LDL-receptor class A domains. Contains 1 peptidase S1 domain. Contains 1 SRCR domain.