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Description

Properdin (UniProt: P27918, also known as Complement factor P) is encoded by the CEP (also known as PFC) gene (Gene ID: 5199) in human. (ABF185: Properdin (UniProt P27918, also known as Complement factor P) is encoded by the CFP (also known as PFC, PFD) gene (Gene ID 5199) in human. Properdin is a plasma glycoprotein and the only known positive regulator of the complement cascade. It binds and stabilizes the alternative pathway (AP) C3 convertase C3bBb and may also serve as a platform to form new C3bBb convertases on cell surface. Properdin-mediated AP activation plays an important role in host defense, and properdin deficiency (PFD) increases the risk for Neisseria meningitidis infection in human. Properdin-deficiency exacerbated disease condition in a murine model of factor H- (fH-) related C3 glomerulopathy, but prevented the development of allergen-induced airway inflammation. Human properdin is produced with a signal peptide sequence (a.a. 1-27), which is removed posttranslationally to yield the mature protein (a.a. 28-469) composed entirely of seven TSP type-1 domains (domain 0 through 6, a.a. 28-462). TSP type-1 domains 0 and 6 bind to each other and mediate multimerization.