6sej
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proteopedia linkproteopedia linkStructure of a functional monomeric properdin lacking TSR3
Structural highlights
DiseasePROP_HUMAN Defects in CFP are the cause of properdin deficiency (PFD) [MIM:312060. PFD results in higher susceptibility to bacterial infections; especially to meningococcal infections. Three phenotypes have been reported: complete deficiency (type I), incomplete deficiency (type II), and dysfunction of properdin (type III).[1] [2] [3] FunctionPROP_HUMAN A positive regulator of the alternate pathway of complement. It binds to and stabilizes the C3- and C5-convertase enzyme complexes. See AlsoReferences
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