CFP
Domain
TSP type-1 domain 0 binds to TSP type-1 domain 4, and TSP type-1 domain 1 binds to TSP type-1 domain 6 (PubMed:15491616, PubMed:28264884, PubMed:31507604). These interactions mediate multimerization (PubMed:15491616, PubMed:28264884, PubMed:31507604).
Function
A positive regulator of the alternate pathway (AP) of complement (PubMed:20382442, PubMed:28264884). It binds to and stabilizes the C3- and C5-convertase enzyme complexes (PubMed:20382442, PubMed:28264884). Inhibits CFI-CFH mediated degradation of Complement C3 beta chain (C3b) (PubMed:31507604).
Involvement in disease
Properdin deficiency
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).
None
The disease is caused by variants affecting the gene represented in this entry.
Cellular localization
- Secreted
Alternative names
PFC, CFP, Properdin, Complement factor P