CFPbase: Properdin deficiency

Version 1.0 last updated 19 October, 2007 contains 36 public entries


Curated:	Mauno Vihinen
Maintained by:	Jouni Väliaho
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Short description of the CFPbase (previously known as PFCbase) :

Properdin is synthesized by monocytes, hepatocytes, and T cells, and it is a component of the secondary granule in neutrophiles. In some families, properdin is undetectable, while in other affected males may have levels as high as 10% of normal. In other kindreds the serum level of properdin are normal but the protein is dysfunctional. Three phenotypes have been reported: complete deficiency (type I), incomplete deficiency (type II), and dysfunction of properdin (type III).

Other IBT bioinformatics services:
Immunodeficiency Resource (IDR)
Immunodeficiency Mutation Databases (MUTbase)
IDdiagnostics
Immunome Knowledge Base (IKB) - An integrated service for immunome research
SH2base - Database for pathogenic SH2 domain mutations

IDbases are linked to University of California Santa Cruz (UCSC) genome browser: CFPbase