Properdin factor C deficiency

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).

Alternative names

Properdin P factor deficiency


  • Defects of the alternative complement pathway




#312060 Properdin deficiency, X-linked

*300383 Properdin P Factor, Complement PFC

Cross references

Phenotypically related immunodeficiencies

IDR factfile for C4 binding protein α deficiency


Incidence is not known.