C4 binding protein β deficiency

Complement component 4-binding protein (C4BP) is one of the two polypeptides that in humans compose the plasma glycoprotein C4b-binding protein. C4BP binds the anticoagulant vitamin K-dependent protein S. It can play a role in the control of the protein C anticoagulatory pathway. Mutations in C4BP are associated with high risk for thromboembolic disorders.

Alternative names

Complement component 4-binding protein beta deficiency


  • Defects of complement regulatory proteins
    • C4-binding protein deficiency


Autosomal recessive


*120831 Complement component 4-binding protein, beta chain; C4BPB

Cross references

Phenotypically related immunodeficiencies

IDR factfile for C4 binding protein α deficiency


Incidence is not known.