Deficiency of protein C inhibitor in combined factor V/VIII deficiency disease.

Abstract

Activated protein C is an anticoagulant plasma protease enzyme that inactivates Factors V and VIII in plasma. Normal plasma contains a protein that inhibits activated protein C and that is distinct from previously described plasma protease inhibitors. Protein C inhibitory activity is not detectable in plasmas from four unrelated patients with combined Factor V/VIII deficiency but is present in normal amounts in plasmas from patients with simple factor V deficiency or Factor VIII deficiency. It is suggested that the molecular basis for combined Factor V/VIII deficiency that exhibits simple autosomal recessive inheritance is a deficiency of protein C inhibitor.

• Authors: R A Marlar, J H Griffin
• Journal: The Journal of clinical investigation (J Clin Invest) Vol. 66 Issue 5 Pg. 1186-9 (Nov 1980) ISSN: 0021-9738 [Print] UNITED STATES
• PMID: 6253526 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Blood Proteins
• Protein C Inhibitor

Topics

• Blood Proteins (deficiency)
• Chromatography, Ion Exchange
• Factor V Deficiency (blood)
• Hemophilia A (blood)
• Protein C Inhibitor