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

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.
AuthorsR A Marlar, J H Griffin
JournalThe Journal of clinical investigation (J Clin Invest) Vol. 66 Issue 5 Pg. 1186-9 (Nov 1980) ISSN: 0021-9738 [Print] UNITED STATES
PMID6253526 (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
  • Blood Proteins (deficiency)
  • Chromatography, Ion Exchange
  • Factor V Deficiency (blood)
  • Hemophilia A (blood)
  • Protein C Inhibitor

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