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. 1980 Nov;66(5):1186–1189. doi: 10.1172/JCI109952

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

R A Marlar, J H Griffin
PMCID: PMC371561  PMID: 6253526

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.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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