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Research Article Free access | 10.1172/JCI110385

Deficiency of protein C in congenital thrombotic disease.

J H Griffin, B Evatt, T S Zimmerman, A J Kleiss, and C Wideman

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Published November 1, 1981 - More info

Published in Volume 68, Issue 5 on November 1, 1981
J Clin Invest. 1981;68(5):1370–1373. https://doi.org/10.1172/JCI110385.
© 1981 The American Society for Clinical Investigation
Published November 1, 1981 - Version history
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Abstract

A family with a history of recurring thrombosis was studied to determine if a plasma protein deficiency could account for the observed disease. Protein C levels in plasma were determined immunologically using the Laurell rocket technique. The propositus, his father, and his paternal uncle, who are severely affected, had 38-49% of normal levels of protein C antigen, whereas unaffected family members had normal levels. There was no familial deficiency of antithrombin III and plasminogen. Because activated protein C is a potent in vitro anticoagulant enzyme and an in vivo profibrinolytic agent, it is suggested that the recurrent thrombotic disease in this family is due to an inherited deficiency in protein C.

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