Work

Deficiency of protein C in congenital thrombotic disease.

Year: 1981

Type: article

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.

Source: Journal of Clinical Investigation

DOI: 10.1172/jci110385

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

Institutions: Centers for Disease Control and Prevention, Scripps Clinic

Language: English

Topic: Blood Coagulation and Thrombosis Mechanisms

Subfield: Hematology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: bronze

Funder: National Institutes of Health