Autoimmune Protein S Deficiency in a Boy with Severe Thromboembolic Disease
Open Access
- 17 June 1993
- journal article
- research article
- Published by Massachusetts Medical Society in New England Journal of Medicine
- Vol. 328 (24) , 1753-1757
- https://doi.org/10.1056/nejm199306173282405
Abstract
Protein S is a vitamin K-dependent plasma protein involved in the regulation of the protein C anticoagulant pathway,1 the system responsible for the inactivation of factors Va and VIIIa2. Protein S functions as a cofactor for activated protein C by increasing its affinity for the cell surface3 and by blocking the capacity of factor Xa to protect factor Va from inactivation by activated protein C4; it also enhances the profibrinolytic effects of activated protein C5-7.Keywords
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