Association of Protein S Deficiency with Thrombosis in a Kindred with Increased Levels of Plasminogen Activator Inhibitor-1
WALTER REED ARMY INST OF RESEARCH WASHINGTON DC
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The most common inherited abnormalities associated with familial venous thrombosis are deficiencies of protein S, protein C, and antithrombin III 1-3. All are inherited in an autosomal dominant manner and can result in venous thrombosis at young age. The most common of these three deficiencies is protein S, which circulates in a free from and in an inactive complex with C4b-binding protein. Only unbound or free protein S has functional activity as a cofactor for activated protein C in inhibiting factors Va and VIIIa 4. Thus, assessment of patients for protein S deficiency includes measurement of the level or activity of free protein S.
- Medicine and Medical Research