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Prothrombotic Phenotype of Protein Z Deficiency

Zheng-Feng Yin, Zhong-Fu Huang, Jisong Cui, Ryan Fiehler, Nina Lasky, David Ginsburg and George J. Broze, Jr.
Proceedings of the National Academy of Sciences of the United States of America
Vol. 97, No. 12 (Jun. 6, 2000), pp. 6734-6738
Stable URL: http://www.jstor.org/stable/122706
Page Count: 5
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Prothrombotic Phenotype of Protein Z Deficiency
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Abstract

Protein Z (PZ) is a vitamin K-dependent plasma protein whose function has been uncertain. The structure of PZ is very similar to that of the coagulation-related factors VII, IX, and X and PC, but PZ differs from these other proteins in that it is not the zymogen of a serine protease. We have shown recently that PZ forms a calcium ion-dependent complex with activated factor X at phospholipid surfaces and that this interaction leads to the inhibition of activated factor X activity through, in part, the action of a previously unidentified plasma protein named PZ-dependent protease inhibitor. Herein, we report that the presence of PZ dampens the coagulation response in human plasma and that concomitant PZ deficiency dramatically increases the severity of the prothrombotic phenotype of factor VLeiden mice. The results indicate that PZ plays a physiologically important role in the regulation of coagulation.

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