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Blood, 15 May 2008, Vol. 111, No. 10, pp. 4973-4978. Prepublished online as a Blood First Edition Paper on March 14, 2008; DOI 10.1182/blood-2007-12-126391. This Article Full Text (PDF) All Versions of this Article: blood-2007-12-126391v1 111/10/4973    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Zhang, J. Articles by Broze, G. J. Search for Related Content PubMed PubMed Citation Articles by Zhang, J. Articles by Broze, G. J., Jr. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted December 7, 2007 Accepted February 5, 2008 Protein Z-dependent protease inhibitor deficiency produces a more severe murine phenotype than protein Z deficiency Jing Zhang, Yizheng Tu, Lan Lu, Nina Lasky, and George J. Broze Jr.* Division of Hematology, Washington University, St. Louis, MO, United States * Corresponding author; email: gbroze{at}im.wustl.edu. Protein Z (PZ) is a plasma vitamin K-dependent protein that functions as a cofactor to dramatically enhance the inhibition of coagulation factor Xa by the serpin, protein Z-dependent protease inhibitor (ZPI). In vitro, ZPI not only inhibits factor Xa in a calcium ion-, phospholipid- and PZ-dependent fashion, but also directly inhibits coagulation factor XIa. In murine gene-deletion models, PZ and ZPI deficiency enhance thrombosis following arterial injury and increase mortality from pulmonary thromboembolism following collagen/epinephrine infusion. On a factor V Leiden genetic background, ZPI deficiency produces a significantly more severe phenotype than PZ deficiency, implying that factor XIa inhibition by ZPI is physiologically relevant. The studies in mice suggest that human PZ and ZPI deficiency would be associated with a modest thrombotic risk with ZPI deficiency producing a more severe phenotype. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: Z. Wei, Y. Yan, R. W. Carrell, and A. Zhou Crystal structure of protein Z-dependent inhibitor complex shows how protein Z functions as a cofactor in the membrane inhibition of factor X Blood, October 22, 2009; 114(17): 3662 - 3667. [Abstract] [Full Text] [PDF] F. M.K. Williams, A. M. Carter, B. Kato, M. Falchi, L. Bathum, G. Surdulescu, K. O. Kyvik, A. Palotie, T. D. Spector, P. J. Grant, et al. Identification of Quantitative Trait Loci for Fibrin Clot Phenotypes: The EuroCLOT Study Arterioscler Thromb Vasc Biol, April 1, 2009; 29(4): 600 - 605. [Abstract] [Full Text] [PDF] X. Huang, R. Swanson, G. J. Broze Jr., and S. T. Olson Kinetic Characterization of the Protein Z-dependent Protease Inhibitor Reaction with Blood Coagulation Factor Xa J. Biol. Chem., October 31, 2008; 283(44): 29770 - 29783. [Abstract] [Full Text] [PDF]

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