IMR Press / FBL / Volume 6 / Issue 3 / DOI: 10.2741/chan

Frontiers in Bioscience-Landmark (FBL) is published by IMR Press from Volume 26 Issue 5 (2021). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on imrpress.com as a courtesy and upon agreement with Frontiers in Bioscience.

Open Access Article
Gene targeting in hemostasis. tissue factor pathway inhibitor
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1 251 Nieuwland Science Hall, W.M. Keck Center for Transgene Research and the Department of Chemistry and Biochemistry, University of Notre Dame, Notre Dame, USA

Academic Editor: Francis Castellino

Front. Biosci. (Landmark Ed) 2001, 6(3), 216–221; https://doi.org/10.2741/chan
Published: 1 February 2001
(This article belongs to the Special Issue Gene targeting in hemostasis)
Abstract

Tissue Factor Pathway Inhibitor (TFPI) is a serine protease inhibitor of the Factor VIIa/Tissue Factor (FVIIa/TF)-initiated clotting cascade. Mice expressing a mutant form of TFPI, in which its Kunitz–1 domain has been deleted (TFPIKu1δ/δ), die prematurely in embryogenesis between E9.5dpc and birth. These results provide a rationale for the absence of TFPI-deficient patients. This early mortality can be ameliorated by an accompanying heterozygous or homozygous deficiency in FVII. Thus, diminishment of FVII activity precludes the requirement for TFPI-mediated inhibition of the FVIIa/TF pathway during embryogenesis.

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