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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

Gene targeting in hemostasis. tissue factor pathway inhibitor.

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 (TFPIKu1delta/delta), 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.[1]


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