
doi: 10.2741/chan , 10.2741/a607
pmid: 11171546
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.
Hemostasis, Serine Proteinase Inhibitors, Factor VII Deficiency, Lipoproteins, Fibrinogen, Gene Expression, Coagulation Protein Disorders, Factor VII, Kidney, Mice, Phenotype, Animals, Newborn, Liver, Gene Targeting, Animals, Humans
Hemostasis, Serine Proteinase Inhibitors, Factor VII Deficiency, Lipoproteins, Fibrinogen, Gene Expression, Coagulation Protein Disorders, Factor VII, Kidney, Mice, Phenotype, Animals, Newborn, Liver, Gene Targeting, Animals, Humans
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