Article
Mutations of TFPI-binding exosites on factor VII cause bleeding phenotypes in factor VII deficiency.
Blood advances - 22 Nov 2022
Seanoon Karnsasin, Payongsri Panwajee, Vivithanaporn Pornpun, Sirachainan Nongnuch, Chuansumrit Ampaiwan, Hongeng Suradej, Tanratana Pansakorn
Abstract excerpt
Tissue factor (TF) pathway inhibitor (TFPI) is a Kunitz-type anticoagulation protein that inhibits activated factor VII (FVIIa)/TF complex. Incidentally, many different F7 gene variants, including TFPI-binding exosite mutations, have been reported in patients with congenital FVII deficiency and clinical bleeding variabilities. Here, TFPI-binding exosites (R147 and K192) on FVII zymogen were selectively disrupted...
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