Specific cleavage of Lys-147/Gly-1448 in the serine protease domain of human factor IXabeta by plasmin:effect on catalytic efficiency and factor VIIIa binding

摘要

Hemophilia B is an X-linked recessive bleeding disorder that is caused by the absence or deficiency of Factor IX protein.Human blood clotting factor IX circulates in blood as a single chain protein of 415 amino acids.Upon activation (by factor XIa/Ca~(2+) or TF/VIIa/CA~(2+)),two peptide bonds in factor IX are cleaved with resultant formation of a serine protease,Factor IXabeta,and release of an activation peptide.