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Factor Ix





PHYSIOLOGY

Factor IX is inactive unless activated by Factor XI a (of the contact pathway) or Factor VII a (of the tissue factor pathway). When activated into factor IXa, it acts by hydrolysing one Arginine - Isoleucine bond in Factor X to form factor Xa. It requires Calcium , membrane Phospholipid s, and Factor VIII as Cofactor s to do so.


GENETICS

The Gene for factor IX is located on the X Chromosome (Xq27.1-q27.2).


ROLE IN DISEASE

Deficiency of factor IX causes Christmas disease ( Hemophilia B ). Over 100 mutations of factor IX have been described; some cause no symptoms, but many lead to a significant bleeding disorder.


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