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Factor IX (EC 3.4.21.22), also known as Christmas factor, is one of the serine proteases involved in coagulation; it belongs to peptidase family S1. Deficiency of this protein causes haemophilia B. It was discovered in 1952 after a young boy named Stephen Christmas was found to be lacking this exact factor, leading to haemophilia. [5] .
Hemophilia B, also called factor IX (FIX) deficiency or Christmas disease, is a genetic disorder caused by missing or defective factor IX, a clotting protein. Although it is passed down from parents to children, about 1/3 of cases are caused by a spontaneous mutation, a change in a gene.
Factor IX is a protein produced naturally in the body. It helps the blood form clots to stop bleeding. Injections of factor IX are used to treat hemophilia B, which is sometimes called Christmas disease.
Two products for adeno-associated virus (AAV)-mediated gene therapy for hemophilia B are now approved for use in adult males with factor IX levels ≤ 2%. Most individuals who receive gene therapy for hemophilia B will have mild hemophilia following treatment, although levels vary widely.
Factor IX (FIX, Christmas factor) is a blood clotting factor, a zymogen of serine protease. Upon activation, FIX is converted into the active serine protease and, in the presence of Ca 2+ and membrane phospholipids, it hydrolyses one arginine-isoleucine bond in factor X to form the activated factor X (Xa) [ 1 ].
Factor IX is a vitamin K-dependent zymogen that, when absent or deficient, results in a bleeding disorder called hemophilia B. It is activated by either factor XIa/Ca++ or factor VIIa/tissue factor/Ca++ and functions as a serine protease in the coagulation cascade, converting factor X to factor Xa.
Hemophilia B is a rare genetic bleeding disorder in which affected individuals have insufficient levels of a blood protein called factor IX. Factor IX is a clotting factor. Clotting factors are specialized proteins needed for blood clotting, the process by which blood seals a wound to stop bleeding and promote healing.
Factor IX consists of an NH2-terminal Gla domain, two epidermal growth factor (EGF)-like domains, and a C-terminal domain containing Ser in its active site. Factor IX deficiency in human plasma results in the disease known as hemophilia B.
Human coagulation factor IX (FIX) is a vitamin K dependent glycoprotein. It is used in therapy of factor IX deficiency, known as haemophilia B. This article explained the coagulation process involved in FIX and the principle of haemophilia B caused by its absence.
The factor IX test is part of a larger screening test to find out what type of bleeding disorder you have. Factor IX is a protein that helps your blood clot. If you are lacking this protein, you may have a bleeding disorder called hemophilia B. Hemophilia B is found mostly in males.