The vitamin K dependent coagulation factor IX circulates in the blood as an inactive zymogen at the normal concentration of approximately 3 μg/ml. This factor is converted to an active form by factor XIa, which excises the activation peptide and thus generates a heavy chain and a light chain held together by one or more disulfide bonds. The role of this activated factor IX in the blood coagulation cascade is to activate factor X to its active form through interactions with Ca+2 ions, membrane phospholipids, and factor VIII. Alterations of this gene, including point mutations, insertions and deletions, cause factor IX deficiency, which is a recessive X-linked disorder, also called hemophilia B or Christmas disease.
Type: Primary
Antigen: Factor IX
Clonality: Monoclonal
Clone: [9D]
Conjugation: No
Epitope:
Host: Mouse
Isotype: IgG1
Reactivity: Human