Anti Factor IX antibody, Mouse monoclonal (mouse IgG1 isotype) is derived from the HIX-1 hybridoma produced by the fusion of mouse Sp2/0-Ag14 myeloma cells and splenocytes from BALB/c mice immunized with factor IX purified from human plasma. Factor IX concentration in human plasma ranges between 2.5-5 mg/ml and its half-life is approximately 24 hours. The human factor IX gene is about 40 Kb in size and is localized at the distal end of the X-chromosome.
Biochem/physiol Actions
FIX plays a key role in the intrinsic and extrinsic blood coagulation systems. It is synthesized in liver and later upon activation in plasma it gets converted into a serine protease. In second step, FIX undergoes series of complex post-translation modifications such as γ-carboxylation of 12 N-terminal glutamic acid residues, N- and O-linked glycosylation, phosphorylation, β-hydroxylation, sulfation, disulfide bond formation etc. It is secreted into the plasma after completion of post-translation modifications. Hereditary deficiencies or dysfunctions of factor IX cause hemophilia B or "Christmas Disease" (the surname of the first family described). In haemophilia B, FIX showed impaired coagulation and increased tendency to bleed as a reason of mutation in the X chromosome linked FIX gene. Factor IX is synthesized in liver parenchymal cells and requires a post-translational vitamin K-dependent modification in order to become a mature plasma zymogen. When patients lack vitamin-K or take oral anticoagulants that interfere with the metabolism of vitamin-K, a hypocoagulable or antithrombotic state is induced.
Anti-Factor IX antibody, Mouse monoclonal 준비 용품 및 원자재
