General description
Four and a half LIM domains protein 1 is a protein encoded by the FHL1 gene in human and located on human chromosome Xq27.2. Factor IX (or Christmas factor) is one of the serine proteases of the coagulation system. It belongs to peptidase family S1. The proteins belong to a novel family of LIM proteins that are expressed in human skeletal muscle.
Monoclonal Anti-Human Factor IX (mouse IgG1 isotype) is derived from the HIX-5 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 is a 55 kDa, single chain, vitamin K-dependent plasma zymogen which plays a key role in the intrinsic and extrinsic blood coagulation systems. A disulfide bond in factor IX connects the N-terminal sequence (light chain) of factor IX to the C-terminal sequence (heavy chain).
Immunogen
Factor IX from pooled normal human plasma.
Application
Monoclonal Anti-Factor IX antibody produced in mouse is suitable for indirect ELISA and western blotting at a dilution of 1:500.
Monoclonal Anti-Factor IX has been used in circulating protein assay. It may also be used in the preparation of factor IX depleted plasma and for purification of factor IX.
Biochem/physiol Actions
Four and a half LIM domains protein 1 (FHL1) downregulation in oral squamous cell carcinoma (OSCC) occurs through DNA methylation of the promoter region rather than histone deacetylation or mutation. Upon activation of factor IX to factor IXa by factor XIa in the intrinsic system, an 11 kDa activation peptide is removed from the factor IX molecule by cleavage of two peptide bonds. These changes allow the exposure of the serine protease site on the heavy chain which can then activate factor X in the presence of factor VIII, Ca2+, and phospholipid. When patients lack Vitamin K, or take oral anticoagulants that interfere with the metabolism of vitamin K, a hypo coagulable or antithrombotic state is induced. This state stems from the diminished ability of factor IX to bind to phospholipids. Factor IX is synthesized in liver parenchymal cells and requires a post-translational, vitamin K-dependent, modification to become a mature plasma zymogen. Hereditary deficiencies or dysfunctions of factor IX cause hemophilia B or Christmas disease.
Physical form
Solution in 10 mM HEPES, pH 7.4, with 140 mM sodium chloride and 0.05% sodium azide.
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.