The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
生理活性Activity is determined in a one-stage clotting assay.
% SDS-PAGE. ab95260 is prepared from highly purified factor IX by activation with factor XIa. The factor IXa is further purified by gel filtration, followed by immunoaffinity purification. Purity is assessed by SDS-PAGE analysis.
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Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
Constituents: 50% Glycerol
This product is an active protein and may elicit a biological response in vivo, handle with caution.
Coagulant factor IX
Coagulation factor IX
Coagulation factor IXa heavy chain
Plasma thromboplastin component
PLASMA THROMBOPLASTIN COMPONENT DEFICIENCY
機能Factor IX is a vitamin K-dependent plasma protein that participates in the intrinsic pathway of blood coagulation by converting factor X to its active form in the presence of Ca(2+) ions, phospholipids, and factor VIIIa.
組織特異性Synthesized primarily in the liver and secreted in plasma.
関連疾患Defects in F9 are the cause of recessive X-linked hemophilia B (HEMB) [MIM:306900]; also known as Christmas disease. Note=Mutations in position 43 (Oxford-3, San Dimas) and 46 (Cambridge) prevents cleavage of the propeptide, mutation in position 93 (Alabama) probably fails to bind to cell membranes, mutation in position 191 (Chapel-Hill) or in position 226 (Nagoya OR Hilo) prevent cleavage of the activation peptide. Defects in F9 are the cause of thrombophilia due to factor IX defect (THR-FIX) [MIM:300807]. A hemostatic disorder characterized by a tendency to thrombosis.
配列類似性Belongs to the peptidase S1 family. Contains 2 EGF-like domains. Contains 1 Gla (gamma-carboxy-glutamate) domain. Contains 1 peptidase S1 domain.
ドメインCalcium binds to the gamma-carboxyglutamic acid (Gla) residues and, with stronger affinity, to another site, beyond the Gla domain.
翻訳後修飾Activated by factor XIa, which excises the activation peptide. The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.