KNG1 / BDK / Kininogen-1抗体

KNG1 / BDK / Kininogen-1抗体产品信息

免疫原 :
Recombinant human KNG1 protein ( Catalog#10529-H08H )
Antibody Type : Rabbit Polyclonal Antibody ( Antibody Purification Platform )
抗体宿主 :
Rabbit IgG
缓冲液 : 0.2 μm filtered solution in PBS, 5% trehalose may be added in some batches. Please read the hardcopy of COA or contact our customer service to confirm the formulation.
制备方法 :
Produced in rabbits immunized with purified, human cell-derived, recombinant human KNG1 ( rh KNG1 ; Catalog#10529-H08H ; NP_001095886.1 ; Gln 19 - Ser 644 ). KNG1 specific IgG was purified by human KNG1 affinity chromatography
KNG1 / BDK / Kininogen-1抗体背景综述
Kininogen-1, also known as High molecular weight kininogen, Williams-Fitzgerald-Flaujeac factor, Alpha-2-thiol proteinase inhibitor, Fitzgerald factor, KNG1 and BDK, is a secreted protein which contains three cystatin domains. Kininogen-1 / KNG1 is a protein from the blood coagulation system as well as the kinin-kallikrein system. It is a protein that adsorbs to the surface of biomaterials that come in contact with blood. Kininogen-1 / KNG1 circulates throughout the blood and quickly adsorbs to the material surfaces. Kininogen-1 / KNG1 is one of the early participants of the intrinsic pathway of coagulation, together with Factor XII (Hageman factor) and prekallikrein. Kininogen-1 / KNG1 is one of the kininogens, a class of proteins. As with many other coagulation proteins, the protein was initially named after the patients in whom deficiency was first observed. When the clinical data were combined, it turned out that all patients, in fact, had a deficiency of the same protein. Defects in KNG1 are the cause of high molecular weight kininogen deficiency (HMWK deficiency) which is an autosomal recessive coagulation defect. Patients with HWMK deficiency do not have a hemorrhagic tendency, but they exhibit abnormal surface-mediated activation of fibrinolysis.
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