描述:
Clone GMA-001
物种反应性:Human, Rat

来源:Murine

特异性:Gla domain (residues 1-40) of FIX/FIXa

免疫原:Human Factor IX

同种型:IgG2a

应用:

ELISA:

Binds immobilized Human and Rat FIX, Human FIXa only in the presence of calcium.

Immunoblotting:

Western blot detects Human FIX and light chain of Human FIXa under reduced conditions in presence of calcium.

Inhibition:

Prolongs plasma clot time in APTT clotting assay.

背景:

Factor IX (FIX) is a vitamin K-dependent zymogen that plays an essential role in the coagulation cascade leading to thrombus formation. In the presence of calcium, activated Factor IX (FIXa) complexes with Factor VIIIa on phospholipid surfaces to create the tenase complex, which converts Factor X to its activated form. Absent or defective FIX is the cause of the X-linked recessive bleeding disorder hemophilia B. GMA-001 (BC2) binds to the N-terminal Gla domain of FIX and the light chain of FIXa, as detected by Western blot and ELISA. In addition, GMA-001 prolongs clotting time in APTT-based plasma clotting assays and prevents arterial thrombosis in vivo in a rat model.