GMA-8026

Clone GMA-8026

Background

Factor VIII (FVIII) is a heterodimer consisting of a heavy chain (ranging in mass from 90 to 200 kDa) bound via metal ions to a light chain (80 kDa). In plasma, FVIII circulates in an inactive form bound to von Willebrand factor. Following activation by factor Xa or thrombin, factor VIIIa can function as cofactor for the enzyme factor IXa in the activation of factor X in the presence of phospholipid and Ca²⁺. Absent or defective FVIII is the cause of the X-linked recessive bleeding disorder hemophilia A. GMA-8026 (also known as G99)¹ recognizes the C2 domain of FVIII through the Pro2221-Trp2229 loop², inhibits FVIII activation by thrombin or factor Xa, and is unaffected by FVIII binding to vWF.³