GMA-8013

Clone GMA-8013

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-8013 (also known as 3E6 or 3E6.1B)¹ recognizes the C2 domain of FVIII, and the X-ray crystal structure of this interaction has been determined.² GMA-8013 is an inhibitor of FVIII activation, and is suitable for surface plasmon resonance,³ ELISA, and bio-layer interferometry pairing experiments.