Bernard-Soulier syndrome is a bleeding disorder caused by a defect of glycoprotein Ib (GpIb) on platelets. Platelets lacking GpIb cannot adhere to the wall of the damaged blood vessels, because GpIb is the receptor for which essential coagulation protein?
A) Hageman factor (factor XII)
B) Von Willebrand factor
C) Fibrinogen
D) Factor VIII
E) Thrombin

Question

Quizplus · Accepted Answer

GpIb, a platelet cell membrane glycoprotein, is the receptor for the von Willebrand factor (vWF), which mediates the adhesion of platelets to the damaged vascular wall. Congenital deficiency of GpIb, as seen in Bernard-Soulier syndrome, is similar to the deficiency of vWF in von Willebrand disease and results in a bleeding disorder.

Bernard-Soulier Syndrome Is a Bleeding Disorder Caused by a Defect