Glycoprotein Ib
Encyclopedia
Glycoprotein Ib also known as CD42,
is a component of the GPIb-V-IX complex on platelets. The GPIb-V-IX complex binds von Willebrand factor
, allowing platelet adhesion and platelet plug formation at sites of vascular injury.
It is deficient in the Bernard-Soulier syndrome
. A gain-of-function mutation causes platelet-type von Willebrand's disease.
Autoantibodies against Ib/IX can be produced in immune thrombocytopenic purpura.
Components include GP1BA
and GP1BB
.
It complexes with Glycoprotein IX
.
is a component of the GPIb-V-IX complex on platelets. The GPIb-V-IX complex binds von Willebrand factor
Von Willebrand factor
von Willebrand factor is a blood glycoprotein involved in hemostasis. It is deficient or defective in von Willebrand disease and is involved in a large number of other diseases, including thrombotic thrombocytopenic purpura, Heyde's syndrome, and possibly hemolytic-uremic syndrome...
, allowing platelet adhesion and platelet plug formation at sites of vascular injury.
It is deficient in the Bernard-Soulier syndrome
Bernard-Soulier syndrome
Bernard–Soulier syndrome , also called hemorrhagiparous thrombocytic dystrophy, is a rare autosomal recessive coagulopathy that causes a deficiency of glycoprotein Ib , the receptor for von Willebrand factor, which is important in clot formation.The incidence is estimated to be less than 1 in 1...
. A gain-of-function mutation causes platelet-type von Willebrand's disease.
Autoantibodies against Ib/IX can be produced in immune thrombocytopenic purpura.
Components include GP1BA
GP1BA
Glycoprotein Ib , alpha polypeptide also known as CD42b , is a human gene.-Interactions:GP1BA has been shown to interact with YWHAZ and FLNB.-Further reading:...
and GP1BB
GP1BB
Glycoprotein Ib , beta polypeptide also known as CD42c , is a human gene.-External links:...
.
It complexes with Glycoprotein IX
Glycoprotein IX
Glycoprotein IX also known as CD42a , is a human gene.-External links:...
.