What are VWF multimers?
VWF is a multimeric glycoprotein built from identical subunits that contain binding sites for both platelet glycoprotein receptors and collagen. The adhesive activity of VWF depends on the size of its multimers, which range from 500 to over 10 000 kDa.
Is TTP the same as von Willebrand disease?
Thrombotic thrombocytopenic purpura (TTP) is a disorder with characteristic von Willebrand factor (VWF)-rich microthrombi affecting the arterioles and capillaries of multiple organs. The disorder frequently leads to early death unless the patients are treated with plasma exchange or infusion.
What cleaves VWF multimers?
ADAMTS13 is a plasma protease that cleaves circulating VWF between Tyr 1605 and Met 1606 in the A2 domain. Its main target is the ULvWF multimers, which spontaneously bind GPIbα on platelets.
What are Multimers?
Noun. multimer (plural multimers) (biochemistry) A protein consisting of multiple monomers.
What are the different types of von Willebrand disease?
The main types are:
- type 1 – the mildest and most common type. People with type 1 VWD have a reduced level of von Willebrand factor in their blood.
- type 2 – in people with this type of VWD, von Willebrand factor does not work properly.
- type 3 – the most severe and rarest type.
Is ITP and TTP the same?
Are ITP and TTP the same thing? No, ITP and TTP are not the same thing. Both ITP and TTP are bleeding disorders, but they occur for different reasons and may require different treatments.
What is TTP?
TTP is a rare, life-threatening blood disorder. In TTP, blood clots form in small blood vessels throughout your body. The clots can limit or block the flow of blood to your organs, such as your brain, kidneys, and heart. This can prevent your organs from working properly and can damage your organs.
What cleaves von Willebrand?
Human von Willebrand factor (VWF) is cleaved by plasmin in a shear-dependent manner.
What glycoprotein is responsible for platelet aggregation?
Activated glycoprotein IIb/IIIa receptors
Activated glycoprotein IIb/IIIa receptors become receptive to fibrinogen, and when fibrinogen binds to the glycoprotein IIb/IIIa receptors located on two different platelets it builds the cross-links for platelet-to-platelet aggregation. The glycoprotein IIb/IIIa also mediates platelet adhesion and spreading.
What is ADAMTS13 inhibitor?
ADAMTS13 is a plasma protein that regulates the interaction of platelets with von Willebrand factor. Absent or low ADAMTS13 activity allows formation of platelet microthrombi, which in turn obstruct arterioles and capillaries, generating the clinical sequelae of TTP.
What is the role of ADAMTS13?
Normal Function The ADAMTS13 gene provides instructions for making an enzyme that is involved in regulating blood clotting. After an injury, clots normally protect the body by sealing off damaged blood vessels and preventing further blood loss. The ADAMTS13 enzyme processes a large protein called von Willebrand factor.