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  2. von Willebrand disease - Wikipedia

    en.wikipedia.org/wiki/Von_Willebrand_disease

    Von Willebrand disease (VWD) is the most common hereditary blood-clotting disorder in humans. An acquired form can sometimes result from other medical conditions. [1] It arises from a deficiency in the quality or quantity of von Willebrand factor (VWF), a multimeric protein that is required for platelet adhesion. It is known to affect several ...

  3. von Willebrand factor - Wikipedia

    en.wikipedia.org/wiki/Von_Willebrand_factor

    Von Willebrand factor (VWF) (German: [fɔn ˈvɪləbʁant]) is a blood glycoprotein that promotes primary hemostasis, specifically, platelet adhesion.It is deficient and/or defective in von Willebrand disease and is involved in many other diseases, including thrombotic thrombocytopenic purpura, Heyde's syndrome, and possibly hemolytic–uremic syndrome. [5]

  4. GP1BA - Wikipedia

    en.wikipedia.org/wiki/GP1BA

    The Gp Ib functions as a receptor for von Willebrand factor (VWF). The complete receptor complex includes noncovalent association of the alpha and beta subunits with platelet glycoprotein IX and platelet glycoprotein V to form the glycoprotein Ib-IX-V complex .

  5. ADAMTS13 - Wikipedia

    en.wikipedia.org/wiki/ADAMTS13

    ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13)—also known as von Willebrand factor-cleaving protease (VWFCP)—is a zinc-containing metalloprotease enzyme that cleaves von Willebrand factor (vWf), a large protein involved in blood clotting.

  6. Ristocetin - Wikipedia

    en.wikipedia.org/wiki/Ristocetin

    In the case of type 2B vWD, the gain-of-function mutation involves von Willebrand's factor (VWF gene), and in platelet-type vWD, the receptor is the object of the mutation (GPIb). This increased binding causes vWD because the high-molecular weight multimers are removed from circulation in plasma since they remain attached to the patient's ...

  7. Weibel–Palade body - Wikipedia

    en.wikipedia.org/wiki/Weibel–Palade_body

    Weibel–Palade bodies are the storage granules of endothelial cells, the cells that form the inner lining of the blood vessels and heart. [1] They manufacture, store and release two principal molecules, von Willebrand factor and P-selectin, and thus play a dual role in hemostasis and inflammation.

  8. Glycoprotein Ib - Wikipedia

    en.wikipedia.org/wiki/Glycoprotein_Ib

    The GPIb-V-IX complex binds von Willebrand factor, allowing platelet adhesion and platelet plug formation at sites of vascular injury. Glycoprotein Ibα (GPIbα) is the major ligand-binding subunit of the GPIb-V-IX complex. GPIbα is heavily glycosylated. [2] It is deficient in the Bernard–Soulier syndrome.

  9. Von Willebrand factor type D domain - Wikipedia

    en.wikipedia.org/wiki/Von_Willebrand_factor_type...

    Von Willebrand factor type D domain (vWD) is an evolutionarily-conserved protein domain found in, among others, the von Willebrand factor (vWF). vWF is a large multimeric glycoprotein and it is synthesized by a type of bone marrow cell called megakaryocytes. The vWD domain allows vWF to perform its blood-clotting function by carrying factor ...

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