Target intelligence / Profile preview

High Molecular Weight von Willebrand Factor Multimer (HMW-VWF Multimer)

Target
HMW-VWF Multimer
Molecular classification
Glycoprotein, Multimeric Protein
01

Overview

High molecular weight von Willebrand factor (VWF) multimers are the largest and most functionally active forms of VWF, a multimeric glycoprotein essential for hemostasis. VWF is synthesized as a large precursor protein that undergoes multimerization, resulting in a range of sizes. The high molecular weight forms are particularly important for mediating platelet adhesion and aggregation at sites of vascular injury. These large structures have an α–β–α sandwich fold within their subdomains, and are cleaved by the metalloprotease ADAMTS13. Deficiency or dysfunction leads to bleeding disorders such as von Willebrand disease (VWD), while excessive accumulation due to impaired cleavage can cause microvascular thrombosis.

Other names
Large von Willebrand Factor MultimersHigh Molecular Weight VWFHMW VWFUltra-large von Willebrand factor multimers
02

Mechanism of action

Enhances platelet adhesion and aggregation under high shear stress by binding to platelet glycoprotein Ib/IX/V and subendothelial collagen. Stabilizes Factor VIII.

03

Biological functions

Platelet adhesionPlatelet aggregationPrimary hemostasisFactor VIII bindingCollagen binding
04

Disease associations

von Willebrand Disease (VWD)Thrombotic Thrombocytopenic Purpura (TTP)Bleeding disordersMicrovascular thrombosis
05

Safety considerations

Excessive accumulation can cause microvascular thrombosis.Deficiency leads to bleeding tendency.Inhibition can lead to increased risk of bleeding
06

Interacting drugs

ADAMTS13 (therapeutic, enzyme replacement)

1 more in the full profile.

07

Biomarkers

VWF multimer distributionVWF antigen levelsADAMTS13 activity

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