Article
Identification of a point mutation in type IIB von Willebrand disease illustrating the regulation of von Willebrand factor affinity for the platelet membrane glycoprotein Ib-IX receptor.
Proceedings of the National Academy of Sciences of the United States of America - 1 Apr 1991
Ware J, Dent J A, Azuma H, Sugimoto M, Kyrle P A, Yoshioka A, Ruggeri Z M
Abstract excerpt
von Willebrand factor (vWF) supports platelet adhesion on thrombogenic surfaces by binding to platelet membrane glycoprotein (GP) Ib in the GP Ib-IX receptor complex. This interaction is physiologically regulated so that it does not occur between circulating vWF and platelets but, rather, only at a site of vascular injury. The abnormal vWF found in type IIB von Willebrand disease, however, has a...
Topics
- Cloning, Molecular
- Humans
- Kinetics
- Macromolecular Substances
- Male
- Models, Structural
- Mutation
- Platelet Membrane Glycoproteins
- Protein Conformation
- Recombinant Proteins