Encyclopedia of Signaling Molecules

2018 Edition
| Editors: Sangdun Choi

von Willebrand Factor

  • Christian R. Robinson
  • Ina Laura Pieper
  • Venkateswarlu Kanamarlapudi
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-67199-4_101990

Synonyms

Historical Background

A hereditary bleeding disorder, which is now known as von Willebrand disease (VWD), was first discovered in 1924 by Dr. Erik von Willebrand, who noted that this disorder was different to hemophilia and exhibited prolonged bleeding time, normal clotting time, and was an autosomal inherited condition. It was further discovered that blood transfusions were able to prevent this bleeding condition, suggesting that there may be a plasma factor that is deficient within the circulation (Von Willebrand 1999). The reason for the prolonged bleeding time was first revealed to be an abnormality or lack of von Willebrand factor (vWF) (Soulier and Larrieu 1954). vWF is a large glycoprotein made up of numerous subunits, each with an approximate molecular weight of 220 kDa. These subunits undergo dimerization and multimerization to form larger vWF molecules consisting of a mature peptide with molecular weights ranging from 450...

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References

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Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  1. 1.Institute of Life Science 1School of Medicine, Swansea UniversitySwanseaUK
  2. 2.Calon Cardio-Technology Ltd, Institute of Life Science 2Medical School, Swansea UniversitySwanseaUK