Abstract
Von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder and can also arise as an acquired syndrome (AVWS). These disorders develop due to defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for the VWF-related disorders requires assessment of both VWF level and VWF activity, the latter requiring multiple assays because of the many functions carried out by VWF to help prevent bleeding. As an additional step, an evaluation of VWF structural features by multimer analysis is useful in selective investigations. The current paper therefore describes a protocol for assessment of VWF multimers by gel electrophoresis, thus enabling identification of protein bands that represent differently sized multimers. The sample protocol described in this chapter is the methodology developed by Sebia.
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Abbreviations
- HMW:
-
High molecular weight (VWF)
- IMW:
-
Intermediate molecular weight (VWF)
- LMW:
-
Low molecular weight (VWF)
- PT:
-
Platelet type (VWD)
- TTP:
-
Thrombotic thrombocytopenia purpura
- VWF:
-
von Willebrand factor
- VWF:Ag:
-
von Willebrand factor antigen
- VWF:CB:
-
Collagen binding assay
- VWF:RCo:
-
Ristocetin cofactor assay
- VWF:GPIb:
-
von Willebrand factor activity assay based on the binding of VWF to GPIb
- VWD:
-
von Willebrand disease
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Oliver, S., Lau, K.K.E., Chapman, K., Favaloro, E.J. (2017). Laboratory Testing for Von Willebrand Factor Multimers. In: Favaloro, E., Lippi, G. (eds) Hemostasis and Thrombosis. Methods in Molecular Biology, vol 1646. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-7196-1_36
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DOI: https://doi.org/10.1007/978-1-4939-7196-1_36
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Publisher Name: Humana Press, New York, NY
Print ISBN: 978-1-4939-7194-7
Online ISBN: 978-1-4939-7196-1
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