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Investigating von Willebrand Factor Pathophysiology Using a Flow Chamber Model of von Willebrand Factor-platelet String Formation
Published on: August 14, 2017
Multimeric analysis of von Willebrand factor
1Molecular Haemostasis Laboratory, Department of Haematology, Laboratory Medicine Block, The Birmingham Children's Hospital NHS Trust, Birmingham, West Midlands, UK.
Abstract:
Von Willebrand Factor (vWF) in normal plasma is composed of a series of high molecular multimers, ranging in size from 8×10(5) to over 15×10(6) Daltons (1). The multimeric structure of vWF was first investigated by two-dimensional crossed immunoelectrophoresis (2D-IEP). In 1974, Kernoff et al. (2) used this method, and since then multimer sizing of von Willebrand Factor Antigen (vWF∶Ag) in this form and other variations have been used in the diagnosis and classification of von Willebrand's disease (vWD).
Insights
Von Willebrand Factor (vWF) exists as high molecular multimers in plasma. Multimer sizing using methods like two-dimensional crossed immunoelectrophoresis is crucial for diagnosing and classifying von Willebrand
Area of Science:
- Biochemistry
- Hematology
- Immunology
Background:
- Normal plasma contains Von Willebrand Factor (vWF) as high molecular multimers.
- These multimers range in size from 8x10^5 to over 15x10^6 Daltons.
Purpose of the Study:
- To highlight the historical significance of multimer sizing in understanding vWF structure.
- To underscore the role of vWF multimer analysis in diagnosing von Willebrand's disease (vWD).
Main Methods:
- Two-dimensional crossed immunoelectrophoresis (2D-IEP) was an early method for vWF multimer investigation.
- Variations of multimer sizing techniques are employed.
Main Results:
- The multimeric structure of vWF has been characterized.
- Multimer sizing has been established as a diagnostic tool.
Conclusions:
- The analysis of vWF multimer size is fundamental in the diagnosis and classification of vWD.
- Historical methods like 2D-IEP laid the groundwork for current diagnostic approaches.

