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Fibril-associated Collagen01:11

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Fibril-associated collagens are a type of collagens present in the extracellular matrix with interrupted triple helices or FACIT (Fibril-associated collagens interrupted triple-helices). FACIT help connect and attach the collagen fibrils with each other as well as with other proteins of the extracellular matrix.
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Investigating von Willebrand Factor Pathophysiology Using a Flow Chamber Model of von Willebrand Factor-platelet String Formation
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Laboratory Testing for von Willebrand Factor Collagen Binding (VWF:CB).

Emmanuel J Favaloro1, Soma Mohammed2,3

  • 1Haematology Department, Sydney Centres for Thrombosis and Haemostasis, Institute of Clinical Pathology and Medical Research (ICPMR), Westmead Hospital, NSW Health Pathology, Westmead, NSW, 2145, Australia. emmanuel.favaloro@health.nsw.gov.au.

Methods in Molecular Biology (Clifton, N.J.)
|August 15, 2017
PubMed
Summary

Von Willebrand disease (VWD) and acquired VWD (AVWS) stem from von Willebrand factor (VWF) issues. This chapter details VWF collagen binding (VWF:CB) assays, like ELISA and CLIA, to assess VWF activity for accurate diagnosis.

Keywords:
VWDVWFVWF:CBvon Willebrand diseasevon Willebrand factorvon Willebrand factor collagen binding

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Area of Science:

  • Hematology
  • Clinical Chemistry
  • Molecular Biology

Background:

  • Von Willebrand disease (VWD) is the most prevalent inherited bleeding disorder, with acquired forms (AVWS) also recognized.
  • VWD and AVWS result from deficiencies or defects in von Willebrand factor (VWF), a critical plasma protein.
  • Diagnosing VWF-related disorders necessitates evaluating both VWF levels and VWF activity due to its multifaceted hemostatic functions.

Purpose of the Study:

  • To describe protocols for assessing VWF activity through VWF collagen binding (VWF:CB) assays.
  • To provide methodologies for quantifying VWF's adhesive function to collagen.
  • To detail common VWF:CB assay formats, including ELISA and CLIA.

Main Methods:

  • VWF:CB assays measure VWF's ability to adhere to collagen or collagen peptides.
  • Quantitative assessment of VWF adhesion is followed by immunological detection.
  • Enzyme-linked immunosorbent assays (ELISA) and chemiluminescent assays (CLIA) are commonly employed VWF:CB methods.

Main Results:

  • The described protocols enable quantitative assessment of VWF's collagen-binding activity.
  • ELISA and CLIA are presented as standard methods for VWF:CB assessment.
  • These assays facilitate the evaluation of VWF's functional capacity in hemostasis.

Conclusions:

  • VWF:CB assays are essential for evaluating VWF activity in VWD and AVWS.
  • Standardized protocols using ELISA and CLIA provide reliable methods for VWF:CB measurement.
  • Accurate assessment of VWF activity is crucial for the diagnosis and management of bleeding disorders.