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Updated: May 7, 2026

Investigating von Willebrand Factor Pathophysiology Using a Flow Chamber Model of von Willebrand Factor-platelet String Formation
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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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Proteolytic processing of von Willebrand factor by adamts13 and leukocyte proteases

Stefano Lancellotti1, Maria Basso, Raimondo De Cristofaro

  • 1Institute of Internal Medicine and Geriatrics, Haemostasis and Thrombosis Center, Catholic University School of Medicine, ROMA - ITALY.

Mediterranean Journal of Hematology and Infectious Diseases
|October 10, 2013
PubMed

Insights

ADAMTS13 protease reduces von Willebrand factor (VWF) size, preventing platelet clumps that cause thrombotic microangiopathies (TMAs). This review explores ADAMTS13 structure, function, and its role in TMA pathogenesis.

Area of Science:

  • Biochemistry
  • Hematology
  • Molecular Biology

Background:

  • ADAMTS13 is a zinc protease that cleaves von Willebrand factor (VWF) multimers.
  • Dysfunctional ADAMTS13 activity is linked to thrombotic microangiopathies (TMAs).

Purpose of the Study:

  • To review the structure-function relationship of ADAMTS13.
  • To discuss ADAMTS13's role in TMA pathogenesis.
  • To explore VWF multimer processing by various proteases.

Main Methods:

  • Literature review of ADAMTS13 structure and function.
  • Analysis of ADAMTS13's role in thrombotic microangiopathy (TMA) pathogenesis.
  • Review of recent findings on VWF multimer processing.

Main Results:

  • ADAMTS13 cleaves VWF at Tyr1605-Met1606, reducing ultra-large VWF polymer size.
  • This cleavage is crucial for preventing platelet aggregation and TMA formation under shear stress.
  • Other proteases, including leukocyte-derived enzymes, also process VWF multimers.

Conclusions:

  • ADAMTS13's structure dictates its function in VWF regulation.
  • Understanding ADAMTS13 is key to managing TMAs.
  • Further research into VWF processing proteases is warranted.

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