Von Willebrand Factor (VWF) is a multimeric plasma glycoprotein that mediates platelet adhesion and aggregation, a process critical for both hemostasis and thrombosis. Under normal conditions, VWF binds to platelets at sites of vascular injury or damage, leading to blood clot formation and wound healing. VWF contains four types of repeating domains in the following sequence: D1-D2-D’-D3-A1-A2-A3-D4-B1-B2-B3-C1-C2-CK (CK: cystine knot). It is synthesized and secreted into plasma by endothelial cells and megakaryocytes. Many newly-secreted VWF multimers are huge in size, thus they are termed ultra-large VWF (ULVWF). ULVWF is thrombogenic, so it is reduced to smaller VWF multimers by ADAMTS13, a metalloprotease that cleaves the Tyr1605-Met1606 bond in the A2 domain of VWF. Proper ULVWF cleavage and subsequent VWF cleavage result in appropriate size distribution of VWF in plasma, which is required for its hemostatic function. On the one hand, insufficient cleavage of ULVWF leads to thrombotic thrombocytopenic purpura (TTP), a disease characterized by microvascular thrombosis; on the other hand, excessive cleavage of VWF leads to Von Willebrand disease (VWD), a potentially-fatal bleeding disorder manifested by lack of large VWF multimers in plasma [1]. Therefore, understanding VWF cleavage by ADAMTS13 is crucial for understanding VWF function and its related diseases.
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ASME 2011 Summer Bioengineering Conference
June 22–25, 2011
Farmington, Pennsylvania, USA
Conference Sponsors:
- Bioengineering Division
ISBN:
978-0-7918-5458-7
PROCEEDINGS PAPER
Effect of ST2, a Fragment of ADAMTS13, on Cleavage of Von Willebrand Factor
Jin-Yu Shao,
Jin-Yu Shao
Washington University in Saint Louis, Saint Louis, MO
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Yingchen Ling,
Yingchen Ling
Washington University in Saint Louis, Saint Louis, MO
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J. Evan Sadler,
J. Evan Sadler
Washington University in Saint Louis, Saint Louis, MO
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Elaine M. Majerus
Elaine M. Majerus
Washington University in Saint Louis, Saint Louis, MO
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Jin-Yu Shao
Washington University in Saint Louis, Saint Louis, MO
Yingchen Ling
Washington University in Saint Louis, Saint Louis, MO
J. Evan Sadler
Washington University in Saint Louis, Saint Louis, MO
Elaine M. Majerus
Washington University in Saint Louis, Saint Louis, MO
Paper No:
SBC2011-53317, pp. 351-352; 2 pages
Published Online:
July 17, 2013
Citation
Shao, J, Ling, Y, Sadler, JE, & Majerus, EM. "Effect of ST2, a Fragment of ADAMTS13, on Cleavage of Von Willebrand Factor." Proceedings of the ASME 2011 Summer Bioengineering Conference. ASME 2011 Summer Bioengineering Conference, Parts A and B. Farmington, Pennsylvania, USA. June 22–25, 2011. pp. 351-352. ASME. https://doi.org/10.1115/SBC2011-53317
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