The Intriguing Relationships of von Willebrand Factor, ADAMTS13 and Cardiac Disease
von Willebrand factor (VWF) is an adhesive protein involved in primary hemostasis and facilitates platelet adhesion to sites of vascular injury, thereby promoting thrombus formation. VWF exists in plasma as multimers of increasing size, with the largest (high molecular weight; HMW) expressing the gr...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-09-01
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| Series: | Journal of Cardiovascular Development and Disease |
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| Online Access: | https://www.mdpi.com/2308-3425/8/9/115 |
| _version_ | 1829092247313842176 |
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| author | Benjamin Reardon Leonardo Pasalic Emmanuel J. Favaloro |
| author_facet | Benjamin Reardon Leonardo Pasalic Emmanuel J. Favaloro |
| author_sort | Benjamin Reardon |
| collection | DOAJ |
| description | von Willebrand factor (VWF) is an adhesive protein involved in primary hemostasis and facilitates platelet adhesion to sites of vascular injury, thereby promoting thrombus formation. VWF exists in plasma as multimers of increasing size, with the largest (high molecular weight; HMW) expressing the greatest functional activity. A deficiency of VWF is associated with a bleeding disorder called von Willebrand disease (VWD), whereas an excess of VWF, in particular the HMW forms, is associated with thrombosis. ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif-13), also known as VWF-cleaving protease, functions to moderate the activity of VWF by cleaving multimers of VWF and limiting the expression of the largest multimers of VWF. A deficiency of ADAMTS13 is therefore associated with an excess of (HMW forms of) VWF, and thus thrombosis. Indeed, any disturbance of the VWF/ADAMTS13 ratio or ‘axis’ may be associated with pathophysiological processes, including prothrombotic tendency. However, both thrombosis or bleeding may be associated with such disturbances, depending on the presenting events. This review evaluates the relationship of VWF and ADAMTS13 with cardiac disease, including cardiac failure, and associated pathophysiology. |
| first_indexed | 2024-03-10T07:33:35Z |
| format | Article |
| id | doaj.art-2c010d86da99483ca54175bc8516c01b |
| institution | Directory Open Access Journal |
| issn | 2308-3425 |
| language | English |
| last_indexed | 2024-03-10T07:33:35Z |
| publishDate | 2021-09-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Cardiovascular Development and Disease |
| spelling | doaj.art-2c010d86da99483ca54175bc8516c01b2023-11-22T13:38:24ZengMDPI AGJournal of Cardiovascular Development and Disease2308-34252021-09-018911510.3390/jcdd8090115The Intriguing Relationships of von Willebrand Factor, ADAMTS13 and Cardiac DiseaseBenjamin Reardon0Leonardo Pasalic1Emmanuel J. Favaloro2Institute of Clinical Pathology and Medical Research (ICPMR), Westmead Hospital, Westmead, NSW 2145, AustraliaInstitute of Clinical Pathology and Medical Research (ICPMR), Westmead Hospital, Westmead, NSW 2145, AustraliaInstitute of Clinical Pathology and Medical Research (ICPMR), Westmead Hospital, Westmead, NSW 2145, Australiavon Willebrand factor (VWF) is an adhesive protein involved in primary hemostasis and facilitates platelet adhesion to sites of vascular injury, thereby promoting thrombus formation. VWF exists in plasma as multimers of increasing size, with the largest (high molecular weight; HMW) expressing the greatest functional activity. A deficiency of VWF is associated with a bleeding disorder called von Willebrand disease (VWD), whereas an excess of VWF, in particular the HMW forms, is associated with thrombosis. ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif-13), also known as VWF-cleaving protease, functions to moderate the activity of VWF by cleaving multimers of VWF and limiting the expression of the largest multimers of VWF. A deficiency of ADAMTS13 is therefore associated with an excess of (HMW forms of) VWF, and thus thrombosis. Indeed, any disturbance of the VWF/ADAMTS13 ratio or ‘axis’ may be associated with pathophysiological processes, including prothrombotic tendency. However, both thrombosis or bleeding may be associated with such disturbances, depending on the presenting events. This review evaluates the relationship of VWF and ADAMTS13 with cardiac disease, including cardiac failure, and associated pathophysiology.https://www.mdpi.com/2308-3425/8/9/115von Willebrand factorendotheliumcardiac failureADAMTS13 |
| spellingShingle | Benjamin Reardon Leonardo Pasalic Emmanuel J. Favaloro The Intriguing Relationships of von Willebrand Factor, ADAMTS13 and Cardiac Disease Journal of Cardiovascular Development and Disease von Willebrand factor endothelium cardiac failure ADAMTS13 |
| title | The Intriguing Relationships of von Willebrand Factor, ADAMTS13 and Cardiac Disease |
| title_full | The Intriguing Relationships of von Willebrand Factor, ADAMTS13 and Cardiac Disease |
| title_fullStr | The Intriguing Relationships of von Willebrand Factor, ADAMTS13 and Cardiac Disease |
| title_full_unstemmed | The Intriguing Relationships of von Willebrand Factor, ADAMTS13 and Cardiac Disease |
| title_short | The Intriguing Relationships of von Willebrand Factor, ADAMTS13 and Cardiac Disease |
| title_sort | intriguing relationships of von willebrand factor adamts13 and cardiac disease |
| topic | von Willebrand factor endothelium cardiac failure ADAMTS13 |
| url | https://www.mdpi.com/2308-3425/8/9/115 |
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