ADAMTS proteases in cardiovascular physiology and disease
The a disintegrin-like and metalloproteinase with thrombospondin motif (ADAMTS) family comprises 19 proteases that regulate the structure and function of extracellular proteins in the extracellular matrix and blood. The best characterized cardiovascular role is that of ADAMTS-13 in blood. Moderately...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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The Royal Society
2020-12-01
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| Series: | Open Biology |
| Subjects: | |
| Online Access: | https://royalsocietypublishing.org/doi/pdf/10.1098/rsob.200333 |
| _version_ | 1818449880608145408 |
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| author | Salvatore Santamaria Rens de Groot |
| author_facet | Salvatore Santamaria Rens de Groot |
| author_sort | Salvatore Santamaria |
| collection | DOAJ |
| description | The a disintegrin-like and metalloproteinase with thrombospondin motif (ADAMTS) family comprises 19 proteases that regulate the structure and function of extracellular proteins in the extracellular matrix and blood. The best characterized cardiovascular role is that of ADAMTS-13 in blood. Moderately low ADAMTS-13 levels increase the risk of ischeamic stroke and very low levels (less than 10%) can cause thrombotic thrombocytopenic purpura (TTP). Recombinant ADAMTS-13 is currently in clinical trials for treatment of TTP. Recently, new cardiovascular roles for ADAMTS proteases have been discovered. Several ADAMTS family members are important in the development of blood vessels and the heart, especially the valves. A number of studies have also investigated the potential role of ADAMTS-1, -4 and -5 in cardiovascular disease. They cleave proteoglycans such as versican, which represent major structural components of the arteries. ADAMTS-7 and -8 are attracting considerable interest owing to their implication in atherosclerosis and pulmonary arterial hypertension, respectively. Mutations in the ADAMTS19 gene cause progressive heart valve disease and missense variants in ADAMTS6 are associated with cardiac conduction. In this review, we discuss in detail the evidence for these and other cardiovascular roles of ADAMTS family members, their proteolytic substrates and the potential molecular mechanisms involved. |
| first_indexed | 2024-12-14T20:42:26Z |
| format | Article |
| id | doaj.art-275b912dc7714f73b080e8eee7820439 |
| institution | Directory Open Access Journal |
| issn | 2046-2441 |
| language | English |
| last_indexed | 2024-12-14T20:42:26Z |
| publishDate | 2020-12-01 |
| publisher | The Royal Society |
| record_format | Article |
| series | Open Biology |
| spelling | doaj.art-275b912dc7714f73b080e8eee78204392022-12-21T22:48:13ZengThe Royal SocietyOpen Biology2046-24412020-12-01101210.1098/rsob.200333200333ADAMTS proteases in cardiovascular physiology and diseaseSalvatore SantamariaRens de GrootThe a disintegrin-like and metalloproteinase with thrombospondin motif (ADAMTS) family comprises 19 proteases that regulate the structure and function of extracellular proteins in the extracellular matrix and blood. The best characterized cardiovascular role is that of ADAMTS-13 in blood. Moderately low ADAMTS-13 levels increase the risk of ischeamic stroke and very low levels (less than 10%) can cause thrombotic thrombocytopenic purpura (TTP). Recombinant ADAMTS-13 is currently in clinical trials for treatment of TTP. Recently, new cardiovascular roles for ADAMTS proteases have been discovered. Several ADAMTS family members are important in the development of blood vessels and the heart, especially the valves. A number of studies have also investigated the potential role of ADAMTS-1, -4 and -5 in cardiovascular disease. They cleave proteoglycans such as versican, which represent major structural components of the arteries. ADAMTS-7 and -8 are attracting considerable interest owing to their implication in atherosclerosis and pulmonary arterial hypertension, respectively. Mutations in the ADAMTS19 gene cause progressive heart valve disease and missense variants in ADAMTS6 are associated with cardiac conduction. In this review, we discuss in detail the evidence for these and other cardiovascular roles of ADAMTS family members, their proteolytic substrates and the potential molecular mechanisms involved.https://royalsocietypublishing.org/doi/pdf/10.1098/rsob.200333adamtsproteoglycansatherosclerosisaortic aneurysmsheart valvecardiovascular |
| spellingShingle | Salvatore Santamaria Rens de Groot ADAMTS proteases in cardiovascular physiology and disease Open Biology adamts proteoglycans atherosclerosis aortic aneurysms heart valve cardiovascular |
| title | ADAMTS proteases in cardiovascular physiology and disease |
| title_full | ADAMTS proteases in cardiovascular physiology and disease |
| title_fullStr | ADAMTS proteases in cardiovascular physiology and disease |
| title_full_unstemmed | ADAMTS proteases in cardiovascular physiology and disease |
| title_short | ADAMTS proteases in cardiovascular physiology and disease |
| title_sort | adamts proteases in cardiovascular physiology and disease |
| topic | adamts proteoglycans atherosclerosis aortic aneurysms heart valve cardiovascular |
| url | https://royalsocietypublishing.org/doi/pdf/10.1098/rsob.200333 |
| work_keys_str_mv | AT salvatoresantamaria adamtsproteasesincardiovascularphysiologyanddisease AT rensdegroot adamtsproteasesincardiovascularphysiologyanddisease |