Angiotensin, transforming growth factor β and aortic dilatation in Marfan syndrome: Of mice and humans
Marfan syndrome is consequent upon mutations in FBN1, which encodes the extracellular matrix microfibrillar protein fibrillin-1. The phenotype is characterised by development of thoracic aortic aneurysm. Current understanding of the pathogenesis of aneurysms in Marfan syndrome focuses upon abnormal...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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Elsevier
2018-03-01
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| Series: | International Journal of Cardiology: Heart & Vasculature |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2352906717300714 |
| _version_ | 1818192418098380800 |
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| author | Christopher Yu Richmond W. Jeremy |
| author_facet | Christopher Yu Richmond W. Jeremy |
| author_sort | Christopher Yu |
| collection | DOAJ |
| description | Marfan syndrome is consequent upon mutations in FBN1, which encodes the extracellular matrix microfibrillar protein fibrillin-1. The phenotype is characterised by development of thoracic aortic aneurysm. Current understanding of the pathogenesis of aneurysms in Marfan syndrome focuses upon abnormal vascular smooth muscle cell signalling through the transforming growth factor beta (TGFβ) pathway. Angiotensin II (Ang II) can directly induce aortic dilatation and also influence TGFβ synthesis and signalling. It has been hypothesised that antagonism of Ang II signalling may protect against aortic dilatation in Marfan syndrome. Experimental studies have been supportive of this hypothesis, however results from multiple clinical trials are conflicting. This paper examines current knowledge about the interactions of Ang II and TGFβ signalling in the vasculature, and critically interprets the experimental and clinical findings against these signalling interactions. Keywords: Aneurysm, Angiotensin blocker, Cell Signalling, Clinical trial |
| first_indexed | 2024-12-12T00:30:11Z |
| format | Article |
| id | doaj.art-2520899b212649819c40281e22d2b4fc |
| institution | Directory Open Access Journal |
| issn | 2352-9067 |
| language | English |
| last_indexed | 2024-12-12T00:30:11Z |
| publishDate | 2018-03-01 |
| publisher | Elsevier |
| record_format | Article |
| series | International Journal of Cardiology: Heart & Vasculature |
| spelling | doaj.art-2520899b212649819c40281e22d2b4fc2022-12-22T00:44:30ZengElsevierInternational Journal of Cardiology: Heart & Vasculature2352-90672018-03-01187180Angiotensin, transforming growth factor β and aortic dilatation in Marfan syndrome: Of mice and humansChristopher Yu0Richmond W. Jeremy1Sydney Medical School, University of Sydney, Sydney 2006, AustraliaCorresponding author at: Suite 407, 100 Carillon Avenue, Newtown, NSW 2042, Australia.; Sydney Medical School, University of Sydney, Sydney 2006, AustraliaMarfan syndrome is consequent upon mutations in FBN1, which encodes the extracellular matrix microfibrillar protein fibrillin-1. The phenotype is characterised by development of thoracic aortic aneurysm. Current understanding of the pathogenesis of aneurysms in Marfan syndrome focuses upon abnormal vascular smooth muscle cell signalling through the transforming growth factor beta (TGFβ) pathway. Angiotensin II (Ang II) can directly induce aortic dilatation and also influence TGFβ synthesis and signalling. It has been hypothesised that antagonism of Ang II signalling may protect against aortic dilatation in Marfan syndrome. Experimental studies have been supportive of this hypothesis, however results from multiple clinical trials are conflicting. This paper examines current knowledge about the interactions of Ang II and TGFβ signalling in the vasculature, and critically interprets the experimental and clinical findings against these signalling interactions. Keywords: Aneurysm, Angiotensin blocker, Cell Signalling, Clinical trialhttp://www.sciencedirect.com/science/article/pii/S2352906717300714 |
| spellingShingle | Christopher Yu Richmond W. Jeremy Angiotensin, transforming growth factor β and aortic dilatation in Marfan syndrome: Of mice and humans International Journal of Cardiology: Heart & Vasculature |
| title | Angiotensin, transforming growth factor β and aortic dilatation in Marfan syndrome: Of mice and humans |
| title_full | Angiotensin, transforming growth factor β and aortic dilatation in Marfan syndrome: Of mice and humans |
| title_fullStr | Angiotensin, transforming growth factor β and aortic dilatation in Marfan syndrome: Of mice and humans |
| title_full_unstemmed | Angiotensin, transforming growth factor β and aortic dilatation in Marfan syndrome: Of mice and humans |
| title_short | Angiotensin, transforming growth factor β and aortic dilatation in Marfan syndrome: Of mice and humans |
| title_sort | angiotensin transforming growth factor β and aortic dilatation in marfan syndrome of mice and humans |
| url | http://www.sciencedirect.com/science/article/pii/S2352906717300714 |
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