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Pisani Funds"},{"name":"Fighting Aneurysmal Disease","award":["EU FP-7 200647"],"award-info":[{"award-number":["EU FP-7 200647"]}]},{"name":"NONAGE"},{"DOI":"10.13039\/501100001677","name":"Inserm","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100001677","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2018,3,15]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Vascular smooth muscle cells (vSMCs) play a crucial role in both the pathogenesis of Aneurysms and Dissections of the ascending thoracic aorta (TAAD) in humans and in the associated adaptive compensatory responses, since thrombosis and inflammatory processes are absent in the majority of cases. Aneurysms and dissections share numerous characteristics, including aetiologies and histopathological alterations: vSMC disappearance, medial areas of mucoid degeneration, and extracellular matrix (ECM) breakdown. Three aetiologies predominate in TAAD in humans: (i) genetic causes in heritable familial forms, (ii) an association with bicuspid aortic valves, and (iii) a sporadic degenerative form linked to the aortic aging process. Genetic forms include mutations in vSMC genes encoding for molecules of the ECM or the TGF-\u03b2 pathways, or participating in vSMC tone. On the other hand, aneurysms and dissections, whatever their aetiologies, are characterized by an increase in wall permeability leading to transmural advection of plasma proteins which could interact with vSMCs and ECM components. In this context, blood-borne plasminogen appears to play an important role, because its outward convection through the wall is increased in TAAD, and it could be converted to active plasmin at the vSMC membrane. Active plasmin can induce vSMC disappearance, proteolysis of adhesive proteins, activation of MMPs and release of TGF-\u03b2 from its ECM storage sites. Conversely, vSMCs could respond to aneurysmal biomechanical and proteolytic injury by an epigenetic phenotypic switch, including constitutional overexpression and nuclear translocation of Smad2 and an increase in antiprotease and ECM protein synthesis. In contrast, such an epigenetic phenomenon is not observed in dissections. In this context, dysfunction of proteins involved in vSMC tone are interesting to study, particularly in interaction with plasma protein transport through the wall and TGF-\u03b2 activation, to establish the relationship between these dysfunctions and ECM proteolysis.<\/jats:p>","DOI":"10.1093\/cvr\/cvy006","type":"journal-article","created":{"date-parts":[[2018,1,16]],"date-time":"2018-01-16T15:10:08Z","timestamp":1516115408000},"page":"578-589","source":"Crossref","is-referenced-by-count":188,"title":["From genetics to response to injury: vascular smooth muscle cells in aneurysms and dissections of the ascending aorta"],"prefix":"10.1093","volume":"114","author":[{"given":"Jean-Baptiste","family":"Michel","sequence":"first","affiliation":[{"name":"UMR 1148, Laboratory for Translational Vascular Science, Inserm and Paris 7- Denis Diderot University, Xavier Bichat Hospital, 75018 Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guillaume","family":"Jondeau","sequence":"additional","affiliation":[{"name":"UMR 1148, Laboratory for Translational Vascular Science, Inserm and Paris 7- Denis Diderot University, Xavier Bichat Hospital, 75018 Paris, France;"},{"name":"Cardiology Department, National Reference Center for Marfan Syndrome and Related Diseases, APHP Hopital Bichat, 75018 Paris;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dianna M","family":"Milewicz","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Internal Medicine, University of Texas Medical School at Houston, Houston, TX 77030, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2018,1,17]]},"reference":[{"key":"2019081408050401700_cvy006-B1","doi-asserted-by":"crossref","DOI":"10.1093\/med\/9780198755777.003.0016","volume-title":"Biology of Vascular Wall Dilation and Rupture","author":"Michel","year":"2017"},{"key":"2019081408050401700_cvy006-B2","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1093\/cvr\/cvs135","article-title":"The vascular smooth muscle cell in arterial pathology: a cell that can take on multiple roles","volume":"95","author":"Lacolley","year":"2012","journal-title":"Cardiovasc Res"},{"key":"2019081408050401700_cvy006-B3","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1093\/cvr\/cvq337","article-title":"Novel aspects of the pathogenesis of aneurysms of the abdominal aorta in humans","volume":"90","author":"Michel","year":"2011","journal-title":"Cardiovasc Res"},{"key":"2019081408050401700_cvy006-B4","doi-asserted-by":"crossref","first-page":"1206","DOI":"10.1136\/hrt.2010.212100","article-title":"The translational science of Marfan syndrome","volume":"97","author":"Jondeau","year":"2011","journal-title":"Heart"},{"key":"2019081408050401700_cvy006-B5","article-title":"Clearance of plasmin-PN-1 complexes by vascular smooth muscle cells in human aneurysm of the ascending aorta","author":"Boukais","year":"2017","journal-title":"Cardiovascular Pathology"},{"key":"2019081408050401700_cvy006-B6","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/S0002-9149(77)80004-0","article-title":"Histologic changes in the normal aging aorta: implications for dissecting aortic aneurysm","volume":"39","author":"Schlatmann","year":"1977","journal-title":"Am J Cardiol"},{"key":"2019081408050401700_cvy006-B7","first-page":"R124","article-title":"Effect of chronic ANG I-converting enzyme inhibition on aging processes. 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