Vascular Smooth Muscle Cell Phenotypic Changes in Patients With Marfan Syndrome | Zendy

Eva CrosasMolist | Zendy; Thayna Meirelles | Zendy; Judit LópezLuque | Zendy; Carla SerraPeinado | Zendy; Javier Selva | Zendy; Laia Caja | Zendy; Darya Gorbenko del Blanco | Zendy; Juan J. Uriarte | Zendy; Esther Bertrán | Zendy; Yolanda Mendizábal | Zendy; Vanessa Hernández | Zendy; Carolina García-Calero | Zendy; Óscar Busnadiego | Zendy; Enric Condom | Zendy; David Toral | Zendy; Manuel Castellá | Zendy; Alberto Forteza | Zendy; Daniel Navajas | Zendy; Elisabet Sarri | Zendy; Fernando RodríguezPascual | Zendy; Harry C. Dietz | Zendy; Isabel Fabregat | Zendy; Gustavo Egea | Zendy

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Vascular Smooth Muscle Cell Phenotypic Changes in Patients With Marfan Syndrome

Author(s) -

Eva CrosasMolist,

Thayna Meirelles,

Judit LópezLuque,

Carla SerraPeinado,

Javier Selva,

Laia Caja,

Darya Gorbenko del Blanco,

Juan J. Uriarte,

Esther Bertrán,

Yolanda Mendizábal,

Vanessa Hernández,

Carolina García-Calero,

Óscar Busnadiego,

Enric Condom,

David Toral,

Manuel Castellá,

Alberto Forteza,

Daniel Navajas,

Elisabet Sarri,

Fernando RodríguezPascual,

Harry C. Dietz,

Isabel Fabregat,

Gustavo Egea

Publication year - 2015

Publication title -

arteriosclerosis thrombosis and vascular biology

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 3.007

H-Index - 270

eISSN - 1524-4636

pISSN - 1079-5642

DOI - 10.1161/atvbaha.114.304412

Subject(s) - myocardin , versican , extracellular matrix , vascular smooth muscle , marfan syndrome , calponin , biology , serum response factor , microbiology and biotechnology , fibrillin , transforming growth factor , myosin , actin , medicine , pathology , endocrinology , transcription factor , proteoglycan , biochemistry , smooth muscle , gene

Marfan's syndrome is characterized by the formation of ascending aortic aneurysms resulting from altered assembly of extracellular matrix microfibrils and chronic tissue growth factor (TGF)-β signaling. TGF-β is a potent regulator of the vascular smooth muscle cell (VSMC) phenotype. We hypothesized that as a result of the chronic TGF-β signaling, VSMC would alter their basal differentiation phenotype, which could facilitate the formation of aneurysms. This study explores whether Marfan's syndrome entails phenotypic alterations of VSMC and possible mechanisms at the subcellular level.

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