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Título: | Extracellular Tuning of Mitochondrial Respiration Leads to Aortic Aneurysm |
Autor: | Oller, Jorge; Gabandé-Roldríguez, Enrique; Ruíz-Rodríguez, María J.; Desdín-Mico, Gabriela; Aranda, Juan Francisco CSIC ORCID; Rodriguez-Díez, Raquel; Ballesteros-Martínez, Constanza; Blanco, Eva M.; Roldan-Montero, Raquel; Acuña, Pedro; Forteza Gil, Alberto; Martín-López, Carlos E.; Nistal, J. Francisco; Lino Cardenas, Christian L; Lindsay, Mark Evan; Martín-Ventura, Jose Luis; Briones, Ana M.; Miguel Redondo, Juan; Mittelbrunn, María CSIC ORCID CVN | Fecha de publicación: | 21-may-2021 | Editor: | American Heart Association | Citación: | Circulation 143: 2091- 2109 (2021) | Resumen: | Marfan syndrome (MFS) is an autosomal dominant disorder of the connective tissue caused by mutations in the FBN1 (fibrillin-1) gene encoding a large glycoprotein in the extracellular matrix called fibrillin-1. The major complication of this connective disorder is the risk to develop thoracic aortic aneurysm. To date, no effective pharmacologic therapies have been identified for the management of thoracic aortic disease and the only options capable of preventing aneurysm rupture are endovascular repair or open surgery. Here, we have studied the role of mitochondrial dysfunction in the progression of thoracic aortic aneurysm and mitochondrial boosting strategies as a potential treatment to managing aortic aneurysms. | Versión del editor: | http://dx.doi.org/10.1161/CIRCULATIONAHA.120.051171 | URI: | http://hdl.handle.net/10261/264867 | DOI: | 10.1161/CIRCULATIONAHA.120.051171 | Identificadores: | doi: 10.1161/CIRCULATIONAHA.120.051171 issn: 1524-4539 |
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MittelbrunnMExtracellularTuning.pdf | 4,21 MB | Adobe PDF | Visualizar/Abrir |
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