Activation of sirtuin 1 as therapy for the peroxisomal disease adrenoleukodystrophy

Thumbnail
Ver/Abrir
Fecha de publicación
2015
Autor/a
Morató, Laia
Ruiz, M.
Boada Pallàs, Jordi
Calingasan, Noel Ylagan
Galino, Jorge
Guilera, Cristina
Jové Font, Mariona
Naudí i Farré, Alba
Ferrer, Isidre
Pamplona Gras, Reinald
Serrano, M.
Portero Otín, Manuel
Beal, M. F.
Fourcade, Stéphane
Pujol, Aurora
Cita recomendada
Morató, Laia; Ruiz, M.; Boada Pallàs, Jordi; Calingasan, Noel Ylagan; Galino, Jorge; Guilera, Cristina; ... Pujol, Aurora. (2015) . Activation of sirtuin 1 as therapy for the peroxisomal disease adrenoleukodystrophy. Cell Death and Differentiation, 2015, vol. 22, p. 1742-1753. https://doi.org/10.1038/cdd.2015.20.
Impacto

Logo de Google Académico  Google Académico
Compartir
Exportar a Mendeley
Metadatos
Mostrar el registro completo del ítem
Resumen
Oxidative stress and mitochondrial failure are prominent factors in the axonal degeneration process. In this study, we demonstrate that sirtuin 1 (SIRT1), a key regulator of the mitochondrial function, is impaired in the axonopathy and peroxisomal disease X-linked adrenoleukodystrophy (X-ALD). We have
restored SIRT1 activity using a dual strategy of resveratrol treatment or by the moderate transgenic overexpression of SIRT1 in a X-ALD mouse model. Both strategies normalized redox homeostasis, mitochondrial respiration, bioenergetic failure, axonal degeneration and associated locomotor disabilities in the X-ALD mice. These results indicate that the reactivation of SIRT1 may be a valuable strategy to treat X-ALD and other axonopathies in which the control of redox and energetic homeostasis is impaired.
URI
http://hdl.handle.net/10459.1/58437
DOI
https://doi.org/10.1038/cdd.2015.20
Es parte de
Cell Death and Differentiation, 2015, vol. 22, p. 1742-1753
Colecciones