Activation of sirtuin 1 as therapy for the peroxisomal disease adrenoleukodystrophy
Fecha de publicación
2015Autor/a
Morató, Laia
Ruiz, M.
Calingasan, Noel Ylagan
Galino, Jorge
Guilera, Cristina
Ferrer, Isidre
Serrano, M.
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.
Metadatos
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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.