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Activation of sirtuin 1 as therapy for the peroxisomal disease adrenoleukodystrophy #MMPMID25822341
Morató L; Ruiz M; Boada J; Calingasan NY; Galino J; Guilera C; Jové M; Naudí A; Ferrer I; Pamplona R; Serrano M; Portero-Otín M; Beal MF; Fourcade S; Pujol A
Cell Death Differ 2015[Nov]; 22 (11): 1742-53 PMID25822341show ga
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.