| First Author | Morató L | Year | 2015 |
| Journal | Cell Death Differ | Volume | 22 |
| Issue | 11 | Pages | 1742-53 |
| PubMed ID | 25822341 | Mgi Jnum | J:259020 |
| Mgi Id | MGI:6140679 | Doi | 10.1038/cdd.2015.20 |
| Citation | Morato L, et al. (2015) Activation of sirtuin 1 as therapy for the peroxisomal disease adrenoleukodystrophy. Cell Death Differ 22(11):1742-53 |
| abstractText | 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. |
