Morato 2015 Cell Death Differ

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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 (2015) Activation of sirtuin 1 as therapy for the peroxisomal disease adrenoleukodystrophy. Cell Death Differ 22:1742-53.

» PMID: 25822341 Open Access

Morato L, Ruiz M, Boada J, Calingasan NY, Galino J, Guilera C, Jove M, Naudi A, Ferrer I, Pamplona R, Serrano M, Portero-Otin M, Beal MF, Fourcade S, Pujol A (2015) Cell Death Differ

Abstract: 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.


Bioblast editor: Kandolf G O2k-Network Lab: CH Lausanne Sandi C, ES Lleida Boada J


Labels: MiParea: Respiration, mt-Medicine, Pharmacology;toxicology  Pathology: Inherited, Neurodegenerative 

Organism: Mouse  Tissue;cell: Nervous system  Preparation: Permeabilized tissue 


Coupling state: LEAK, OXPHOS  Pathway: N, S, NS, ROX  HRR: Oxygraph-2k 

Labels, 2018-01, Resveratrol