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Morato 2015 Cell Death Differ

From Bioblast
Publications in the MiPMap
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