| dc.contributor.author | Morató, Laia | |
| dc.contributor.author | Ruiz, M. | |
| dc.contributor.author | Boada Pallàs, Jordi | |
| dc.contributor.author | Calingasan, Noel Ylagan | |
| dc.contributor.author | Galino, Jorge | |
| dc.contributor.author | Guilera, Cristina | |
| dc.contributor.author | Jové Font, Mariona | |
| dc.contributor.author | Naudí i Farré, Alba | |
| dc.contributor.author | Ferrer, Isidre | |
| dc.contributor.author | Pamplona Gras, Reinald | |
| dc.contributor.author | Serrano, M. | |
| dc.contributor.author | Portero Otín, Manuel | |
| dc.contributor.author | Beal, M. F. | |
| dc.contributor.author | Fourcade, Stéphane | |
| dc.contributor.author | Pujol, Aurora | |
| dc.date.accessioned | 2016-11-09T10:14:00Z | |
| dc.date.issued | 2015 | |
| dc.identifier.issn | 1350-9047 | |
| dc.identifier.uri | http://hdl.handle.net/10459.1/58437 | |
| dc.description.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. | ca_ES |
| dc.description.sponsorship | This work was supported by grants from the European Commission [FP7-241622], the Spanish Institute for Health Carlos III [FIS PI11/01043], the Oliver’s Army Foundation, the Government of Catalonia [2009SGR85 to AP], the Spanish Institute for Health Carlos III [Miguel Servet program CP11/00080 to S.F.], the European Leukodystrophy Association (ELA) [ELA 2010-020F1 to SF, ELA 2013-003F1 to LM], the Spanish Ministry of Education [FPU program AP2008-03728 to LM]. JG held an IDIBELL PhD fellowship. The studies conducted at the Experimental Medicine Department were supported in part by R+D grants from the Spanish Ministry of Science and Innovation [BFU2009-11879/BFI], the Spanish Ministry of Health [PI081843, PI1300584], the Government of Catalonia [2009SGR735], the 'La Caixa' Foundation and COST B35 Action of the European Union. CIBERER and CIBERNED are initiatives of the Spanish Institute for Health Carlos III. | ca_ES |
| dc.language.iso | eng | ca_ES |
| dc.publisher | Macmillan Publishers | ca_ES |
| dc.relation | MICINN/PN2008-2011/BFU2009-11879/BFI | |
| dc.relation.isformatof | Reproducció del document publicat a https://doi.org/10.1038/cdd.2015.20 | ca_ES |
| dc.relation.ispartof | Cell Death and Differentiation, 2015, vol. 22, p. 1742-1753 | ca_ES |
| dc.rights | (c) Macmillan Publishers Limited, 2015 | ca_ES |
| dc.title | Activation of sirtuin 1 as therapy for the peroxisomal disease adrenoleukodystrophy | ca_ES |
| dc.type | article | ca_ES |
| dc.identifier.idgrec | 022612 | |
| dc.type.version | publishedVersion | ca_ES |
| dc.rights.accessRights | info:eu-repo/semantics/restrictedAccess | ca_ES |
| dc.identifier.doi | https://doi.org/10.1038/cdd.2015.20 | |
| dc.relation.projectID | info:eu-repo/grantAgreement/EC/FP7/241622 | |
| dc.date.embargoEndDate | 2025-01-01 | |
