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http://hdl.handle.net/2445/154018
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DC Field | Value | Language |
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dc.contributor.author | Coppa, Andrea | - |
dc.contributor.author | Guha, Sanjib | - |
dc.contributor.author | Fourcade, Stéphane | - |
dc.contributor.author | Parameswaran, Janani | - |
dc.contributor.author | Ruiz, Montserrat | - |
dc.contributor.author | Moser, Ann B. | - |
dc.contributor.author | Schlüter, Agatha | - |
dc.contributor.author | Murphy, Michael P. | - |
dc.contributor.author | Lizcano, José Miguel | - |
dc.contributor.author | Miranda Vizuete, Antonio | - |
dc.contributor.author | Dalfó Capella, Esther | - |
dc.contributor.author | Pujol Onofre, Aurora | - |
dc.date.accessioned | 2020-03-26T09:42:45Z | - |
dc.date.available | 2021-02-01T06:10:15Z | - |
dc.date.issued | 2020-02-01 | - |
dc.identifier.uri | http://hdl.handle.net/2445/154018 | - |
dc.description.abstract | Adrenoleukodystrophy is a neurometabolic disorder caused by a defective peroxisomal ABCD1 transporter of very long-chain fatty acids (VLCFAs). Its pathogenesis is incompletely understood. Here we characterize a nematode model of X-ALD with loss of the pmp-4 gene, the worm orthologue of ABCD1. These mutants recapitulate the hallmarks of X-ALD: i) VLCFAs accumulation and impaired mitochondrial redox homeostasis and ii) axonal damage coupled to locomotor dysfunction. Furthermore, we identify a novel role for PMP-4 in modulating lipid droplet dynamics. Importantly, we show that the mitochondria targeted antioxidant MitoQ normalizes lipid droplets size, and prevents axonal degeneration and locomotor disability, highlighting its therapeutic potential. Moreover, PMP-4 acting solely in the hypodermis rescues axonal and locomotion abnormalities, suggesting a myelin-like role for the hypodermis in providing essential peroxisomal functions for the nematode nervous system. | ca |
dc.format.mimetype | application/pdf | - |
dc.language.iso | eng | ca |
dc.publisher | Elsevier BV | ca |
dc.relation.isformatof | Versió postprint del document publicat a: https://doi.org/10.1016/j.freeradbiomed.2020.01.177 | - |
dc.relation.ispartof | Free Radical Biology and Medicine, 2020 | - |
dc.relation.uri | https://doi.org/10.1016/j.freeradbiomed.2020.01.177 | - |
dc.rights | cc-by-nc-nd (c) Elsevier BV, 2020 | - |
dc.rights.uri | http://creativecommons.org/licenses/by-nc-nd/3.0/es/ | * |
dc.source | Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) | - |
dc.subject.classification | Trastorns del metabolisme | - |
dc.subject.classification | Peroxisomes | - |
dc.subject.other | Disorders of metabolism | - |
dc.subject.other | Peroxisomes | - |
dc.title | The peroxisomal fatty acid transporter ABCD1/PMP-4 is required in the C. elegans hypodermis for axonal maintenance: A worm model for adrenoleukodystrophy | ca |
dc.type | info:eu-repo/semantics/article | ca |
dc.date.updated | 2020-02-24T10:37:32Z | - |
dc.rights.accessRights | info:eu-repo/semantics/openAccess | - |
Appears in Collections: | Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) |
Files in This Item:
File | Description | Size | Format | |
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Coppa2020.pdf | 3.92 MB | Adobe PDF | View/Open |
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