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http://hdl.handle.net/2445/154941
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DC Field | Value | Language |
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dc.contributor.author | López Erauskin, Jone | - |
dc.contributor.author | Ferrer, Isidro (Ferrer Abizanda) | - |
dc.contributor.author | Galea, Elena | - |
dc.contributor.author | Pujol Onofre, Aurora | - |
dc.date.accessioned | 2020-04-03T10:49:44Z | - |
dc.date.available | 2020-04-03T10:49:44Z | - |
dc.date.issued | 2013-05-01 | - |
dc.identifier.issn | 0021-9258 | - |
dc.identifier.uri | http://hdl.handle.net/2445/154941 | - |
dc.description.abstract | Abstract: X-linked adrenoleukodystrophy (X-ALD) is a severe inherited neurodegenerative disorder characterized by adrenal insufficiency and graded damage in the nervous system. Loss of function of the peroxisomal ABCD1 fatty-acid transporter, resulting in the accumulation of very long-chain fatty acids in organs and plasma, is the genetic cause. Treatment with a combination of antioxidants halts the axonal degeneration and locomotor impairment displayed by the animal model of X-ALD, and is a proof of concept that oxidative stress contributes to axonal damage. New evidence demonstrates that metabolic failure and the opening of the mitochondrial permeability transition pore orchestrated by cyclophilin D underlies oxidative stress-induced axonal degeneration. Thus, cyclophilin D could serve as a therapeutic target for the treatment of X-ALD and cyclophilin D-dependent neurodegenerative and non-neurodegenerative diseases. | - |
dc.format.extent | 9 p. | - |
dc.format.mimetype | application/pdf | - |
dc.language.iso | eng | - |
dc.publisher | American Society for Biochemistry and Molecular Biology | - |
dc.relation.isformatof | Reproducció del document publicat a: https://doi.org/10.1515/hsz-2012-0323 | - |
dc.relation.ispartof | Journal of Biological Chemistry, 2013, vol. 394, num. 5, p. 621-629 | - |
dc.relation.uri | https://doi.org/10.1515/hsz-2012-0323 | - |
dc.rights | (c) American Society for Biochemistry and Molecular Biology, 2013 | - |
dc.source | Articles publicats en revistes (Patologia i Terapèutica Experimental) | - |
dc.subject.classification | Mitocondris | - |
dc.subject.classification | Estrès oxidatiu | - |
dc.subject.classification | Malalties neurodegeneratives | - |
dc.subject.classification | Prescripció de medicaments | - |
dc.subject.other | Mitochondria | - |
dc.subject.other | Oxidative stress | - |
dc.subject.other | Neurodegenerative Diseases | - |
dc.subject.other | Drug prescribing | - |
dc.title | Cyclophilin D as a potential target for antioxidants in neurodegeneration: the X-ALD case | - |
dc.type | info:eu-repo/semantics/article | - |
dc.type | info:eu-repo/semantics/publishedVersion | - |
dc.identifier.idgrec | 647602 | - |
dc.date.updated | 2020-04-03T10:49:44Z | - |
dc.relation.projectID | info:eu-repo/grantAgreement/EC/FP7/241622/EU//LEUKOTREAT | - |
dc.rights.accessRights | info:eu-repo/semantics/openAccess | - |
dc.identifier.pmid | 23492556 | - |
Appears in Collections: | Articles publicats en revistes (Patologia i Terapèutica Experimental) Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) |
Files in This Item:
File | Description | Size | Format | |
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647602.pdf | 735.25 kB | Adobe PDF | View/Open |
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