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Título: | Cyclophilin D as a potential target for antioxidants in neurodegeneration: the X-ALD case |
Autor: | López Erauskin, Jone Ferrer, Isidro (Ferrer Abizanda) Galea, Elena Pujol Onofre, Aurora |
Materia: | Mitocondris Estrès oxidatiu Malalties neurodegeneratives Prescripció de medicaments Mitochondria Oxidative stress Neurodegenerative Diseases Drug prescribing |
Fecha de publicación: | 1-may-2013 |
Publicado por: | American Society for Biochemistry and Molecular Biology |
Resumen: | 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. |
Nota: | Reproducció del document publicat a: https://doi.org/10.1515/hsz-2012-0323 |
Es parte de: | Journal of Biological Chemistry, 2013, vol. 394, num. 5, p. 621-629 |
URI: | https://hdl.handle.net/2445/154941 |
Recurso relacionado: | https://doi.org/10.1515/hsz-2012-0323 |
ISSN: | 0021-9258 |
Aparece en las colecciones: | Articles publicats en revistes (Patologia i Terapèutica Experimental) Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) |
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