Por favor, use este identificador para citar o enlazar este documento: https://hdl.handle.net/2445/154941
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))

Archivos de este documento:
Archivo Descripción DimensionesFormato 
647602.pdf735.25 kBAdobe PDFMostrar/Abrir


Este documento tiene todos los derechos reservados