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2013-05-01

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Si us plau utilitzeu sempre aquest identificador per citar o enllaçar aquest document: https://hdl.handle.net/2445/154941

Cyclophilin D as a potential target for antioxidants in neurodegeneration: the X-ALD case

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https://doi.org/10.1515/hsz-2012-0323

Resum

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.

Matèries

Mitocondris, Estrès oxidatiu, Malalties neurodegeneratives, Prescripció de medicaments

Matèries (anglès)

Mitochondria, Oxidative stress, Neurodegenerative Diseases, Drug prescribing

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Articles publicats en revistes (Patologia i Terapèutica Experimental)
Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))

Citació

LÓPEZ ERAUSKIN, Jone, et al. Cyclophilin D as a potential target for antioxidants in neurodegeneration: the X-ALD case. Journal of Biological Chemistry. 2013. Vol. 394, num. 5, pags. 621-629. ISSN 0021-9258. [consulted: 9 of September of 2026]. Available at: https://hdl.handle.net/2445/154941

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