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Cyclophilin D as a potential target for antioxidants in neurodegeneration: the X-ALD case
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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.
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LÓPEZ ERAUSKIN, Jone, FERRER, Isidro (ferrer abizanda), GALEA, Elena, PUJOL ONOFRE, Aurora. Cyclophilin D as a potential target for antioxidants in neurodegeneration: the X-ALD case. _Journal of Biological Chemistry_. 2013. Vol. 394, núm. 5, pàgs. 621-629. [consulta: 26 de febrer de 2026]. ISSN: 0021-9258. [Disponible a: https://hdl.handle.net/2445/154941]