López Erauskin, JoneFerrer, Isidro (Ferrer Abizanda)Galea, ElenaPujol Onofre, Aurora2020-04-032020-04-032013-05-010021-9258https://hdl.handle.net/2445/154941Abstract: 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.9 p.application/pdfeng(c) American Society for Biochemistry and Molecular Biology, 2013MitocondrisEstrès oxidatiuMalalties neurodegenerativesPrescripció de medicamentsMitochondriaOxidative stressNeurodegenerative DiseasesDrug prescribinginfo:eu-repo/semantics/article6476022020-04-03info:eu-repo/semantics/openAccess23492556