Difference between revisions of "Guillet 2010 Mitochondrion"
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|abstract=Ethambutol (EMB), widely used in the treatment of tuberculosis, has been reported to cause Leber's hereditary optic neuropathy in patients carrying mitochondrial DNA mutations. We study the effect of EMB on mitochondrial metabolism in fibroblasts from controls and from a man carrying an OPA1 mutation, in whom the drug induced the development of autosomal dominant optic atrophy (ADOA). EMB produced a mitochondrial coupling defect together with a 25% reduction in complex IV activity. EMB induced the formation of vacuoles associated with decreased mitochondrial membrane potential and increased fragmentation of the mitochondrial network. Mitochondrial genetic variations may therefore be predisposing factors in EMB-induced ocular injury. | |abstract=Ethambutol (EMB), widely used in the treatment of tuberculosis, has been reported to cause Leber's hereditary optic neuropathy in patients carrying mitochondrial DNA mutations. We study the effect of EMB on mitochondrial metabolism in fibroblasts from controls and from a man carrying an OPA1 mutation, in whom the drug induced the development of autosomal dominant optic atrophy (ADOA). EMB produced a mitochondrial coupling defect together with a 25% reduction in complex IV activity. EMB induced the formation of vacuoles associated with decreased mitochondrial membrane potential and increased fragmentation of the mitochondrial network. Mitochondrial genetic variations may therefore be predisposing factors in EMB-induced ocular injury. | ||
|keywords=Ethambutol, OPA1, Autosomal dominant optic atrophy, Mitochondria | |keywords=Ethambutol, OPA1, Autosomal dominant optic atrophy, Mitochondria | ||
|mipnetlab=FR Angers | |mipnetlab=FR Angers Gueguen N | ||
|discipline=Mitochondrial Physiology, Pharmacology; Biotechnology | |discipline=Mitochondrial Physiology, Pharmacology; Biotechnology | ||
}} | }} |
Latest revision as of 12:38, 16 February 2018
Guillet V, Chevrollier A, Cassereau J, Letournel F, Gueguen N, Richard L, Desquiret V, Verny C, Procaccio V, Amati-Bonneau P, Reynier P, Bonneau D (2010) Ethambutol-induced optic neuropathy linked to OPA1 mutation and mitochondrial toxicity. Mitochondrion 10:115-24. |
Guillet V, Chevrollier A, Cassereau J, Letournel F, Gueguen N, Richard L, Desquiret V, Verny C, Procaccio V, Amati-Bonneau P, Reynier P, Bonneau D (2010) Mitochondrion
Abstract: Ethambutol (EMB), widely used in the treatment of tuberculosis, has been reported to cause Leber's hereditary optic neuropathy in patients carrying mitochondrial DNA mutations. We study the effect of EMB on mitochondrial metabolism in fibroblasts from controls and from a man carrying an OPA1 mutation, in whom the drug induced the development of autosomal dominant optic atrophy (ADOA). EMB produced a mitochondrial coupling defect together with a 25% reduction in complex IV activity. EMB induced the formation of vacuoles associated with decreased mitochondrial membrane potential and increased fragmentation of the mitochondrial network. Mitochondrial genetic variations may therefore be predisposing factors in EMB-induced ocular injury. โข Keywords: Ethambutol, OPA1, Autosomal dominant optic atrophy, Mitochondria
โข O2k-Network Lab: FR Angers Gueguen N
Labels: MiParea: Respiration, mt-Structure;fission;fusion, mtDNA;mt-genetics, Pharmacology;toxicology
Pathology: Neurodegenerative
Organism: Human Tissue;cell: Fibroblast Preparation: Intact cells, Enzyme Enzyme: Complex IV;cytochrome c oxidase
Coupling state: OXPHOS
HRR: Oxygraph-2k
Abbreviations: EMB, ethambutol; ADOA, autosomal dominant optic atrophy; OPA1, optic atrophy 1; LHON, Leberโs hereditary optic neuropathy; OXPHOS, oxidative phosphorylation