Afficher la couverture 'Ethambutol-induced optic neuropathy linked to OPA1 mutation and mitochondrial toxicity | Guillet, Virginie' en grand format
/5

0 avis

Ethambutol-induced optic neuropathy linked to OPA1 mutation and mitochondrial toxicity

Archive ouverte

Guillet, Virginie | Chevrollier, Arnaud | Cassereau, Julien | Letournel, Franck | Gueguen, Naïg | Richard, Laurence | Desquiret-Dumas, Valérie | Verny, Christophe | Procaccio, Vincent | Amati-Bonneau, Patrizia | Reynier, Pascal | Bonneau, Dominique

Edité par CCSD ; Elsevier -

International audience.

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.

Consulter en ligne

Suggestions

Du même auteur

Idebenone increases mitochondrial complex I activity in fibroblasts from LHON patients while producing contradictory effects on respiration.

Archive ouverte | Angebault, Claire | CCSD

International audience. UNLABELLED: ABSTRACT: BACKGROUND: Leber's hereditary optic neuropathy (LHON) is caused by mutations in the complex I subunits of the respiratory chain. Although patients have been treated wit...

Mitochondrial complex I deficiency in GDAP1-related autosomal dominant Charcot-Marie-Tooth disease (CMT2K)

Archive ouverte | Cassereau, Julien | CCSD

International audience. Mutations in GDAP1, an outer mitochondrial membrane protein responsible for recessive Charcot-Marie-Tooth disease (CMT4A), have also been associated with CMT2K, a dominant form of the disease...

Hereditary spastic paraplegia-like disorder due to a mitochondrial ATP6 gene point mutation

Archive ouverte | Verny, Christophe | CCSD

International audience. Hereditary spastic paraplegia refers to a genetically heterogeneous syndrome. We identified five members of a family suffering from a late-onset spastic paraplegia-like disorder, carrying the...

Chargement des enrichissements...