Difference between revisions of "Guillet 2010 Mitochondrion"
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{{Labeling | {{Labeling | ||
|area=Respiration, mt-Structure;fission;fusion, mtDNA;mt-genetics, Pharmacology;toxicology | |||
|organism=Human | |organism=Human | ||
|model cell lines=Fibroblast | |model cell lines=Fibroblast | ||
|preparations=Intact cells, Enzyme | |preparations=Intact cells, Enzyme | ||
|enzymes=Complex IV; Cytochrome c Oxidase | |enzymes=Complex IV; Cytochrome c Oxidase | ||
| | |diseases=Neurodegenerative | ||
|couplingstates=OXPHOS | |couplingstates=OXPHOS | ||
|instruments=Oxygraph-2k | |instruments=Oxygraph-2k | ||
Revision as of 23:52, 11 August 2013
| 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: 115-124. |
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. • Keywords: Ethambutol, OPA1, Autosomal dominant optic atrophy, Mitochondria
• O2k-Network Lab: FR_Angers_Douay O
Labels: MiParea: Respiration, mt-Structure;fission;fusion, mtDNA;mt-genetics, Pharmacology;toxicology
Pathology: Neurodegenerative
Organism: Human
Preparation: Intact cells, Enzyme Enzyme: Complex IV; Cytochrome c Oxidase"Complex IV; Cytochrome c Oxidase" is not in the list (Adenine nucleotide translocase, Complex I, Complex II;succinate dehydrogenase, Complex III, Complex IV;cytochrome c oxidase, Complex V;ATP synthase, Inner mt-membrane transporter, Marker enzyme, Supercomplex, TCA cycle and matrix dehydrogenases, ...) of allowed values for the "Enzyme" property.
Coupling state: OXPHOS
HRR: Oxygraph-2k
Abbreviations: EMB, ethambutol; ADOA, autosomal dominant optic atrophy; OPA1, optic atrophy 1; LHON, Leber’s hereditary optic neuropathy; OXPHOS, oxidative phosphorylation
