| Article ID | Journal | Published Year | Pages | File Type |
|---|---|---|---|---|
| 8261388 | Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease | 2012 | 11 Pages |
Abstract
⺠Heterozygous OPA1 mutations are associated with defective mitochondrial fusion. ⺠Mitochondrial morphology may be elongated despite defective mitochondrial fusion. ⺠Mutant OPA1 cells have altered mitochondrial dynamic response to nutritional stress. ⺠Mitochondrial activities are significantly altered in mutant OPA1 cells. ⺠Physical link between OPA1 and OXPHOS proteins is widespread.
Keywords
SOD1ADOATMRMSOD2CCCPOXPHOSVO2Cu,Zn-superoxide dismutaseMitochondrial DNAΔψautosomal dominant optic atrophyMitochondrial diseasemtDNAOxidative phosphorylationEnergy metabolismtetramethylrhodamine methyl esterMn-Superoxide dismutaseOxygen consumption rateAspect ratioMitochondrial fusiontert-butyl hydroperoxideMitochondrial inner membrane potentialcarbonyl cyanide m-chlorophenyl hydrazone
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Authors
Virginie Agier, Patricia Oliviero, Jeanne Lainé, Caroline L'Hermitte-Stead, Samantha Girard, Sandrine Fillaut, Claude Jardel, Frédéric Bouillaud, Anne Laure Bulteau, Anne Lombès,