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Genet."},{"key":"10.1016\/j.celrep.2022.111124_bib86","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1016\/j.mito.2017.01.004","article-title":"Evaluating the therapeutic potential of idebenone and related quinone analogues in Leber hereditary optic neuropathy","volume":"36","author":"Yu-Wai-Man","year":"2017","journal-title":"Mitochondrion"},{"key":"10.1016\/j.celrep.2022.111124_bib87","doi-asserted-by":"crossref","first-page":"4935","DOI":"10.1172\/JCI134965","article-title":"PRICKLE3 linked to ATPase biogenesis manifested Leber's hereditary optic neuropathy","volume":"130","author":"Yu","year":"2020","journal-title":"J.\u00a0Clin. Invest."},{"key":"10.1016\/j.celrep.2022.111124_bib88","doi-asserted-by":"crossref","first-page":"4029","DOI":"10.1038\/s41467-020-17821-1","article-title":"Inhibition of autophagy curtails visual loss in a model of autosomal dominant optic atrophy","volume":"11","author":"Zaninello","year":"2020","journal-title":"Nat. Commun."},{"key":"10.1016\/j.celrep.2022.111124_bib89","doi-asserted-by":"crossref","first-page":"997","DOI":"10.1007\/s10495-005-0742-5","article-title":"Caspase-independent death of Leber's hereditary optic neuropathy cybrids is driven by energetic failure and mediated by AIF and Endonuclease G","volume":"10","author":"Zanna","year":"2005","journal-title":"Apoptosis"},{"key":"10.1016\/j.celrep.2022.111124_bib90","doi-asserted-by":"crossref","first-page":"33154","DOI":"10.1074\/jbc.M110.143685","article-title":"Exposure to hydrogen peroxide induces oxidation and activation of AMP-activated protein kinase","volume":"285","author":"Zmijewski","year":"2010","journal-title":"J.\u00a0Biol. Chem."},{"key":"10.1016\/j.celrep.2022.111124_bib91","doi-asserted-by":"crossref","unstructured":"Lodi, R., Taylor, D.J., Tabrizi, S.J., Kumar, S., Sweeney, M., Wood, N.W., Styles, P., Radda, G.K., and Schapira, A.H. In vivo skeletal muscle mitochondrial function in Leber's hereditary optic neuropathy assessed by 31P magnetic resonance spectroscopy. Ann Neurol. 1997 Oct;42(4):573-9. doi: 10.1002\/ana.410420407. 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