{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,19]],"date-time":"2026-02-19T08:28:33Z","timestamp":1771489713433,"version":"3.50.1"},"reference-count":30,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[2024,11,13]],"date-time":"2024-11-13T00:00:00Z","timestamp":1731456000000},"content-version":"vor","delay-in-days":317,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"French National Scientific Research Council"},{"DOI":"10.13039\/501100001677","name":"French National Institute for Health and Medical Research","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001677","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100008280","name":"University of Angers","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100008280","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100013875","name":"Retina France","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100013875","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Ouvrir-Les-Yeux and Kjer-France"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,10,30]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Hereditary optic neuropathies, including dominant optic atrophy and Leber\u2019s hereditary optic neuropathy, are genetic disorders characterized by retinal ganglion cell degeneration leading to vision loss, mainly associated with mitochondrial dysfunction. In this study, we analysed mitochondrial distribution and ultrastructure in the retina and longitudinal optic nerve sections of pre-symptomatic hereditary optic neuropathies mouse models with Opa1 and Nd6 deficiency to identify early mitochondrial changes. Our results show significant mitochondrial fragmentation and increased mitophagy in Opa1+\/\u2212 mice, indicating early mitochondrial changes prior to neuronal loss. Conversely, Nd6P25L mice exhibited mitochondrial hypertrophy, suggesting an adaptive response to compensate for altered energy metabolism. These pre-symptomatic mitochondrial changes were mainly observed in the unmyelinated portion of the retinal ganglion cell axons, where the transmission of the visual information requires high energy expenditure, constituting the specific point of vulnerability in hereditary optic neuropathies. These findings highlight early focal mitochondrial changes prior to neuronal loss in hereditary optic neuropathies and provide insight into pre-symptomatic therapeutic approaches.<\/jats:p>","DOI":"10.1093\/braincomms\/fcae404","type":"journal-article","created":{"date-parts":[[2024,11,13]],"date-time":"2024-11-13T11:37:41Z","timestamp":1731497861000},"source":"Crossref","is-referenced-by-count":2,"title":["Opa1 and MT-Nd6 mutations induce early mitochondrial changes in the retina and prelaminar optic nerve of hereditary optic neuropathy mouse models"],"prefix":"10.1093","volume":"6","author":[{"given":"Jacques","family":"Bureau","sequence":"first","affiliation":[{"name":"Laboratoire de neurobiologie et neuropathologie, Centre Hospitalier Universitaire d\u2019Angers , 49933 Angers ,","place":["France"]},{"name":"University of Angers, Equipe MitoLab, Unit\u00e9 MitoVasc, INSERM U1083, CNRS 6015, SFR ICAT , 49933 Angers ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Florence","family":"Manero","sequence":"additional","affiliation":[{"name":"University of Angers, SCIAM Microscopy Core Facility, SFR ICAT , F-49000, 49933 Angers ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Olivier","family":"Baris","sequence":"additional","affiliation":[{"name":"University of Angers, Equipe MitoLab, Unit\u00e9 MitoVasc, INSERM U1083, CNRS 6015, SFR ICAT , 49933 Angers ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexia","family":"Bodin","sequence":"additional","affiliation":[{"name":"University of Angers, Equipe MitoLab, Unit\u00e9 MitoVasc, INSERM U1083, CNRS 6015, SFR ICAT , 49933 Angers ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christophe","family":"Verny","sequence":"additional","affiliation":[{"name":"University of Angers, Equipe MitoLab, Unit\u00e9 MitoVasc, INSERM U1083, CNRS 6015, SFR ICAT , 49933 Angers ,","place":["France"]},{"name":"Service de neurologie, centre de r\u00e9f\u00e9rence des maladies neurog\u00e9n\u00e9tiques, Centre Hospitalier Universitaire d\u2019Angers , 49933 Angers ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arnaud","family":"Chevrollier","sequence":"additional","affiliation":[{"name":"University of Angers, Equipe MitoLab, Unit\u00e9 MitoVasc, INSERM U1083, CNRS 6015, SFR ICAT , 49933 Angers ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Guy","family":"Lenaers","sequence":"additional","affiliation":[{"name":"University of Angers, Equipe MitoLab, Unit\u00e9 MitoVasc, INSERM U1083, CNRS 6015, SFR ICAT , 49933 Angers ,","place":["France"]},{"name":"Service de neurologie, centre de r\u00e9f\u00e9rence des maladies neurog\u00e9n\u00e9tiques, Centre Hospitalier Universitaire d\u2019Angers , 49933 Angers 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