{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T21:58:58Z","timestamp":1791928738471,"version":"4.3.4"},"reference-count":54,"publisher":"Association for Research in Vision and Ophthalmology (ARVO)","issue":"6","license":[{"start":{"date-parts":[[2020,6,19]],"date-time":"2020-06-19T00:00:00Z","timestamp":1592524800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Invest. Ophthalmol. Vis. Sci."],"published-print":{"date-parts":[[2020,6,19]]},"DOI":"10.1167\/iovs.61.6.42","type":"journal-article","created":{"date-parts":[[2020,6,19]],"date-time":"2020-06-19T20:31:55Z","timestamp":1592598715000},"page":"42","source":"Crossref","is-referenced-by-count":34,"title":["Opa1 Deficiency Leads to Diminished Mitochondrial Bioenergetics With Compensatory Increased Mitochondrial Motility"],"prefix":"10.1167","volume":"61","author":[{"given":"Shanshan","family":"Sun","sequence":"first","affiliation":[{"name":"School of Optometry and Vision Sciences, Cardiff University, Cardiff, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irina","family":"Erchova","sequence":"additional","affiliation":[{"name":"School of Optometry and Vision Sciences, Cardiff University, Cardiff, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank","family":"Sengpiel","sequence":"additional","affiliation":[{"name":"School of Biosciences, Cardiff University, Cardiff, United Kingdom"},{"name":"Neuroscience & Mental Health Research Institute, Cardiff University, Cardiff, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcela","family":"Votruba","sequence":"additional","affiliation":[{"name":"School of Optometry and Vision Sciences, Cardiff University, Cardiff, United Kingdom"},{"name":"Cardiff Eye Unit, University Hospital of Wales, Cardiff, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"27","reference":[{"key":"bib1","doi-asserted-by":"publisher","first-page":"596","DOI":"10.1038\/eye.2011.2","article-title":"Pattern of retinal ganglion cell loss in dominant optic atrophy due to OPA1 mutations","volume":"25","author":"Yu-Wai-Man","year":"2011","journal-title":"Eye (Lond)"},{"key":"bib2","doi-asserted-by":"publisher","first-page":"337","DOI":"10.1111\/j.1600-0420.2005.00448.x","article-title":"Dominant optic atrophy: correlation between clinical and molecular genetic studies","volume":"83","author":"Puomila","year":"2005","journal-title":"Acta Ophthalmol Scand"},{"key":"bib3","doi-asserted-by":"publisher","first-page":"475","DOI":"10.1097\/ICU.0000000000000314","article-title":"Dominant optic atrophy: updates on the pathophysiology and clinical manifestations of the optic atrophy 1 mutation","volume":"27","author":"Chun","year":"2016","journal-title":"Curr Opin Ophthalmol"},{"key":"bib4","doi-asserted-by":"publisher","first-page":"129","DOI":"10.1016\/j.spen.2017.06.001","article-title":"Dominant Optic Atrophy and Leber's Hereditary Optic Neuropathy: Update on Clinical Features and Current Therapeutic Approaches","volume":"24","author":"Chun","year":"2017","journal-title":"Semin Pediatr Neurol"},{"key":"bib5","doi-asserted-by":"publisher","first-page":"E692","DOI":"10.1002\/humu.v30:7","article-title":"Molecular screening of 980 cases of suspected hereditary optic neuropathy with a report on 77 novel OPA1 mutations","volume":"30","author":"Ferre","year":"2009","journal-title":"Hum Mutat"},{"key":"bib6","doi-asserted-by":"publisher","first-page":"32","DOI":"10.1186\/1750-1326-8-32","article-title":"Dominant optic atrophy, OPA1, and mitochondrial quality control: understanding mitochondrial network dynamics","volume":"8","author":"Alavi","year":"2013","journal-title":"Mol Neurodegener"},{"key":"bib7","doi-asserted-by":"publisher","first-page":"300","DOI":"10.1111\/j.1755-3768.1983.tb01424.x","article-title":"Histopathology of eye, optic nerve and brain in a case of dominant optic atrophy","volume":"61","author":"Kjer","year":"1983","journal-title":"Acta Ophthalmol (Copenh)"},{"key":"bib8","doi-asserted-by":"publisher","first-page":"186","DOI":"10.1016\/j.mito.2017.08.014","article-title":"Mitochondrial dynamics, transport, and quality control: A bottleneck for retinal ganglion cell viability in optic neuropathies","volume":"36","author":"Ito","year":"2017","journal-title":"Mitochondrion"},{"key":"bib9","doi-asserted-by":"publisher","first-page":"1307","DOI":"10.1093\/hmg\/ddm079","article-title":"Opa1 deficiency in a mouse model of autosomal dominant optic atrophy impairs mitochondrial morphology, optic nerve structure and visual function","volume":"16","author":"Davies","year":"2007","journal-title":"Hum Mol Genet"},{"key":"bib10","first-page":"643","article-title":"Purification and culture of retinal ganglion cells from rodents","volume":"2013","author":"Winzeler","year":"2013","journal-title":"Cold Spring Harb Protoc"},{"key":"bib11","doi-asserted-by":"publisher","first-page":"791","DOI":"10.1016\/0896-6273(88)90127-4","article-title":"Immunological, morphological, and electrophysiological variation among retinal ganglion cells purified by panning","volume":"1","author":"Barres","year":"1988","journal-title":"Neuron"},{"key":"bib12","first-page":"447","article-title":"Parkin overexpression protects retinal ganglion cells against glutamate excitotoxicity","volume":"23","author":"Hu","year":"2017","journal-title":"Mol Vis"},{"key":"bib13","first-page":"387","article-title":"Comparative analysis of three purification protocols for retinal ganglion cells from rat","volume":"22","author":"Gao","year":"2016","journal-title":"Mol Vis"},{"key":"bib14","doi-asserted-by":"publisher","first-page":"4288","DOI":"10.1167\/iovs.03-1407","article-title":"Expression of the Opa1 mitochondrial protein in retinal ganglion cells: its downregulation causes aggregation of the mitochondrial network","volume":"46","author":"Kamei","year":"2005","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"bib15","doi-asserted-by":"publisher","first-page":"71","DOI":"10.1111\/tra.2017.18.issue-1","article-title":"KymoAnalyzer: a software tool for the quantitative analysis of intracellular transport in neurons","volume":"18","author":"Neumann","year":"2017","journal-title":"Traffic"},{"key":"bib16","doi-asserted-by":"publisher","first-page":"e2309","DOI":"10.1038\/cddis.2016.160","article-title":"Mitochondrial dysfunction in an Opa1(Q285STOP) mouse model of dominant optic atrophy results from Opa1 haploinsufficiency","volume":"7","author":"Kushnareva","year":"2016","journal-title":"Cell Death Dis"},{"key":"bib17","doi-asserted-by":"publisher","first-page":"5704","DOI":"10.1038\/s41598-017-05571-y","article-title":"A novel ADOA-associated OPA1 mutation alters the mitochondrial function, membrane potential, ROS production and apoptosis","volume":"7","author":"Zhang","year":"2017","journal-title":"Sci Rep"},{"key":"bib18","doi-asserted-by":"publisher","first-page":"131","DOI":"10.1212\/WNL.0000000000003491","article-title":"Dysregulated mitophagy and mitochondrial organization in optic atrophy due to OPA1 mutations","volume":"88","author":"Liao","year":"2017","journal-title":"Neurology"},{"key":"bib19","doi-asserted-by":"publisher","first-page":"2269","DOI":"10.1167\/iovs.14-16288","article-title":"Changes in Mitochondrial Morphology and Bioenergetics in Human Lymphoblastoid Cells With Four Novel OPA1 Mutations","volume":"56","author":"Kao","year":"2015","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"bib20","doi-asserted-by":"publisher","first-page":"90","DOI":"10.1016\/j.ceb.2016.02.022","article-title":"Mechanics of mitochondrial motility in neurons","volume":"38","author":"Barnhart","year":"2016","journal-title":"Curr Opin Cell Biol"},{"key":"bib21","doi-asserted-by":"publisher","first-page":"557","DOI":"10.1002\/dneu.v74.6","article-title":"AMP-activated protein kinase mediates activity-dependent axon branching by recruiting mitochondria to axon","volume":"74","author":"Tao","year":"2014","journal-title":"Dev Neurobiol"},{"key":"bib22","doi-asserted-by":"publisher","first-page":"2187","DOI":"10.1242\/jcs.118844","article-title":"Principles of the mitochondrial fusion and fission cycle in neurons","volume":"126","author":"Cagalinec","year":"2013","journal-title":"J Cell Sci"},{"key":"bib23","doi-asserted-by":"publisher","first-page":"C477","DOI":"10.1152\/ajpcell.00427.2009","article-title":"Biophysical properties of mitochondrial fusion events in pancreatic beta-cells and cardiac cells unravel potential control mechanisms of its selectivity","volume":"299","author":"Twig","year":"2010","journal-title":"Am J Physiol Cell Physiol"},{"key":"bib24","doi-asserted-by":"publisher","first-page":"3074","DOI":"10.1038\/emboj.2009.255","article-title":"Mitochondrial \u2018kiss-and-run\u2019: interplay between mitochondrial motility and fusion-fission dynamics","volume":"28","author":"Liu","year":"2009","journal-title":"EMBO J"},{"key":"bib25","doi-asserted-by":"publisher","first-page":"383","DOI":"10.1016\/j.cell.2006.09.021","article-title":"Mitochondrial inner-membrane fusion and crista maintenance requires the dynamin-related GTPase Mgm1","volume":"127","author":"Meeusen","year":"2006","journal-title":"Cell"},{"key":"bib26","doi-asserted-by":"publisher","first-page":"160","DOI":"10.1016\/j.cell.2013.08.032","article-title":"Mitochondrial cristae shape determines respiratory chain supercomplexes assembly and respiratory efficiency","volume":"155","author":"Cogliati","year":"2013","journal-title":"Cell"},{"key":"bib27","doi-asserted-by":"publisher","first-page":"177","DOI":"10.1016\/j.cell.2006.06.025","article-title":"OPA1 controls apoptotic cristae remodeling independently from mitochondrial fusion","volume":"126","author":"Frezza","year":"2006","journal-title":"Cell"},{"key":"bib28","doi-asserted-by":"publisher","first-page":"505","DOI":"10.1146\/annurev-physiol-021115-105011","article-title":"Mito-Morphosis: Mitochondrial Fusion, Fission, and Cristae Remodeling as Key Mediators of Cellular Function","volume":"78","author":"Pernas","year":"2016","journal-title":"Annu Rev Physiol"},{"key":"bib29","doi-asserted-by":"publisher","first-page":"1481","DOI":"10.1016\/j.bbamcr.2017.05.004","article-title":"Mitochondrial contact site and cristae organizing system: A central player in membrane shaping and crosstalk","volume":"1864","author":"Wollweber","year":"2017","journal-title":"Biochim Biophys Acta Mol Cell Res"},{"key":"bib30","doi-asserted-by":"publisher","first-page":"589","DOI":"10.1038\/ncb2220","article-title":"During autophagy mitochondria elongate, are spared from degradation and sustain cell viability","volume":"13","author":"Gomes","year":"2011","journal-title":"Nat Cell Biol"},{"key":"bib31","doi-asserted-by":"publisher","first-page":"719","DOI":"10.1002\/(ISSN)1531-8249","article-title":"Deficit of in vivo mitochondrial ATP production in OPA1-related dominant optic atrophy","volume":"56","author":"Lodi","year":"2004","journal-title":"Ann Neurol"},{"key":"bib32","doi-asserted-by":"publisher","first-page":"352","DOI":"10.1093\/brain\/awm335","article-title":"OPA1 mutations associated with dominant optic atrophy impair oxidative phosphorylation and mitochondrial fusion","volume":"131","author":"Zanna","year":"2008","journal-title":"Brain"},{"key":"bib33","doi-asserted-by":"publisher","first-page":"22","DOI":"10.1186\/1471-2091-9-22","article-title":"Mitochondrial oxidative phosphorylation in autosomal dominant optic atrophy","volume":"9","author":"Mayorov","year":"2008","journal-title":"BMC Biochem"},{"key":"bib34","doi-asserted-by":"publisher","first-page":"3291","DOI":"10.1093\/hmg\/ddn225","article-title":"A novel deletion in the GTPase domain of OPA1 causes defects in mitochondrial morphology and distribution, but not in function","volume":"17","author":"Spinazzi","year":"2008","journal-title":"Hum Mol Genet"},{"key":"bib35","doi-asserted-by":"publisher","first-page":"R12","DOI":"10.1093\/hmg\/ddx182","article-title":"Inherited eye-related disorders due to mitochondrial dysfunction","volume":"26","author":"Yu-Wai-Man","year":"2017","journal-title":"Hum Mol Genet"},{"key":"bib36","doi-asserted-by":"publisher","first-page":"789","DOI":"10.1007\/s00401-016-1625-2","article-title":"A neurodegenerative perspective on mitochondrial optic neuropathies","volume":"132","author":"Yu-Wai-Man","year":"2016","journal-title":"Acta Neuropathol"},{"key":"bib37","doi-asserted-by":"publisher","first-page":"e11583","DOI":"10.7554\/eLife.11583","article-title":"Dendritic mitochondria reach stable positions during circuit development","volume":"5","author":"Faits","year":"2016","journal-title":"Elife"},{"key":"bib38","doi-asserted-by":"publisher","first-page":"10515","DOI":"10.1073\/pnas.1509879112","article-title":"In vivo imaging of axonal transport of mitochondria in the diseased and aged mammalian CNS","volume":"112","author":"Takihara","year":"2015","journal-title":"Proc Natl Acad Sci USA"},{"issue":"Pt 3","key":"bib39","doi-asserted-by":"crossref","first-page":"917","DOI":"10.1242\/jcs.104.3.917","article-title":"The regulation of bidirectional mitochondrial transport is coordinated with axonal outgrowth","volume":"104","author":"Morris","year":"1993","journal-title":"J Cell Sci"},{"key":"bib40","doi-asserted-by":"publisher","first-page":"8618","DOI":"10.1523\/JNEUROSCI.23-24-08618.2003","article-title":"Response of mitochondrial traffic to axon determination and differential branch growth","volume":"23","author":"Ruthel","year":"2003","journal-title":"J Neurosci"},{"key":"bib41","doi-asserted-by":"publisher","first-page":"2662","DOI":"10.1091\/mbc.e16-05-0286","article-title":"AMPK activity regulates trafficking of mitochondria to the leading edge during cell migration and matrix invasion","volume":"27","author":"Cunniff","year":"2016","journal-title":"Mol Biol Cell"},{"key":"bib42","doi-asserted-by":"publisher","first-page":"7402","DOI":"10.1167\/iovs.12-10298","article-title":"Mitochondrial dynamics regulate growth cone motility, guidance, and neurite growth rate in perinatal retinal ganglion cells in vitro","volume":"53","author":"Steketee","year":"2012","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"bib43","doi-asserted-by":"publisher","first-page":"1189","DOI":"10.1111\/ejn.2004.20.issue-5","article-title":"BDNF increases rat brain mitochondrial respiratory coupling at complex I, but not complex II","volume":"20","author":"Markham","year":"2004","journal-title":"Eur J Neurosci"},{"key":"bib44","doi-asserted-by":"publisher","first-page":"1272","DOI":"10.1016\/j.cub.2004.07.027","article-title":"Nerve growth factor signaling regulates motility and docking of axonal mitochondria","volume":"14","author":"Chada","year":"2004","journal-title":"Curr Biol"},{"key":"bib45","doi-asserted-by":"publisher","first-page":"963","DOI":"10.1126\/science.1161566","article-title":"Promoting axon regeneration in the adult CNS by modulation of the PTEN\/mTOR pathway","volume":"322","author":"Park","year":"2008","journal-title":"Science"},{"key":"bib46","doi-asserted-by":"publisher","first-page":"369","DOI":"10.1038\/35000309","article-title":"Nogo in nerve regeneration","volume":"403","author":"Goldberg","year":"2000","journal-title":"Nature"},{"key":"bib47","doi-asserted-by":"publisher","first-page":"36","DOI":"10.1046\/j.1471-4159.2002.00788.x","article-title":"Identification and characterization of a novel Nogo-interacting mitochondrial protein (NIMP)","volume":"81","author":"Hu","year":"2002","journal-title":"J Neurochem"},{"key":"bib48","doi-asserted-by":"publisher","first-page":"10669","DOI":"10.1038\/s41598-019-46956-5","article-title":"MTP18 is a Novel Regulator of Mitochondrial Fission in CNS Neuron Development, Axonal Growth, and Injury Responses","volume":"9","author":"Kreymerman","year":"2019","journal-title":"Sci Rep"},{"key":"bib49","doi-asserted-by":"publisher","first-page":"2426","DOI":"10.1093\/brain\/awq155","article-title":"Melanopsin retinal ganglion cells are resistant to neurodegeneration in mitochondrial optic neuropathies","volume":"133","author":"La Morgia","year":"2010","journal-title":"Brain"},{"key":"bib50","doi-asserted-by":"publisher","first-page":"e56350","DOI":"10.1371\/journal.pone.0056350","article-title":"Non-image-forming light driven functions are preserved in a mouse model of autosomal dominant optic atrophy","volume":"8","author":"Perganta","year":"2013","journal-title":"PLoS One"},{"key":"bib51","doi-asserted-by":"publisher","first-page":"4835","DOI":"10.1167\/iovs.15-16743","article-title":"Influence of Opa1 Mutation on Survival and Function of Retinal Ganglion Cells","volume":"56","author":"Gonzalez-Menendez","year":"2015","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"bib52","doi-asserted-by":"publisher","first-page":"77","DOI":"10.1016\/j.mito.2017.04.003","article-title":"Melanopsin-expressing retinal ganglion cells are resistant to cell injury, but not always","volume":"36","author":"Georg","year":"2017","journal-title":"Mitochondrion"},{"key":"bib53","doi-asserted-by":"publisher","first-page":"95","DOI":"10.1016\/0168-0102(93)90076-3","article-title":"Formation and maturation of synapses in primary cultures of rat cerebral cortical cells: an electron microscopic study","volume":"16","author":"Ichikawa","year":"1993","journal-title":"Neurosci Res"},{"key":"bib54","doi-asserted-by":"publisher","first-page":"657","DOI":"10.1126\/science.291.5504.657","article-title":"Control of synapse number by glia","volume":"291","author":"Ullian","year":"2001","journal-title":"Science"}],"container-title":["Investigative Opthalmology &amp; Visual Science"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/iovs.arvojournals.org\/article.aspx?articleid=2770165","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T21:31:19Z","timestamp":1791927079000},"score":1,"resource":{"primary":{"URL":"https:\/\/iovs.arvojournals.org\/article.aspx?articleid=2770165"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,6,19]]},"references-count":54,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2020,6,3]]}},"URL":"https:\/\/doi.org\/10.1167\/iovs.61.6.42","relation":{},"ISSN":["1552-5783"],"issn-type":[{"value":"1552-5783","type":"print"}],"subject":[],"published":{"date-parts":[[2020,6,19]]}}}