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The\n                    <jats:italic>i<\/jats:italic>\n                    \u2010AAA protease YME1L ensures mitochondrial proteostasis and regulates mitochondrial dynamics by processing of the dynamin\u2010like GTPase OPA1. Mutations in\n                    <jats:italic>YME1L<\/jats:italic>\n                    cause a multi\u2010systemic mitochondriopathy associated with neurological dysfunction and mitochondrial fragmentation but pathogenic mechanisms remained enigmatic. Here, we report on striking cell\u2010type\u2010specific defects in mice lacking YME1L in the nervous system. YME1L\u2010deficient mice manifest ocular dysfunction with microphthalmia and cataracts and develop deficiencies in locomotor activity due to specific degeneration of spinal cord axons, which relay proprioceptive signals from the hind limbs to the cerebellum. Mitochondrial fragmentation occurs throughout the nervous system and does not correlate with the degenerative phenotype. Deletion of\n                    <jats:italic>Oma1<\/jats:italic>\n                    restores tubular mitochondria but deteriorates axonal degeneration in the absence of YME1L, demonstrating that impaired mitochondrial proteostasis rather than mitochondrial fragmentation causes the observed neurological defects.\n                  <\/jats:p>","DOI":"10.15252\/emmm.201809288","type":"journal-article","created":{"date-parts":[[2018,11,2]],"date-time":"2018-11-02T08:05:16Z","timestamp":1541145916000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":54,"title":["Loss of the mitochondrial i\u2010AAA protease YME1L leads to ocular dysfunction and spinal axonopathy"],"prefix":"10.1038","volume":"11","author":[{"given":"Hans\u2010Georg","family":"Sprenger","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gulzar","family":"Wani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annika","family":"Hesseling","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tim","family":"K\u00f6nig","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Patron","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"MacVicar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sofia","family":"Ahola","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy","family":"Wai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Esther","family":"Barth","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena I","family":"Rugarli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matteo","family":"Bergami","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1250-1462","authenticated-orcid":false,"given":"Thomas","family":"Langer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2018,11,2]]},"reference":[{"key":"BFEMMM201809288_CR1","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.ydbio.2007.01.045","volume":"305","author":"R Adler","year":"2007","unstructured":"Adler R, Canto\u2010Soler MV (2007) Molecular mechanisms of optic vesicle development: complexities, ambiguities and controversies. 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