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Mutated HSP proteins include myelin proteolipid protein (PLP) and axon-enriched proteins involved in mitochondrial function, smooth endoplasmic reticulum (SER) structure, and microtubule (MT) stability\/function. We characterized axonal mitochondria, SER, and MTs in rodent optic nerves where PLP is replaced by the peripheral nerve myelin protein, P0 (P0-CNS mice). Mitochondrial pathology and degeneration were prominent in juxtaparanodal axoplasm at 1 mo of age. In wild-type (WT) optic nerve axons, 25% of mitochondria\u2013SER associations occurred on extensions of the mitochondrial outer membrane. Mitochondria\u2013SER associations were reduced by 86% in 1-mo-old P0-CNS juxtaparanodal axoplasm. 1-mo-old P0-CNS optic nerves were more sensitive to oxygen-glucose deprivation and contained less adenosine triphosphate (ATP) than WT nerves. MT pathology and paranodal axonal ovoids were prominent at 6 mo. These data support juxtaparanodal mitochondrial degeneration, reduced mitochondria\u2013SER associations, and reduced ATP production as causes of axonal ovoid formation and axonal degeneration.<\/jats:p>","DOI":"10.1083\/jcb.201607099","type":"journal-article","created":{"date-parts":[[2016,11,21]],"date-time":"2016-11-21T09:40:36Z","timestamp":1479721236000},"page":"531-542","update-policy":"https:\/\/doi.org\/10.1083\/jcb.crossmarkpolicy","source":"Crossref","is-referenced-by-count":54,"title":["Proteolipid protein\u2013deficient myelin promotes axonal mitochondrial dysfunction via altered metabolic coupling"],"prefix":"10.1083","volume":"215","author":[{"given":"Xinghua","family":"Yin","sequence":"first","affiliation":[{"name":"Department of Neurosciences, Lerner Research Institute, Cleveland Clinic, Cleveland, OH 44195 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3956-2206","authenticated-orcid":false,"given":"Grahame J.","family":"Kidd","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Lerner Research Institute, Cleveland Clinic, Cleveland, OH 44195 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6536-2753","authenticated-orcid":false,"given":"Nobuhiko","family":"Ohno","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Lerner Research Institute, Cleveland Clinic, Cleveland, OH 44195 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1878-8735","authenticated-orcid":false,"given":"Guy A.","family":"Perkins","sequence":"additional","affiliation":[{"name":"National Center for Microscopy and Imaging Research, University of California, San Diego, La Jolla, CA 92093 2"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark H.","family":"Ellisman","sequence":"additional","affiliation":[{"name":"National Center for Microscopy and Imaging Research, University of California, San Diego, La Jolla, CA 92093 2"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5759-5496","authenticated-orcid":false,"given":"Chinthasagar","family":"Bastian","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Lerner Research Institute, Cleveland Clinic, Cleveland, OH 44195 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7506-9585","authenticated-orcid":false,"given":"Sylvain","family":"Brunet","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Lerner Research Institute, Cleveland Clinic, Cleveland, OH 44195 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1123-3582","authenticated-orcid":false,"given":"Selva","family":"Baltan","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Lerner Research Institute, Cleveland Clinic, Cleveland, OH 44195 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6682-3737","authenticated-orcid":false,"given":"Bruce D.","family":"Trapp","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Lerner Research Institute, Cleveland Clinic, Cleveland, OH 44195 1"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"291","published-online":{"date-parts":[[2016,11,21]]},"reference":[{"key":"2023072309062506100_bib1","doi-asserted-by":"publisher","first-page":"505","DOI":"10.1016\/j.cell.2013.07.005","article-title":"AMPD2 regulates GTP synthesis and is mutated in a potentially treatable neurodegenerative brainstem disorder","volume":"154","author":"Akizu","year":"2013","journal-title":"Cell."},{"key":"2023072309062506100_bib2","doi-asserted-by":"publisher","first-page":"1736","DOI":"10.1093\/brain\/awn119","article-title":"Abnormally phosphorylated tau is associated with neuronal and axonal loss in experimental autoimmune encephalomyelitis and multiple sclerosis","volume":"131","author":"Anderson","year":"2008","journal-title":"Brain."},{"key":"2023072309062506100_bib3","doi-asserted-by":"publisher","first-page":"591","DOI":"10.1007\/s00401-010-0671-4","article-title":"Abnormal tau phosphorylation in primary progressive multiple sclerosis","volume":"119","author":"Anderson","year":"2010","journal-title":"Acta Neuropathol."},{"key":"2023072309062506100_bib4","doi-asserted-by":"publisher","first-page":"495","DOI":"10.1083\/jcb.111.2.495","article-title":"Individual microtubules in the axon consist of domains that differ in both composition and stability","volume":"111","author":"Baas","year":"1990","journal-title":"J. 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