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Detailed examination of the protein in disease-affected tissues from amyotrophic lateral sclerosis patients, however, remains scarce.<\/jats:p>\n                  <jats:p>We used histological, biochemical and analytical techniques to profile alterations to SOD1 protein deposition, subcellular localization, maturation and post-translational modification in post-mortem spinal cord tissues from amyotrophic lateral sclerosis cases and controls. Tissues were dissected into ventral and dorsal spinal cord grey matter to assess the specificity of alterations within regions of motor neuron degeneration.<\/jats:p>\n                  <jats:p>We provide evidence of the mislocalization and accumulation of structurally disordered, immature SOD1 protein conformers in spinal cord motor neurons of SOD1-linked and non-SOD1-linked familial amyotrophic lateral sclerosis cases, and sporadic amyotrophic lateral sclerosis cases, compared with control motor neurons. These changes were collectively associated with instability and mismetallation of enzymatically active SOD1 dimers, as well as alterations to SOD1 post-translational modifications and molecular chaperones governing SOD1 maturation. Atypical changes to SOD1 protein were largely restricted to regions of neurodegeneration in amyotrophic lateral sclerosis cases, and clearly differentiated all forms of amyotrophic lateral sclerosis from controls. Substantial heterogeneity in the presence of these changes was also observed between amyotrophic lateral sclerosis cases.<\/jats:p>\n                  <jats:p>Our data demonstrate that varying forms of SOD1 proteinopathy are a common feature of all forms of amyotrophic lateral sclerosis, and support the presence of one or more convergent biochemical pathways leading to SOD1 proteinopathy in amyotrophic lateral sclerosis. Most of these alterations are specific to regions of neurodegeneration, and may therefore constitute valid targets for therapeutic development.<\/jats:p>","DOI":"10.1093\/brain\/awac165","type":"journal-article","created":{"date-parts":[[2022,5,4]],"date-time":"2022-05-04T07:18:59Z","timestamp":1651648739000},"page":"3108-3130","source":"Crossref","is-referenced-by-count":80,"title":["Altered SOD1 maturation and post-translational modification in amyotrophic lateral sclerosis spinal cord"],"prefix":"10.1093","volume":"145","author":[{"given":"Benjamin G","family":"Trist","sequence":"first","affiliation":[{"name":"Brain and Mind Centre and School of Medical Sciences (Neuroscience), Faculty of Medicine and Health, The University of Sydney , Sydney 2006 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sian","family":"Genoud","sequence":"additional","affiliation":[{"name":"Brain and Mind Centre and School of Medical Sciences (Neuroscience), Faculty of Medicine 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School of Medical Sciences (Neuroscience), Faculty of Medicine and Health, The University of Sydney , Sydney 2006 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominic J","family":"Hare","sequence":"additional","affiliation":[{"name":"School of Biosciences, The University of Melbourne , Parkville 3010 , Australia"},{"name":"Atomic Medicine Initiative, University of Technology Sydney , Broadway 2007 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melanie","family":"White","sequence":"additional","affiliation":[{"name":"School of Life and Environmental Sciences, Faculty of Science, The University of Sydney , Sydney 2006 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jens","family":"Altvater","sequence":"additional","affiliation":[{"name":"Sydney Mass Spectrometry, The University of Sydney , Sydney 2006 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer A","family":"Fifita","sequence":"additional","affiliation":[{"name":"Centre for Motor Neuron Disease Research, Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University , Sydney , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alison","family":"Hogan","sequence":"additional","affiliation":[{"name":"Centre for Motor Neuron Disease Research, Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University , Sydney , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6434-7706","authenticated-orcid":false,"given":"Natalie","family":"Grima","sequence":"additional","affiliation":[{"name":"Centre for Motor Neuron Disease Research, Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University , Sydney , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ian P","family":"Blair","sequence":"additional","affiliation":[{"name":"Centre for Motor Neuron Disease Research, Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University , Sydney , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kai","family":"Kysenius","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Pharmacology, The University of Melbourne , Parkville 3010 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter J","family":"Crouch","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Pharmacology, The University of Melbourne , Parkville 3010 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Asuncion","family":"Carmona","sequence":"additional","affiliation":[{"name":"University Bordeaux, CNRS, CENBG, UMR 5797 , F-33170 Gradignan , 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