{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T16:54:07Z","timestamp":1788368047115,"version":"build-2803163510"},"reference-count":73,"publisher":"Oxford University Press (OUP)","issue":"21","license":[{"start":{"date-parts":[[2019,9,6]],"date-time":"2019-09-06T00:00:00Z","timestamp":1567728000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100007155","name":"Medical Research Council","doi-asserted-by":"publisher","award":["MR\/P502005\/1"],"award-info":[{"award-number":["MR\/P502005\/1"]}],"id":[{"id":"10.13039\/501100007155","id-type":"DOI","asserted-by":"publisher"}]},{"name":"European Research Council Consolidator Award","award":["311394"],"award-info":[{"award-number":["311394"]}]},{"name":"Motor Neurone Disease Association Prize Studentship"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,11,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>A common pathological hallmark of amyotrophic lateral sclerosis (ALS) and the related neurodegenerative disorder frontotemporal dementia, is the cellular mislocalization of transactive response DNA-binding protein 43\u00a0kDa (TDP-43). Additionally, multiple mutations in the TARDBP gene (encoding TDP-43) are associated with familial forms of ALS. While the exact role for TDP-43 in the onset and progression of ALS remains unclear, the identification of factors that can prevent aberrant TDP-43 localization and function could be clinically beneficial. Previously, we discovered that the oxidation resistance 1 (Oxr1) protein could alleviate cellular mislocalization phenotypes associated with TDP-43 mutations, and that over-expression of Oxr1 was able to delay neuromuscular abnormalities in the hSOD1G93A ALS mouse model. Here, to determine whether Oxr1 can protect against TDP-43-associated phenotypes in vitro and in vivo, we used the same genetic approach in a newly described transgenic mouse expressing the human TDP-43 locus harbouring an ALS disease mutation (TDP-43M337V). We show in primary motor neurons from TDP-43M337V mice that genetically-driven Oxr1 over-expression significantly alleviates cytoplasmic mislocalization of mutant TDP-43. We also further quantified newly-identified, late-onset neuromuscular phenotypes of this mutant line, and demonstrate that neuronal Oxr1 over-expression causes a significant reduction in muscle denervation and neuromuscular junction degeneration in homozygous mutants in parallel with improved motor function and a reduction in neuroinflammation. Together these data support the application of Oxr1 as a viable and safe modifier of TDP-43-associated ALS phenotypes.<\/jats:p>","DOI":"10.1093\/hmg\/ddz190","type":"journal-article","created":{"date-parts":[[2019,8,27]],"date-time":"2019-08-27T15:21:14Z","timestamp":1566919274000},"page":"3584-3599","source":"Crossref","is-referenced-by-count":25,"title":["Neuronal over-expression of Oxr1 is protective against ALS-associated mutant TDP-43 mislocalisation in motor neurons and neuromuscular defects in vivo"],"prefix":"10.1093","volume":"28","author":[{"given":"Matthew G","family":"Williamson","sequence":"first","affiliation":[{"name":"Department of Physiology, Anatomy and Genetics, University of Oxford, Parks Road, Oxford OX1 3PT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matt\u00e9a J","family":"Finelli","sequence":"additional","affiliation":[{"name":"Department of Physiology, Anatomy and Genetics, University of Oxford, Parks Road, Oxford OX1 3PT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James N","family":"Sleigh","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Diseases, Institute of Neurology, University College London, London WC1N 3BG, UK"},{"name":"UK Dementia Research Institute, University College London, London WC1E 6BT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy","family":"Reddington","sequence":"additional","affiliation":[{"name":"Department of Physiology, Anatomy and Genetics, University of Oxford, Parks Road, Oxford OX1 3PT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Gordon","sequence":"additional","affiliation":[{"name":"Nuffield Department of Clinical Neurosciences, University of Oxford, West Wing, John Radcliffe Hospital, Oxford OX3 9DU, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5490-1697","authenticated-orcid":false,"given":"Kevin","family":"Talbot","sequence":"additional","affiliation":[{"name":"Nuffield Department of Clinical Neurosciences, University of Oxford, West Wing, John Radcliffe Hospital, Oxford OX3 9DU, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kay E","family":"Davies","sequence":"additional","affiliation":[{"name":"Department of Physiology, Anatomy and Genetics, University of Oxford, Parks Road, Oxford OX1 3PT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter L","family":"Oliver","sequence":"additional","affiliation":[{"name":"Department of Physiology, Anatomy and Genetics, University of Oxford, Parks Road, Oxford OX1 3PT, UK"},{"name":"MRC Harwell Institute, Harwell Campus, Didcot, Oxfordshire, OX11 0RD, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2019,9,6]]},"reference":[{"key":"2019122311035355900_ref1","doi-asserted-by":"crossref","first-page":"2454","DOI":"10.1007\/s00415-018-8983-8","article-title":"Amyotrophic lateral sclerosis: the complex path to precision medicine","volume":"265","author":"Talbot","year":"2018","journal-title":"J. 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Mutat."},{"key":"2019122311035355900_ref6","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1136\/jmg.2008.062463","article-title":"Contribution of TARDBP mutations to sporadic amyotrophic lateral sclerosis","volume":"46","author":"Daoud","year":"2009","journal-title":"J. Med. Genet."},{"key":"2019122311035355900_ref7","doi-asserted-by":"crossref","first-page":"572","DOI":"10.1038\/ng.132","article-title":"TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis","volume":"40","author":"Kabashi","year":"2008","journal-title":"Nat. Genet."},{"key":"2019122311035355900_ref8","first-page":"1668","article-title":"TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis","volume":"319","author":"Sreedharan","year":"2008","journal-title":"Sci. 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Neurochem."},{"key":"2019122311035355900_ref14","doi-asserted-by":"crossref","first-page":"1098","DOI":"10.1128\/MCB.01279-10","article-title":"TDP-43 is directed to stress granules by sorbitol, a novel physiological osmotic and oxidative stressor","volume":"31","author":"Dewey","year":"2011","journal-title":"Mol. Cell. Biol."},{"key":"2019122311035355900_ref15","doi-asserted-by":"crossref","first-page":"1400","DOI":"10.1093\/hmg\/ddr021","article-title":"TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1","volume":"20","author":"McDonald","year":"2011","journal-title":"Hum. Mol. 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