Targeting the integrated stress response or Ataxin-2 alleviates neurodegeneration in PolyGR models of C9orf72 associated frontotemporal dementia and amyotrophic lateral sclerosis.

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Title: Targeting the integrated stress response or Ataxin-2 alleviates neurodegeneration in PolyGR models of C9orf72 associated frontotemporal dementia and amyotrophic lateral sclerosis.
Authors: Harper NS; Division of Neuroscience and Experimental Psychology, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK., Sharpe JL; Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, 385a Glossop Road, Sheffield, S10 2HQ, UK.; Neuroscience Institute, University of Sheffield, Sheffield, S10 2TN, UK., Speranza J; Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, 385a Glossop Road, Sheffield, S10 2HQ, UK.; Neuroscience Institute, University of Sheffield, Sheffield, S10 2TN, UK., Gulia R; Department of Pathology and Laboratory Medicine, University of California, Irvine, Irvine, CA, 92617, USA., Chen JX; Department of Pathology and Laboratory Medicine, University of California, Irvine, Irvine, CA, 92617, USA., Allen SP; Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, 385a Glossop Road, Sheffield, S10 2HQ, UK.; Neuroscience Institute, University of Sheffield, Sheffield, S10 2TN, UK., Atwal MS; Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, 385a Glossop Road, Sheffield, S10 2HQ, UK.; Neuroscience Institute, University of Sheffield, Sheffield, S10 2TN, UK., Pickering-Brown S; Division of Neuroscience and Experimental Psychology, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK., Livesey MR; Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, 385a Glossop Road, Sheffield, S10 2HQ, UK.; Neuroscience Institute, University of Sheffield, Sheffield, S10 2TN, UK., Bennett CL; Department of Pathology and Laboratory Medicine, University of California, Irvine, Irvine, CA, 92617, USA.; UCI Center for Neurotherapeutics, University of California, Irvine, Irvine, CA, 92697, USA., Prokop A; Division of Molecular and Cellular Function, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, UK., La Spada AR; Department of Pathology and Laboratory Medicine, University of California, Irvine, Irvine, CA, 92617, USA.; UCI Center for Neurotherapeutics, University of California, Irvine, Irvine, CA, 92697, USA.; Department of Biological Chemistry, University of California, Irvine, Irvine, CA, 92617, USA.; Department of Neurology, University of California, Irvine, Irvine, CA, 92617, USA.; Department of Neurobiology and Behavior, University of California, Irvine, Irvine, CA, 92697, USA., West RJH; Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, 385a Glossop Road, Sheffield, S10 2HQ, UK. r.j.west@sheffield.ac.uk.; Neuroscience Institute, University of Sheffield, Sheffield, S10 2TN, UK. r.j.west@sheffield.ac.uk.
Source: Acta neuropathologica communications [Acta Neuropathol Commun] 2026 May 05; Vol. 14 (1). Date of Electronic Publication: 2026 May 05.
Publication Type: Journal Article
Journal Info: Publisher: BioMed Central Country of Publication: England NLM ID: 101610673 Publication Model: Electronic Cited Medium: Internet ISSN: 2051-5960 (Electronic) Linking ISSN: 20515960 NLM ISO Abbreviation: Acta Neuropathol Commun Subsets: MEDLINE
Database: MEDLINE Ultimate
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  Data: Targeting the integrated stress response or Ataxin-2 alleviates neurodegeneration in PolyGR models of C9orf72 associated frontotemporal dementia and amyotrophic lateral sclerosis.
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  Data: <searchLink fieldCode="AU" term="%22Harper+NS%22">Harper NS</searchLink>; Division of Neuroscience and Experimental Psychology, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK.<br /><searchLink fieldCode="AU" term="%22Sharpe+JL%22">Sharpe JL</searchLink>; Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, 385a Glossop Road, Sheffield, S10 2HQ, UK.; Neuroscience Institute, University of Sheffield, Sheffield, S10 2TN, UK.<br /><searchLink fieldCode="AU" term="%22Speranza+J%22">Speranza J</searchLink>; Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, 385a Glossop Road, Sheffield, S10 2HQ, UK.; Neuroscience Institute, University of Sheffield, Sheffield, S10 2TN, UK.<br /><searchLink fieldCode="AU" term="%22Gulia+R%22">Gulia R</searchLink>; Department of Pathology and Laboratory Medicine, University of California, Irvine, Irvine, CA, 92617, USA.<br /><searchLink fieldCode="AU" term="%22Chen+JX%22">Chen JX</searchLink>; Department of Pathology and Laboratory Medicine, University of California, Irvine, Irvine, CA, 92617, USA.<br /><searchLink fieldCode="AU" term="%22Allen+SP%22">Allen SP</searchLink>; Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, 385a Glossop Road, Sheffield, S10 2HQ, UK.; Neuroscience Institute, University of Sheffield, Sheffield, S10 2TN, UK.<br /><searchLink fieldCode="AU" term="%22Atwal+MS%22">Atwal MS</searchLink>; Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, 385a Glossop Road, Sheffield, S10 2HQ, UK.; Neuroscience Institute, University of Sheffield, Sheffield, S10 2TN, UK.<br /><searchLink fieldCode="AU" term="%22Pickering-Brown+S%22">Pickering-Brown S</searchLink>; Division of Neuroscience and Experimental Psychology, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK.<br /><searchLink fieldCode="AU" term="%22Livesey+MR%22">Livesey MR</searchLink>; Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, 385a Glossop Road, Sheffield, S10 2HQ, UK.; Neuroscience Institute, University of Sheffield, Sheffield, S10 2TN, UK.<br /><searchLink fieldCode="AU" term="%22Bennett+CL%22">Bennett CL</searchLink>; Department of Pathology and Laboratory Medicine, University of California, Irvine, Irvine, CA, 92617, USA.; UCI Center for Neurotherapeutics, University of California, Irvine, Irvine, CA, 92697, USA.<br /><searchLink fieldCode="AU" term="%22Prokop+A%22">Prokop A</searchLink>; Division of Molecular and Cellular Function, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, UK.<br /><searchLink fieldCode="AU" term="%22La+Spada+AR%22">La Spada AR</searchLink>; Department of Pathology and Laboratory Medicine, University of California, Irvine, Irvine, CA, 92617, USA.; UCI Center for Neurotherapeutics, University of California, Irvine, Irvine, CA, 92697, USA.; Department of Biological Chemistry, University of California, Irvine, Irvine, CA, 92617, USA.; Department of Neurology, University of California, Irvine, Irvine, CA, 92617, USA.; Department of Neurobiology and Behavior, University of California, Irvine, Irvine, CA, 92697, USA.<br /><searchLink fieldCode="AU" term="%22West+RJH%22">West RJH</searchLink>; Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, 385a Glossop Road, Sheffield, S10 2HQ, UK. r.j.west@sheffield.ac.uk.; Neuroscience Institute, University of Sheffield, Sheffield, S10 2TN, UK. r.j.west@sheffield.ac.uk.
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  Data: <searchLink fieldCode="JN" term="%22101610673%22">Acta neuropathologica communications</searchLink> [Acta Neuropathol Commun] 2026 May 05; Vol. 14 (1). <i>Date of Electronic Publication: </i>2026 May 05.
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