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
Description
ISSN:2051-5960
DOI:10.1186/s40478-026-02301-2