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PolyGR and polyPR knock-in mice reveal a conserved neuroprotective extracellular matrix signature in C9orf72 ALS/FTD neurons.

Autor: Milioto C; UK Dementia Research Institute, University College London, London, UK.; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK., Carcolé M; UK Dementia Research Institute, University College London, London, UK.; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK., Giblin A; UK Dementia Research Institute, University College London, London, UK.; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK.; UCL Institute of Healthy Ageing, Department of Genetics, Evolution and Environment, University College London, London, UK., Coneys R; UK Dementia Research Institute, University College London, London, UK.; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK., Attrebi O; UK Dementia Research Institute, University College London, London, UK.; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK., Ahmed M; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London, UK., Harris SS; UK Dementia Research Institute, University College London, London, UK., Lee BI; UK Dementia Research Institute, University College London, London, UK., Yang M; UK Dementia Research Institute, University College London, London, UK., Ellingford RA; UK Dementia Research Institute, University College London, London, UK., Nirujogi RS; Aligning Science Across Parkinson's (ASAP) Collaborative Research Network, Chevy Chase, MD, USA.; Medical Research Council (MRC) Protein Phosphorylation and Ubiquitylation Unit, School of Life Sciences, University of Dundee, Dundee, UK., Biggs D; Wellcome Centre for Human Genetics, University of Oxford, Oxford, UK., Salomonsson S; UK Dementia Research Institute, University College London, London, UK.; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK., Zanovello M; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London, UK., de Oliveira P; UK Dementia Research Institute, University College London, London, UK.; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK., Katona E; UK Dementia Research Institute, University College London, London, UK.; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK., Glaria I; UK Dementia Research Institute, University College London, London, UK.; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK.; Research Support Service, Institute of Agrobiotechnology, CSIC-Government of Navarra, Mutilva, Spain., Mikheenko A; UK Dementia Research Institute, University College London, London, UK.; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK.; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London, UK., Geary B; Aligning Science Across Parkinson's (ASAP) Collaborative Research Network, Chevy Chase, MD, USA.; Medical Research Council (MRC) Protein Phosphorylation and Ubiquitylation Unit, School of Life Sciences, University of Dundee, Dundee, UK., Udine E; Department of Neuroscience, Mayo Clinic, Jacksonville, FL, USA., Vaizoglu D; UK Dementia Research Institute, University College London, London, UK.; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK., Anoar S; UCL Institute of Healthy Ageing, Department of Genetics, Evolution and Environment, University College London, London, UK., Jotangiya K; UK Dementia Research Institute, University College London, London, UK.; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK., Crowley G; UK Dementia Research Institute, University College London, London, UK., Smeeth DM; UK Dementia Research Institute, University College London, London, UK.; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK., Adams ML; UK Dementia Research Institute, University College London, London, UK.; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK., Niccoli T; UCL Institute of Healthy Ageing, Department of Genetics, Evolution and Environment, University College London, London, UK., Rademakers R; VIB Center for Molecular Neurology, University of Antwerp, Antwerp, Belgium.; Department of Biomedical Sciences, University of Antwerp, Antwerp, Belgium., van Blitterswijk M; Department of Neuroscience, Mayo Clinic, Jacksonville, FL, USA., Devoy A; UK Dementia Research Institute, Maurice Wohl Clinical Neuroscience Institute, Institute of Psychiatry, Psychology and Neuroscience, King's College London, London, UK., Hong S; UK Dementia Research Institute, University College London, London, UK., Partridge L; UCL Institute of Healthy Ageing, Department of Genetics, Evolution and Environment, University College London, London, UK., Coyne AN; Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.; Brain Science Institute, Johns Hopkins University School of Medicine, Baltimore, MD, USA., Fratta P; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London, UK.; UCL Queen Square Motor Neuron Disease Centre, UCL Queen Square Institute of Neurology, London, UK., Alessi DR; Aligning Science Across Parkinson's (ASAP) Collaborative Research Network, Chevy Chase, MD, USA.; Medical Research Council (MRC) Protein Phosphorylation and Ubiquitylation Unit, School of Life Sciences, University of Dundee, Dundee, UK., Davies B; Wellcome Centre for Human Genetics, University of Oxford, Oxford, UK.; Francis Crick Institute, London, UK., Busche MA; UK Dementia Research Institute, University College London, London, UK., Greensmith L; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London, UK.; UCL Queen Square Motor Neuron Disease Centre, UCL Queen Square Institute of Neurology, London, UK., Fisher EMC; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London, UK. elizabeth.fisher@ucl.ac.uk.; UCL Queen Square Motor Neuron Disease Centre, UCL Queen Square Institute of Neurology, London, UK. elizabeth.fisher@ucl.ac.uk., Isaacs AM; UK Dementia Research Institute, University College London, London, UK. a.isaacs@ucl.ac.uk.; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK. a.isaacs@ucl.ac.uk.; UCL Queen Square Motor Neuron Disease Centre, UCL Queen Square Institute of Neurology, London, UK. a.isaacs@ucl.ac.uk.

Publikováno v: Nature neuroscience [Nat Neurosci] 2024 Apr; Vol. 27 (4), pp. 643-655. Date of Electronic Publication: 2024 Feb 29.