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PolyGA targets the ER stress-adaptive response by impairing GRP75 function at the MAM in C9ORF72-ALS/FTD.

Autor: Pilotto F; Department of Neurology, Inselspital, University Hospital, Bern, Switzerland.; Department for BioMedical Research, University of Bern, Bern, Switzerland., Schmitz A; Department of Neurology, Inselspital, University Hospital, Bern, Switzerland.; Department for BioMedical Research, University of Bern, Bern, Switzerland., Maharjan N; Department of Neurology, Inselspital, University Hospital, Bern, Switzerland.; Department for BioMedical Research, University of Bern, Bern, Switzerland., Diab R; Department of Neurology, Inselspital, University Hospital, Bern, Switzerland.; Department for BioMedical Research, University of Bern, Bern, Switzerland., Odriozola A; Institute of Anatomy, University of Bern, Bern, Switzerland., Tripathi P; Institute of Neuropathology, RWTH Aachen University Hospital, Aachen, Germany., Yamoah A; Institute of Neuropathology, RWTH Aachen University Hospital, Aachen, Germany., Scheidegger O; Department of Neurology, Inselspital, University Hospital, Bern, Switzerland., Oestmann A; Department of Neurology, Inselspital, University Hospital, Bern, Switzerland.; Department for BioMedical Research, University of Bern, Bern, Switzerland., Dennys CN; Center for Gene Therapy, The Research Institute at Nationwide Children's Hospital, Columbus, OH, USA., Sinha Ray S; Center for Gene Therapy, The Research Institute at Nationwide Children's Hospital, Columbus, OH, USA., Rodrigo R; Center for Gene Therapy, The Research Institute at Nationwide Children's Hospital, Columbus, OH, USA., Kolb S; Department of Neurology and Biological Chemistry and Pharmacology, The Ohio State University, Columbus, OH, USA., Aronica E; Department of (Neuro) Pathology, Amsterdam UMC, University of Amsterdam, Amsterdam Neuroscience, Amsterdam, The Netherlands., Di Santo S; Department of Neurosurgery, Inselspital, University Hospital, Bern, Switzerland., Widmer HR; Department of Neurosurgery, Inselspital, University Hospital, Bern, Switzerland., Charlet-Berguerand N; CNRS, Inserm, IGBMC UMR 7104, U 1258, Université de Strasbourg, F-67404, Illkirch, France., Selvaraj BT; UK Dementia Research Institute at University of Edinburgh, University of Edinburgh, Edinburgh, EH16 4SB, UK.; Euan MacDonald Centre for MND Research, University of Edinburgh, Edinburgh, EH16 4SB, UK.; Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, EH16 4SB, UK., Chandran S; UK Dementia Research Institute at University of Edinburgh, University of Edinburgh, Edinburgh, EH16 4SB, UK.; Euan MacDonald Centre for MND Research, University of Edinburgh, Edinburgh, EH16 4SB, UK.; Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, EH16 4SB, UK., Meyer K; Center for Gene Therapy, The Research Institute at Nationwide Children's Hospital, Columbus, OH, USA.; Department of Pediatrics, The Ohio State University, Columbus, OH, USA., Zuber B; Institute of Anatomy, University of Bern, Bern, Switzerland., Goswami A; Institute of Neuropathology, RWTH Aachen University Hospital, Aachen, Germany., Weis J; Institute of Neuropathology, RWTH Aachen University Hospital, Aachen, Germany., Saxena S; Department of Neurology, Inselspital, University Hospital, Bern, Switzerland. smita.saxena@dbmr.unibe.ch.; Department for BioMedical Research, University of Bern, Bern, Switzerland. smita.saxena@dbmr.unibe.ch.

Publikováno v: Acta neuropathologica [Acta Neuropathol] 2022 Nov; Vol. 144 (5), pp. 939-966. Date of Electronic Publication: 2022 Sep 19.