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Cytoplasmic accumulation of FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and defects in inhibitory synapses

Jelena Scekic-Zahirovic, Inmaculada Sanjuan-Ruiz, Vanessa Kan, Salim Megat, Pierre De Rossi, Stéphane Dieterlé, Raphaelle Cassel, Pascal Kessler, Diana Wiesner, Laura Tzeplaeff, Valérie Demais, Hans-Peter Muller, Gina Picchiarelli, Nibha Mishra, Sylvie Dirrig-Grosch, Jan Kassubek, Volker Rasche, Albert Ludolph, Anne-Laurence Boutillier, Magdalini Polymenidou, Clotilde Lagier-Tourenne, Sabine Liebscher, Luc Dupuis
doi: https://doi.org/10.1101/2020.06.09.141556
Jelena Scekic-Zahirovic
1Université de Strasbourg, Inserm, Mécanismes centraux et périphériques de la neurodégénérescence, UMR-S1118, Strasbourg, France
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Inmaculada Sanjuan-Ruiz
1Université de Strasbourg, Inserm, Mécanismes centraux et périphériques de la neurodégénérescence, UMR-S1118, Strasbourg, France
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Vanessa Kan
2Institute of Clinical Neuroimmunology, Klinikum der Universität München, Ludwig-Maximilians-University Munich, Munich. Germany
3BioMedical Center, Medical Faculty, Ludwig-Maximilians-University Munich, Munich, Germany
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Salim Megat
1Université de Strasbourg, Inserm, Mécanismes centraux et périphériques de la neurodégénérescence, UMR-S1118, Strasbourg, France
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Pierre De Rossi
4Department of Quantitative Biomedicine, University of Zurich
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Stéphane Dieterlé
1Université de Strasbourg, Inserm, Mécanismes centraux et périphériques de la neurodégénérescence, UMR-S1118, Strasbourg, France
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Raphaelle Cassel
1Université de Strasbourg, Inserm, Mécanismes centraux et périphériques de la neurodégénérescence, UMR-S1118, Strasbourg, France
5Université de Strasbourg, UMR 7364 CNRS, Laboratoire de Neurosciences Cognitives et Adaptatives (LNCA), Strasbourg, France
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Pascal Kessler
6Université de Strasbourg, Inserm, Unité mixte de service du CRBS, UMS 038, Strasbourg, France
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Diana Wiesner
7Department of Neurology, University of Ulm
8Deutsches Zentrum für Neurodegenerative Erkrankungen, Ulm
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Laura Tzeplaeff
5Université de Strasbourg, UMR 7364 CNRS, Laboratoire de Neurosciences Cognitives et Adaptatives (LNCA), Strasbourg, France
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Valérie Demais
9Plateforme Imagerie In Vitro, CNRS UPS-3156, NeuroPôle, Strasbourg, France
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Hans-Peter Muller
7Department of Neurology, University of Ulm
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Gina Picchiarelli
1Université de Strasbourg, Inserm, Mécanismes centraux et périphériques de la neurodégénérescence, UMR-S1118, Strasbourg, France
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Nibha Mishra
10Department of Neurology, The Sean M. Healey and AMG Center for ALS at Mass General, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA
11Broad Institute of Harvard University and MIT, Cambridge, MA, USA
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Sylvie Dirrig-Grosch
1Université de Strasbourg, Inserm, Mécanismes centraux et périphériques de la neurodégénérescence, UMR-S1118, Strasbourg, France
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Jan Kassubek
7Department of Neurology, University of Ulm
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Volker Rasche
7Department of Neurology, University of Ulm
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Albert Ludolph
7Department of Neurology, University of Ulm
8Deutsches Zentrum für Neurodegenerative Erkrankungen, Ulm
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Anne-Laurence Boutillier
5Université de Strasbourg, UMR 7364 CNRS, Laboratoire de Neurosciences Cognitives et Adaptatives (LNCA), Strasbourg, France
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Magdalini Polymenidou
4Department of Quantitative Biomedicine, University of Zurich
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Clotilde Lagier-Tourenne
10Department of Neurology, The Sean M. Healey and AMG Center for ALS at Mass General, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA
11Broad Institute of Harvard University and MIT, Cambridge, MA, USA
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Sabine Liebscher
2Institute of Clinical Neuroimmunology, Klinikum der Universität München, Ludwig-Maximilians-University Munich, Munich. Germany
3BioMedical Center, Medical Faculty, Ludwig-Maximilians-University Munich, Munich, Germany
12Munich Cluster for Systems Neurology (SyNergy), Munich, Germany
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  • For correspondence: ldupuis@unistra.fr sabine.liebscher@med.uni-muenchen.de
Luc Dupuis
1Université de Strasbourg, Inserm, Mécanismes centraux et périphériques de la neurodégénérescence, UMR-S1118, Strasbourg, France
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  • For correspondence: ldupuis@unistra.fr sabine.liebscher@med.uni-muenchen.de
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Article Information

doi 
https://doi.org/10.1101/2020.06.09.141556
History 
  • June 11, 2020.
Copyright 
The copyright holder for this preprint is the author/funder, who has granted bioRxiv a license to display the preprint in perpetuity. It is made available under a CC-BY-NC-ND 4.0 International license.

Author Information

  1. Jelena Scekic-Zahirovic1,*,
  2. Inmaculada Sanjuan-Ruiz1,*,
  3. Vanessa Kan2,3,
  4. Salim Megat1,
  5. Pierre De Rossi4,
  6. Stéphane Dieterlé1,
  7. Raphaelle Cassel1,5,
  8. Pascal Kessler6,
  9. Diana Wiesner7,8,
  10. Laura Tzeplaeff5,
  11. Valérie Demais9,
  12. Hans-Peter Muller7,
  13. Gina Picchiarelli1,
  14. Nibha Mishra10,11,
  15. Sylvie Dirrig-Grosch1,
  16. Jan Kassubek7,
  17. Volker Rasche7,
  18. Albert Ludolph7,8,
  19. Anne-Laurence Boutillier5,
  20. Magdalini Polymenidou4,
  21. Clotilde Lagier-Tourenne10,11,
  22. Sabine Liebscher2,3,12,# and
  23. Luc Dupuis1,#
  1. 1Université de Strasbourg, Inserm, Mécanismes centraux et périphériques de la neurodégénérescence, UMR-S1118, Strasbourg, France
  2. 2Institute of Clinical Neuroimmunology, Klinikum der Universität München, Ludwig-Maximilians-University Munich, Munich. Germany
  3. 3BioMedical Center, Medical Faculty, Ludwig-Maximilians-University Munich, Munich, Germany
  4. 4Department of Quantitative Biomedicine, University of Zurich
  5. 5Université de Strasbourg, UMR 7364 CNRS, Laboratoire de Neurosciences Cognitives et Adaptatives (LNCA), Strasbourg, France
  6. 6Université de Strasbourg, Inserm, Unité mixte de service du CRBS, UMS 038, Strasbourg, France
  7. 7Department of Neurology, University of Ulm
  8. 8Deutsches Zentrum für Neurodegenerative Erkrankungen, Ulm
  9. 9Plateforme Imagerie In Vitro, CNRS UPS-3156, NeuroPôle, Strasbourg, France
  10. 10Department of Neurology, The Sean M. Healey and AMG Center for ALS at Mass General, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA
  11. 11Broad Institute of Harvard University and MIT, Cambridge, MA, USA
  12. 12Munich Cluster for Systems Neurology (SyNergy), Munich, Germany
  1. ↵#Correspondance: Luc DUPUIS, Université de Strasbourg, Inserm, Mécanismes centraux et périphériques de la neurodégénérescence, UMR-S1118, Faculté de médecine, 11 rue Humann, 67085 Strasbourg, Cédex, France, e-mail : ldupuis{at}unistra.fr, Sabine LIEBSCHER: Institute of Clinical Neuroimmunology, Klinikum der Universität München, BioMedical Center, Grosshaderner Str. 9, 82152 Martinsried, e-mail: sabine.liebscher{at}med.uni-muenchen.de
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Cytoplasmic accumulation of FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and defects in inhibitory synapses
Jelena Scekic-Zahirovic, Inmaculada Sanjuan-Ruiz, Vanessa Kan, Salim Megat, Pierre De Rossi, Stéphane Dieterlé, Raphaelle Cassel, Pascal Kessler, Diana Wiesner, Laura Tzeplaeff, Valérie Demais, Hans-Peter Muller, Gina Picchiarelli, Nibha Mishra, Sylvie Dirrig-Grosch, Jan Kassubek, Volker Rasche, Albert Ludolph, Anne-Laurence Boutillier, Magdalini Polymenidou, Clotilde Lagier-Tourenne, Sabine Liebscher, Luc Dupuis
bioRxiv 2020.06.09.141556; doi: https://doi.org/10.1101/2020.06.09.141556
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Cytoplasmic accumulation of FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and defects in inhibitory synapses
Jelena Scekic-Zahirovic, Inmaculada Sanjuan-Ruiz, Vanessa Kan, Salim Megat, Pierre De Rossi, Stéphane Dieterlé, Raphaelle Cassel, Pascal Kessler, Diana Wiesner, Laura Tzeplaeff, Valérie Demais, Hans-Peter Muller, Gina Picchiarelli, Nibha Mishra, Sylvie Dirrig-Grosch, Jan Kassubek, Volker Rasche, Albert Ludolph, Anne-Laurence Boutillier, Magdalini Polymenidou, Clotilde Lagier-Tourenne, Sabine Liebscher, Luc Dupuis
bioRxiv 2020.06.09.141556; doi: https://doi.org/10.1101/2020.06.09.141556

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