Cytoplasmic FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and inhibitory synaptic defects

Mutations in the RNA binding protein FUS are associated with ALS. Here the authors show that in FUS knock-in mice there is a progressive increase in neuronal activity in the frontal cortex which is associated with altered synaptic gene expression.

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Autores principales: Jelena Scekic-Zahirovic, Inmaculada Sanjuan-Ruiz, Vanessa Kan, Salim Megat, Pierre De Rossi, Stéphane Dieterlé, Raphaelle Cassel, Marguerite Jamet, Pascal Kessler, Diana Wiesner, Laura Tzeplaeff, Valérie Demais, Sonu Sahadevan, Katharina M. Hembach, Hans-Peter Muller, Gina Picchiarelli, Nibha Mishra, Stefano Antonucci, Sylvie Dirrig-Grosch, Jan Kassubek, Volker Rasche, Albert Ludolph, Anne-Laurence Boutillier, Francesco Roselli, Magdalini Polymenidou, Clotilde Lagier-Tourenne, Sabine Liebscher, Luc Dupuis
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Lenguaje:EN
Publicado: Nature Portfolio 2021
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Acceso en línea:https://doaj.org/article/81d53965ba1c4f5cbe4fa11bc13113fd
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spelling oai:doaj.org-article:81d53965ba1c4f5cbe4fa11bc13113fd2021-12-02T15:53:07ZCytoplasmic FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and inhibitory synaptic defects10.1038/s41467-021-23187-92041-1723https://doaj.org/article/81d53965ba1c4f5cbe4fa11bc13113fd2021-05-01T00:00:00Zhttps://doi.org/10.1038/s41467-021-23187-9https://doaj.org/toc/2041-1723Mutations in the RNA binding protein FUS are associated with ALS. Here the authors show that in FUS knock-in mice there is a progressive increase in neuronal activity in the frontal cortex which is associated with altered synaptic gene expression.Jelena Scekic-ZahirovicInmaculada Sanjuan-RuizVanessa KanSalim MegatPierre De RossiStéphane DieterléRaphaelle CasselMarguerite JametPascal KesslerDiana WiesnerLaura TzeplaeffValérie DemaisSonu SahadevanKatharina M. HembachHans-Peter MullerGina PicchiarelliNibha MishraStefano AntonucciSylvie Dirrig-GroschJan KassubekVolker RascheAlbert LudolphAnne-Laurence BoutillierFrancesco RoselliMagdalini PolymenidouClotilde Lagier-TourenneSabine LiebscherLuc DupuisNature PortfolioarticleScienceQENNature Communications, Vol 12, Iss 1, Pp 1-19 (2021)
institution DOAJ
collection DOAJ
language EN
topic Science
Q
spellingShingle Science
Q
Jelena Scekic-Zahirovic
Inmaculada Sanjuan-Ruiz
Vanessa Kan
Salim Megat
Pierre De Rossi
Stéphane Dieterlé
Raphaelle Cassel
Marguerite Jamet
Pascal Kessler
Diana Wiesner
Laura Tzeplaeff
Valérie Demais
Sonu Sahadevan
Katharina M. Hembach
Hans-Peter Muller
Gina Picchiarelli
Nibha Mishra
Stefano Antonucci
Sylvie Dirrig-Grosch
Jan Kassubek
Volker Rasche
Albert Ludolph
Anne-Laurence Boutillier
Francesco Roselli
Magdalini Polymenidou
Clotilde Lagier-Tourenne
Sabine Liebscher
Luc Dupuis
Cytoplasmic FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and inhibitory synaptic defects
description Mutations in the RNA binding protein FUS are associated with ALS. Here the authors show that in FUS knock-in mice there is a progressive increase in neuronal activity in the frontal cortex which is associated with altered synaptic gene expression.
format article
author Jelena Scekic-Zahirovic
Inmaculada Sanjuan-Ruiz
Vanessa Kan
Salim Megat
Pierre De Rossi
Stéphane Dieterlé
Raphaelle Cassel
Marguerite Jamet
Pascal Kessler
Diana Wiesner
Laura Tzeplaeff
Valérie Demais
Sonu Sahadevan
Katharina M. Hembach
Hans-Peter Muller
Gina Picchiarelli
Nibha Mishra
Stefano Antonucci
Sylvie Dirrig-Grosch
Jan Kassubek
Volker Rasche
Albert Ludolph
Anne-Laurence Boutillier
Francesco Roselli
Magdalini Polymenidou
Clotilde Lagier-Tourenne
Sabine Liebscher
Luc Dupuis
author_facet Jelena Scekic-Zahirovic
Inmaculada Sanjuan-Ruiz
Vanessa Kan
Salim Megat
Pierre De Rossi
Stéphane Dieterlé
Raphaelle Cassel
Marguerite Jamet
Pascal Kessler
Diana Wiesner
Laura Tzeplaeff
Valérie Demais
Sonu Sahadevan
Katharina M. Hembach
Hans-Peter Muller
Gina Picchiarelli
Nibha Mishra
Stefano Antonucci
Sylvie Dirrig-Grosch
Jan Kassubek
Volker Rasche
Albert Ludolph
Anne-Laurence Boutillier
Francesco Roselli
Magdalini Polymenidou
Clotilde Lagier-Tourenne
Sabine Liebscher
Luc Dupuis
author_sort Jelena Scekic-Zahirovic
title Cytoplasmic FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and inhibitory synaptic defects
title_short Cytoplasmic FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and inhibitory synaptic defects
title_full Cytoplasmic FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and inhibitory synaptic defects
title_fullStr Cytoplasmic FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and inhibitory synaptic defects
title_full_unstemmed Cytoplasmic FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and inhibitory synaptic defects
title_sort cytoplasmic fus triggers early behavioral alterations linked to cortical neuronal hyperactivity and inhibitory synaptic defects
publisher Nature Portfolio
publishDate 2021
url https://doaj.org/article/81d53965ba1c4f5cbe4fa11bc13113fd
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