Early alterations in a mouse model of Rett syndrome: the GABA developmental shift is abolished at birth
| Autor: | M. Billon-Grand, Jean-Luc Gaiarsa, Damien Guimond, Sanaz Eftekhari, Roman Tyzio, Paolo Bonifazi, Yehezkel Ben-Ari, Natalia Lozovaya, Alexandre Pons-Bennaceur, Javier Rasero, T.-T. Bui, Diana C. Ferrari, Morgane Chiesa, Romain Nardou |
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| Přispěvatelé: | Gaiarsa, Jean-Luc, APPEL À PROJETS GÉNÉRIQUE 2018 - Invagination et migration des épithéliums - - Migrafolds2018 - ANR-18-CE13-0021 - AAPG2018 - VALID, Neurochlore (Ben-Ari Institute of Neuroarcheology), Institut de Neurobiologie de la Méditerranée [Aix-Marseille Université] (INMED - INSERM U1249), Institut National de la Santé et de la Recherche Médicale (INSERM)-Aix Marseille Université (AMU), Biocruces Bizkaia Health Research Institute [Baracaldo], ANR-18-CE13-0021,Migrafolds,Invagination et migration des épithéliums(2018) |
| Jazyk: | angličtina |
| Rok vydání: | 2019 |
| Předmět: |
0301 basic medicine
medicine.medical_specialty congenital hereditary and neonatal diseases and abnormalities Methyl-CpG-Binding Protein 2 [SDV.NEU.NB]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology Respiratory System lcsh:Medicine Rett syndrome Biology Synaptic Transmission Article MECP2 03 medical and health sciences Glutamatergic Mice 0302 clinical medicine Sodium Potassium Chloride Symporter Inhibitors Internal medicine medicine Rett Syndrome Premovement neuronal activity Animals lcsh:Science Bumetanide gamma-Aminobutyric Acid Mice Knockout Neurons Multidisciplinary Neuronal Plasticity lcsh:R [SDV.NEU.NB] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology [SDV.BDD.EO] Life Sciences [q-bio]/Development Biology/Embryology and Organogenesis Synaptic Potentials medicine.disease Receptors GABA-A 3. Good health Mice Inbred C57BL 030104 developmental biology Endocrinology Metabotropic receptor [SDV.BDD.EO]Life Sciences [q-bio]/Development Biology/Embryology and Organogenesis Excitatory postsynaptic potential GABAergic lcsh:Q 030217 neurology & neurosurgery Neurological disorders medicine.drug Neuroscience |
| Zdroj: | Scientific Reports Scientific Reports, Nature Publishing Group, 2019, 9, pp.9276. ⟨10.1038/s41598-019-45635-9⟩ Scientific Reports, Vol 9, Iss 1, Pp 1-16 (2019) |
| ISSN: | 2045-2322 |
| DOI: | 10.1038/s41598-019-45635-9 |
| Popis: | Genetic mutations of the Methyl-CpG-binding protein-2 (MECP2) gene underlie Rett syndrome (RTT). Developmental processes are often considered to be irrelevant in RTT pathogenesis but neuronal activity at birth has not been recorded. We report that the GABA developmental shift at birth is abolished in CA3 pyramidal neurons of Mecp2−/y mice and the glutamatergic/GABAergic postsynaptic currents (PSCs) ratio is increased. Two weeks later, GABA exerts strong excitatory actions, the glutamatergic/GABAergic PSCs ratio is enhanced, hyper-synchronized activity is present and metabotropic long-term depression (LTD) is impacted. One day before delivery, maternal administration of the NKCC1 chloride importer antagonist bumetanide restored these parameters but not respiratory or weight deficits, nor the onset of mortality. Results suggest that birth is a critical period in RTT with important alterations that can be attenuated by bumetanide raising the possibility of early treatment of the disorder. |
| Databáze: | OpenAIRE |
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