Treating early postnatal circuit defect delays Huntington’s disease onset and pathology in mice
| العنوان: | Treating early postnatal circuit defect delays Huntington’s disease onset and pathology in mice |
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| المؤلفون: | Barbara Yael Braz, Doris Wennagel, Leslie Ratié, Diego Alves Rodrigues de Souza, Jean Christophe Deloulme, Emmanuel L. Barbier, Alain Buisson, Fabien Lanté, Sandrine Humbert |
| المساهمون: | Université Grenoble Alpes (UGA), [GIN] Grenoble Institut des Neurosciences (GIN), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Grenoble Alpes (UGA), Institut du Cerveau = Paris Brain Institute (ICM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Barbier, Emmanuel |
| المصدر: | Science Science, 2022, 377 (6613), pp.eabq5011. ⟨10.1126/science.abq5011⟩ |
| بيانات النشر: | American Association for the Advancement of Science (AAAS), 2022. |
| سنة النشر: | 2022 |
| مصطلحات موضوعية: | Huntingtin Protein, Multidisciplinary, [SDV.NEU.NB]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology, [SCCO.NEUR]Cognitive science/Neuroscience, Neurogenesis, [SCCO.NEUR] Cognitive science/Neuroscience, [SDV.NEU.NB] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology, Brain, Mice, Transgenic, Disease Models, Animal, Mice, Huntington Disease, Synapses, Animals, Humans, Nerve Net |
| الوصف: | International audience; Recent evidence has shown that even mild mutations in the Huntingtin gene that are associated with late-onset Huntington’s disease (HD) disrupt various aspects of human neurodevelopment. To determine whether these seemingly subtle early defects affect adult neural function, we investigated neural circuit physiology in newborn HD mice. During the first postnatal week, HD mice have less cortical layer 2/3 excitatory synaptic activity than wild-type mice, express fewer glutamatergic receptors, and show sensorimotor deficits. The circuit self-normalizes in the second postnatal week but the mice nonetheless develop HD. Pharmacologically enhancing glutamatergic transmission during the neonatal period, however, rescues these deficits and preserves sensorimotor function, cognition, and spine and synapse density as well as brain region volume in HD adult mice. |
| تدمد: | 1095-9203 0036-8075 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::1c6147460666730e4b2585e2f311226c https://doi.org/10.1126/science.abq5011 |
| رقم الأكسشن: | edsair.doi.dedup.....1c6147460666730e4b2585e2f311226c |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 10959203 00368075 |
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