{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Hulsmann S"],"funding":["Deutsche Forschungsgemeinschaft"],"pagination":["385"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC5018520"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["7"],"pubmed_abstract":["Mutations in methyl-CpG-binding protein 2 (MECP2) gene have been shown to manifest in a neurodevelopmental disorder that is called Rett syndrome. A typical problem that occurs during development is a disturbance of breathing. To address the role of inhibitory neurons, we generated a mouse line that restores MECP2 in inhibitory neurons in the brainstem by crossbreeding a mouse line that expresses the Cre-recombinase (Cre) in inhibitory neurons under the control of the glycine transporter 2 (GlyT2, slc6a5) promotor (GlyT2-Cre) with a mouse line that has a floxed-stop mutation of the Mecp2 gene (Mecp2 (stop/y)). Unrestrained whole-body-plethysmography at postnatal day P60 revealed a low respiratory rate and prolonged respiratory pauses in Mecp2 (stop/y) mice. In contrast, GlyT2-Cre positive M"],"journal":["Frontiers in physiology"],"pubmed_title":["GlyT2-Dependent Preservation of MECP2-Expression in Inhibitory Neurons Improves Early Respiratory Symptoms but Does Not Rescue Survival in a Mouse Model of Rett Syndrome."],"pmcid":["PMC5018520"],"funding_grant_id":["Hu797/8-1"],"pubmed_authors":["Niebert M","Dannenberg J","Arnoldt M","Mesuret G","Hulsmann S"],"additional_accession":[]},"is_claimable":false,"name":"GlyT2-Dependent Preservation of MECP2-Expression in Inhibitory Neurons Improves Early Respiratory Symptoms but Does Not Rescue Survival in a Mouse Model of Rett Syndrome.","description":"Mutations in methyl-CpG-binding protein 2 (MECP2) gene have been shown to manifest in a neurodevelopmental disorder that is called Rett syndrome. A typical problem that occurs during development is a disturbance of breathing. To address the role of inhibitory neurons, we generated a mouse line that restores MECP2 in inhibitory neurons in the brainstem by crossbreeding a mouse line that expresses the Cre-recombinase (Cre) in inhibitory neurons under the control of the glycine transporter 2 (GlyT2, slc6a5) promotor (GlyT2-Cre) with a mouse line that has a floxed-stop mutation of the Mecp2 gene (Mecp2 (stop/y)). Unrestrained whole-body-plethysmography at postnatal day P60 revealed a low respiratory rate and prolonged respiratory pauses in Mecp2 (stop/y) mice. In contrast, GlyT2-Cre positive M","dates":{"release":"2016-01-01T00:00:00Z","publication":"2016","modification":"2025-04-04T09:22:18.073Z","creation":"2019-03-27T02:23:55Z"},"accession":"S-EPMC5018520","cross_references":{"pubmed":["27672368"],"doi":["10.3389/fphys.2016.00385"]}}