Research ArticleRett Syndrome

mGlu7 potentiation rescues cognitive, social, and respiratory phenotypes in a mouse model of Rett syndrome

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Science Translational Medicine  16 Aug 2017:
Vol. 9, Issue 403, eaai7459
DOI: 10.1126/scitranslmed.aai7459

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A positive approach to Rett syndrome

Rett syndrome is an untreatable neurodevelopmental disorder. Gogliotti et al. report a decrease in metabotropic glutamate receptor 7 (mGlu7) expression in brain autopsy samples from patients with Rett syndrome. The authors used in vitro electrophysiological and in vivo pharmacological and behavioral analyses to test whether restoring mGlu7 ameliorated deficits in two Rett syndrome mouse models. mGlu7 reduction disrupted synaptic plasticity in the hippocampus; positive modulation of mGlu7 activity restored synaptic plasticity and reduced cognitive impairments and apneas in the mice. These findings suggest that mGlu7 might be a useful therapeutic target for treating Rett syndrome.