Rett Syndrome: Genes, Synapses, Circuits, and Therapeutics

Development of the nervous system proceeds through a set of complex checkpoints which arise from a combination of sequential gene expression and early neural activity sculpted by the environment. Genetic and environmental insults lead to neurodevelopmental disorders which encompass a large group of...

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Main Authors: Banerjee, Abhishek, Castro, Jorge, Sur, Mriganka
Other Authors: Massachusetts Institute of Technology. Department of Brain and Cognitive Sciences
Format: Article
Language:en_US
Published: Frontiers Research Foundation 2016
Online Access:http://hdl.handle.net/1721.1/102528
https://orcid.org/0000-0003-2442-5671
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author Banerjee, Abhishek
Castro, Jorge
Sur, Mriganka
author2 Massachusetts Institute of Technology. Department of Brain and Cognitive Sciences
author_facet Massachusetts Institute of Technology. Department of Brain and Cognitive Sciences
Banerjee, Abhishek
Castro, Jorge
Sur, Mriganka
author_sort Banerjee, Abhishek
collection MIT
description Development of the nervous system proceeds through a set of complex checkpoints which arise from a combination of sequential gene expression and early neural activity sculpted by the environment. Genetic and environmental insults lead to neurodevelopmental disorders which encompass a large group of diseases that result from anatomical and physiological abnormalities during maturation and development of brain circuits. Rett syndrome (RTT) is a neurological disorder of genetic origin, caused by mutations in the X-linked gene methyl-CpG binding protein 2 (MeCP2). It features a range of neuropsychiatric abnormalities including motor dysfunctions and mild to severe cognitive impairment. Here, we discuss key questions and recent studies describing animal models, cell-type specific functions of methyl-CpG binding protein 2 (MeCP2), defects in neural circuit plasticity, and attempts to evaluate possible therapeutic strategies for RTT. We also discuss how genes, proteins, and overlapping signaling pathways affect the molecular etiology of apparently unrelated neuropsychiatric disorders, an understanding of which can offer novel therapeutic strategies for a range of autism spectrum disorders (ASDs).
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spelling mit-1721.1/1025282022-10-01T01:03:55Z Rett Syndrome: Genes, Synapses, Circuits, and Therapeutics Banerjee, Abhishek Castro, Jorge Sur, Mriganka Massachusetts Institute of Technology. Department of Brain and Cognitive Sciences Picower Institute for Learning and Memory Banerjee, Abhishek Castro, Jorge Sur, Mriganka Development of the nervous system proceeds through a set of complex checkpoints which arise from a combination of sequential gene expression and early neural activity sculpted by the environment. Genetic and environmental insults lead to neurodevelopmental disorders which encompass a large group of diseases that result from anatomical and physiological abnormalities during maturation and development of brain circuits. Rett syndrome (RTT) is a neurological disorder of genetic origin, caused by mutations in the X-linked gene methyl-CpG binding protein 2 (MeCP2). It features a range of neuropsychiatric abnormalities including motor dysfunctions and mild to severe cognitive impairment. Here, we discuss key questions and recent studies describing animal models, cell-type specific functions of methyl-CpG binding protein 2 (MeCP2), defects in neural circuit plasticity, and attempts to evaluate possible therapeutic strategies for RTT. We also discuss how genes, proteins, and overlapping signaling pathways affect the molecular etiology of apparently unrelated neuropsychiatric disorders, an understanding of which can offer novel therapeutic strategies for a range of autism spectrum disorders (ASDs). Simons Foundation (Postdoctoral Fellowship) National Institutes of Health (U.S.) 2016-05-18T16:44:12Z 2016-05-18T16:44:12Z 2012-05 Article http://purl.org/eprint/type/JournalArticle 1664-0640 http://hdl.handle.net/1721.1/102528 Banerjee, Abhishek, Jorge Castro, and Mriganka Sur. “Rett Syndrome: Genes, Synapses, Circuits, and Therapeutics.” Front. Psychiatry 3 (2012). https://orcid.org/0000-0003-2442-5671 en_US http://dx.doi.org/10.3389/fpsyt.2012.00034 Frontiers in Psychiatry Creative Commons Attribution 3.0 Unported licence http://creativecommons.org/licenses/by-nc/3.0/ application/pdf Frontiers Research Foundation Frontiers in Psychiatry
spellingShingle Banerjee, Abhishek
Castro, Jorge
Sur, Mriganka
Rett Syndrome: Genes, Synapses, Circuits, and Therapeutics
title Rett Syndrome: Genes, Synapses, Circuits, and Therapeutics
title_full Rett Syndrome: Genes, Synapses, Circuits, and Therapeutics
title_fullStr Rett Syndrome: Genes, Synapses, Circuits, and Therapeutics
title_full_unstemmed Rett Syndrome: Genes, Synapses, Circuits, and Therapeutics
title_short Rett Syndrome: Genes, Synapses, Circuits, and Therapeutics
title_sort rett syndrome genes synapses circuits and therapeutics
url http://hdl.handle.net/1721.1/102528
https://orcid.org/0000-0003-2442-5671
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