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Neural Plasticity
Volume 2016, Article ID 6154080, 9 pages
http://dx.doi.org/10.1155/2016/6154080
Review Article

Developmental Dynamics of Rett Syndrome

1Picower Institute for Learning and Memory, Department of Brain and Cognitive Sciences, Massachusetts Institute of Technology, Cambridge, MA 02139, USA
2Laboratory of Neural Circuit Dynamics, Brain Research Institute, University of Zurich, 8057 Zurich, Switzerland

Received 27 September 2015; Revised 23 December 2015; Accepted 31 December 2015

Academic Editor: Graham Cocks

Copyright © 2016 Danielle Feldman et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Rett Syndrome was long considered to be simply a disorder of postnatal development, with phenotypes that manifest only late in development and into adulthood. A variety of recent evidence demonstrates that the phenotypes of Rett Syndrome are present at the earliest stages of brain development, including developmental stages that define neurogenesis, migration, and patterning in addition to stages of synaptic and circuit development and plasticity. These phenotypes arise from the pleotropic effects of MeCP2, which is expressed very early in neuronal progenitors and continues to be expressed into adulthood. The effects of MeCP2 are mediated by diverse signaling, transcriptional, and epigenetic mechanisms. Attempts to reverse the effects of Rett Syndrome need to take into account the developmental dynamics and temporal impact of MeCP2 loss.