Abstract

Many neurological disorders, including neurodevelopmental disorders, report hypersynchrony of neuronal networks. These alterations in neuronal synchronization suggest a link to the function of inhibitory interneurons. In Fragile X Syndrome (FXS), it has been reported that altered synchronization may underlie hyperexcitability, cognitive dysfunction and provide a link to the increased incidence of epileptic seizures. Therefore, understanding the roles of inhibitory interneurons and how they control neuronal networks is of great importance in studying neurodevelopmental disorders such as FXS. Here, we present a review of how interneuron populations and inhibition are important contributors to the loss of excitatory/inhibitory balance seen in hypersynchronous and hyperexcitable networks from neurodevelopmental disorders, and specifically in FXS.

Highlights

  • Fragile X Syndrome (FXS) is one of several disorders associated with autism spectrum disorders (ASDs)—a heterogeneous group of behaviorally identified neurodevelopmental disabilities

  • FXS is attributed to the transcriptional silencing of the Fragile X Mental Retardation 1 (FMR1) gene and the consequent loss of the gene product of FMR1— Fragile X Mental Retardation Protein (FMRP; Penagarikano et al, 2007)

  • We summarize current knowledge of FXS behavioral and cognitive phenotype, the circuitry abnormalities related to them and how interneurons are an important subject of study to understand alterations in neuronal networks

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Summary

Introduction

Fragile X Syndrome (FXS) is one of several disorders associated with autism spectrum disorders (ASDs)—a heterogeneous group of behaviorally identified neurodevelopmental disabilities. Understanding the roles of inhibitory interneurons and how they control neuronal networks is of great importance in studying neurodevelopmental disorders such as FXS.

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