From circuits to behavior: Amygdala dysfunction in fragile X syndrome

Fragile X syndrome (FXS) is a neurodevelopmental disorder caused by a repeat expansion mutation in the promotor region of the FMR1 gene resulting in transcriptional silencing and loss of function of fragile X messenger ribonucleoprotein 1 protein (FMRP). FMRP has a well-defined role in the early dev...

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Main Authors: Matthew N. Svalina, Regina Sullivan, Diego Restrepo, Molly M. Huntsman
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-03-01
Series:Frontiers in Integrative Neuroscience
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fnint.2023.1128529/full
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author Matthew N. Svalina
Matthew N. Svalina
Matthew N. Svalina
Matthew N. Svalina
Regina Sullivan
Regina Sullivan
Diego Restrepo
Diego Restrepo
Molly M. Huntsman
Molly M. Huntsman
Molly M. Huntsman
author_facet Matthew N. Svalina
Matthew N. Svalina
Matthew N. Svalina
Matthew N. Svalina
Regina Sullivan
Regina Sullivan
Diego Restrepo
Diego Restrepo
Molly M. Huntsman
Molly M. Huntsman
Molly M. Huntsman
author_sort Matthew N. Svalina
collection DOAJ
description Fragile X syndrome (FXS) is a neurodevelopmental disorder caused by a repeat expansion mutation in the promotor region of the FMR1 gene resulting in transcriptional silencing and loss of function of fragile X messenger ribonucleoprotein 1 protein (FMRP). FMRP has a well-defined role in the early development of the brain. Thus, loss of the FMRP has well-known consequences for normal cellular and synaptic development leading to a variety of neuropsychiatric disorders including an increased prevalence of amygdala-based disorders. Despite our detailed understanding of the pathophysiology of FXS, the precise cellular and circuit-level underpinnings of amygdala-based disorders is incompletely understood. In this review, we discuss the development of the amygdala, the role of neuromodulation in the critical period plasticity, and recent advances in our understanding of how synaptic and circuit-level changes in the basolateral amygdala contribute to the behavioral manifestations seen in FXS.
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spelling doaj.art-2af72f50d48e4f19a3518a0fea1f48b02023-03-09T06:36:47ZengFrontiers Media S.A.Frontiers in Integrative Neuroscience1662-51452023-03-011710.3389/fnint.2023.11285291128529From circuits to behavior: Amygdala dysfunction in fragile X syndromeMatthew N. Svalina0Matthew N. Svalina1Matthew N. Svalina2Matthew N. Svalina3Regina Sullivan4Regina Sullivan5Diego Restrepo6Diego Restrepo7Molly M. Huntsman8Molly M. Huntsman9Molly M. Huntsman10Medical Scientist Training Program, University of Colorado Anschutz Medical Campus, Aurora, CO, United StatesNeuroscience Graduate Program, University of Colorado Anschutz Medical Campus, Aurora, CO, United StatesDepartment of Pharmaceutical Sciences, University of Colorado Anschutz Medical Campus, Aurora, CO, United StatesDepartment of Cell and Developmental Biology, University of Colorado Anschutz Medical Campus, Aurora, CO, United StatesBrain Institute, Nathan Kline Institute, Orangeburg, NY, United StatesChild and Adolescent Psychiatry, Child Study Center, New York University School of Medicine, New York, NY, United StatesNeuroscience Graduate Program, University of Colorado Anschutz Medical Campus, Aurora, CO, United StatesDepartment of Cell and Developmental Biology, University of Colorado Anschutz Medical Campus, Aurora, CO, United StatesNeuroscience Graduate Program, University of Colorado Anschutz Medical Campus, Aurora, CO, United StatesDepartment of Pharmaceutical Sciences, University of Colorado Anschutz Medical Campus, Aurora, CO, United StatesDepartment of Pediatrics, University of Colorado Anschutz Medical Campus, Aurora, CO, United StatesFragile X syndrome (FXS) is a neurodevelopmental disorder caused by a repeat expansion mutation in the promotor region of the FMR1 gene resulting in transcriptional silencing and loss of function of fragile X messenger ribonucleoprotein 1 protein (FMRP). FMRP has a well-defined role in the early development of the brain. Thus, loss of the FMRP has well-known consequences for normal cellular and synaptic development leading to a variety of neuropsychiatric disorders including an increased prevalence of amygdala-based disorders. Despite our detailed understanding of the pathophysiology of FXS, the precise cellular and circuit-level underpinnings of amygdala-based disorders is incompletely understood. In this review, we discuss the development of the amygdala, the role of neuromodulation in the critical period plasticity, and recent advances in our understanding of how synaptic and circuit-level changes in the basolateral amygdala contribute to the behavioral manifestations seen in FXS.https://www.frontiersin.org/articles/10.3389/fnint.2023.1128529/fullcritical periodsynaptic plasticitybasolateral amygdalafragile X syndromedevelopmentE/I balance
spellingShingle Matthew N. Svalina
Matthew N. Svalina
Matthew N. Svalina
Matthew N. Svalina
Regina Sullivan
Regina Sullivan
Diego Restrepo
Diego Restrepo
Molly M. Huntsman
Molly M. Huntsman
Molly M. Huntsman
From circuits to behavior: Amygdala dysfunction in fragile X syndrome
Frontiers in Integrative Neuroscience
critical period
synaptic plasticity
basolateral amygdala
fragile X syndrome
development
E/I balance
title From circuits to behavior: Amygdala dysfunction in fragile X syndrome
title_full From circuits to behavior: Amygdala dysfunction in fragile X syndrome
title_fullStr From circuits to behavior: Amygdala dysfunction in fragile X syndrome
title_full_unstemmed From circuits to behavior: Amygdala dysfunction in fragile X syndrome
title_short From circuits to behavior: Amygdala dysfunction in fragile X syndrome
title_sort from circuits to behavior amygdala dysfunction in fragile x syndrome
topic critical period
synaptic plasticity
basolateral amygdala
fragile X syndrome
development
E/I balance
url https://www.frontiersin.org/articles/10.3389/fnint.2023.1128529/full
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