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Neonatal expression of human FMRP isoform corrects cortical deficits and improves behavior in a mouse model of fragile X syndrome.

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Dysfunctional neural dynamics associated with sensory phenotypes in Fragile X syndrome: insights from mouse models.

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Astrocytic GABA controls fidelity of temporal cortical processing in Fragile X Syndrome.

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Related Experiment Video

Updated: May 2, 2026

Dissecting Cell-Autonomous Function of Fragile X Mental Retardation Protein in an Auditory Circuit by In Ovo Electroporation
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Auditory processing in fragile x syndrome.

Sarah E Rotschafer1, Khaleel A Razak1

  • 1Graduate Neuroscience Program, Department of Psychology, University of California, Riverside, CA USA.

Frontiers in Cellular Neuroscience
|February 20, 2014
PubMed
Summary

Fragile X syndrome (FXS) causes intellectual disability and autism, often involving auditory hypersensitivity. This review highlights auditory hyper-excitability in FXS patients and mouse models as a key biomarker for developing new treatments.

Keywords:
audiogenic seizuresauditory responsesautismbiomarkerscortexfragile X syndromesensory hypersensitivity

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Area of Science:

  • Neuroscience
  • Genetics
  • Developmental Biology

Background:

  • Fragile X syndrome (FXS) is a genetic disorder linked to intellectual disability, autism, and sensory processing deficits.
  • Auditory hypersensitivity is a consistent symptom in humans with FXS and is observed in the Fmr1 knockout (KO) mouse model.
  • Current research focuses on understanding the molecular and circuit-level mechanisms underlying FXS symptoms.

Purpose of the Study:

  • To review clinical and preclinical evidence supporting auditory hypersensitivity as a core FXS phenotype.
  • To establish auditory hyper-excitability as a translatable biomarker for FXS research.
  • To advocate for leveraging auditory processing deficits to develop targeted therapeutics for FXS and related autism spectrum disorders.

Main Methods:

  • Consolidated findings from human clinical, behavioral, and electrophysiological studies.
  • Integrated data from in vivo electrophysiological recordings and behavioral analyses in Fmr1 KO mice.
  • Examined structural and functional studies related to sensory processing in FXS.

Main Results:

  • Consistent auditory hypersensitivity and hyper-excitable auditory cortex responses in humans and Fmr1 KO mice.
  • Fmr1 KO mice exhibit increased acoustic startle response and abnormal spectrotemporal processing.
  • Auditory evoked responses in FXS patients and mice serve as reliable outcome measures for therapeutic interventions.

Conclusions:

  • Auditory hypersensitivity is a robust and translatable biomarker for Fragile X syndrome.
  • Investigating auditory processing deficits offers a tractable pathway to uncover molecular and cellular mechanisms of FXS.
  • Targeting sensory cortical deficits presents a promising strategy for developing novel therapeutics for FXS and autism spectrum disorders.