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Dysregulated Prefrontal Cortex Inhibition in Prepubescent and Adolescent Fragile X Mouse Model.

Ioannis Kramvis1, Rhodé van Westen2, Hanna C A Lammertse2

  • 1Department of Integrative Neurophysiology, Center for Neurogenomics and Cognitive Research, Vrije Universiteit, Amsterdam, Netherlands.

Frontiers in Molecular Neuroscience
|June 13, 2020
PubMed
Summary

Fragile X syndrome (FXS) involves altered brain inhibition. In FXS mice, medial prefrontal cortex inhibitory signaling changes significantly during development, impacting neurodevelopmental disorder pathology.

Keywords:
Fragile XGABAelectrophysiologyplasticityprefrontal cortex

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Area of Science:

  • Neuroscience
  • Neurobiology
  • Developmental Neuroscience

Background:

  • Neurodevelopmental disorders, including intellectual disability and autism, are linked to altered brain excitation-inhibition balance.
  • Fragile X syndrome (FXS), a leading genetic cause of intellectual disability, exhibits known synaptic alterations, particularly in the prefrontal cortex (PFC).
  • While excitatory changes in the FXS mouse model's PFC are documented, the role of inhibitory signaling remains less understood.

Purpose of the Study:

  • To investigate GABAergic signaling alterations in the medial prefrontal cortex (mPFC) of the Fragile X Mental Retardation 1 (FMR1) knock out (Fmr1-KO) mouse model.
  • To characterize changes in inhibition at both molecular and functional levels during key developmental stages (prepubescence and adolescence).

Main Methods:

  • Electrophysiological recordings of inhibitory postsynaptic currents in the mPFC of Fmr1-KO and wild-type (WT) littermate mice.
  • Analysis of synaptic transmission, short-term plasticity, and receptor kinetics.
  • Quantitative assessment of GABAergic receptor subunit expression (GABAA and GABAB) during adolescence.

Main Results:

  • During prepubescence, Fmr1-KO mice exhibited enhanced inhibition with increased synaptic drive, amplitude, and reduced short-term depression.
  • Noise analysis revealed more receptor openings at inhibitory synapses in prepubescent Fmr1-KO mice.
  • During adolescence, inhibitory amplitudes and plasticity normalized, but inhibitory drive decreased, and synaptic kinetics prolonged in Fmr1-KO mice.
  • Adolescent GABAA receptor subunit α2 and GABAB receptor subtype B1 expression levels differed between Fmr1-KO and WT mice.

Conclusions:

  • Synaptic GABAergic alterations are present in the behaviorally relevant mPFC of Fmr1-KO mice, extending previous findings in FXS.
  • These developmental changes in inhibitory signaling contribute to the pathobiology of neurodevelopmental disorders associated with FXS.
  • The study highlights the complex, stage-dependent alterations in inhibitory neurotransmission within the FXS model.