Cited by 53Genetics and Neurodevelopmental DisordersNeuroscience and Neuropharmacology ResearchAutism Spectrum Disorder Research

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Hyperexcitability and homeostasis in fragile X Syndrome.

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Liu, Xiaopeng, Kumar, Vipendra, Tsai, Nien-Pei, Auerbach, Benjamin D.·2022·Frontiers in Molecular Neuroscience

DOI 10.3389/fnmol.2021.805929PMC PMC8770333

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Abstract

Fragile X Syndrome (FXS) is a leading inherited cause of autism and intellectual disability, resulting from a mutation in the FMR1 gene and subsequent loss of its protein product FMRP. Despite this simple genetic origin, FXS is a phenotypically complex disorder with a range of physical and neurocognitive disruptions. While numerous molecular and cellular pathways are affected by FMRP loss, there is growing evidence that circuit hyperexcitability may be a common convergence point that can account for many of the wide-ranging phenotypes seen in FXS. The mechanisms for hyperexcitability in FXS include alterations to excitatory synaptic function and connectivity, reduced inhibitory neuron activity, as well as changes to ion channel expression and conductance. However, understanding the impact of FMR1 mutation on circuit function is complicated by the inherent plasticity in neural circuits, which display an array of homeostatic mechanisms to maintain activity near set levels. FMRP is also an important regulator of activity-dependent plasticity in the brain, meaning that dysregulated plasticity can be both a cause and consequence of hyperexcitable networks in FXS. This makes it difficult to separate the direct effects of FMR1 mutation from the myriad and pleiotropic compensatory changes associated with it, both of which are likely to contribute to FXS pathophysiology. Here we will: (1) review evidence for hyperexcitability and homeostatic plasticity phenotypes in FXS models, focusing on similarities/differences across brain regions, cell-types, and developmental time points; (2) examine how excitability and plasticity disruptions interact with each other to ultimately contribute to circuit dysfunction in FXS; and (3) discuss how these synaptic and circuit deficits contribute to disease-relevant behavioral phenotypes like epilepsy and sensory hypersensitivity. Through this discussion of where the current field stands, we aim to introduce perspectives moving forward in FXS research.

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See all 53

Authors: Liu, X., Kumar, V., Tsai, N.-P., & Auerbach, B. D.
Affiliations: University of Illinois Urbana-Champaign (US)
Corresponding author: Auerbach, Benjamin D.
ORCID: Auerbach, Benjamin D.
Venue: Frontiers in Molecular Neuroscience
Publisher: Frontiers Media SA
Published: 2022-01-06
Volume: 14
Pages: 805929-805929
ISSN: 1662-5099 (ISSN-L)
DOI: 10.3389/fnmol.2021.805929
PMC: PMC8770333
OpenAlex: W4205495648
Cited by: 53
References: 291
Citations by year: 2026: 9 · 2025: 13 · 2024: 10 · 2023: 11 · 2022: 8
FWCI: 6.33
Type: journal article
Language: en
Open access: gold
doi.org
Licence: cc-by
creativecommons.org/licenses/by/4.0/ (vor)
Locations: Frontiers in Molecular Neuroscience (publishedVersion, cc-by)
PubMed Central (publishedVersion, cc-by)
PubMed (publishedVersion)
DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
Funders: National Institute of Mental Health (R01MH124827)
National Institute on Deafness and Other Communication Disorders (K01DC018310)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Neuroscience and Neuropharmacology Research
Life Sciences › Neuroscience › Cognitive Neuroscience › Autism Spectrum Disorder Research
Keywords: fragile X syndrome, FMRP, neuronal hyperexcitability, neuronal homeostasis, homeostatic plasticity, inhibitory neurons, ion channels, activity-dependent plasticity, autism spectrum disorder, intellectual disability, epilepsy, sensory hypersensitivity, circuit dysfunction

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Hyperexcitability and homeostasis in fragile X Syndrome.

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