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A resting EEG study of neocortical hyperexcitability and altered functional connectivity in fragile X syndrome.
Open accessAbstract
BACKGROUND: KO mice, a mouse model of the fragile X syndrome which is the most common single gene cause of autism and intellectual disability. METHODS: We collected resting state dense-array electroencephalography data from 21 fragile X syndrome (FXS) patients and 21 age-matched healthy participants. RESULTS: FXS patients exhibited greater gamma frequency band power, which was correlated with social and sensory processing difficulties. Second, FXS patients showed increased spatial spreading of phase-synchronized high frequency neural activity in the gamma band. Third, we observed increased negative theta-to-gamma but decreased alpha-to-gamma band amplitude coupling, and the level of increased theta power was inversely related to the level of resting gamma power in FXS. CONCLUSIONS: Increased theta band power and coupling from frontal sources may represent a mechanism providing compensatory inhibition of high-frequency gamma band activity, potentially contributing to the widely varying level of neurophysiological and behavioral abnormalities and treatment response seen in full-mutation FXS patients. These findings extend preclinical observations and provide new mechanistic insights into brain alterations and their variability across FXS patients. Electrophysiological measures may provide useful translational biomarkers for advancing drug development and individualizing treatments for neurodevelopmental disorders with associated neuronal hyperexcitability.
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“These phenotypes in the mouse model are remarkably similar to those seen in humans with FXS (Castrén et al., 2003; Ethridge et al., 2016; Wang et al., 2017).”
From The Perineuronal ‘Safety’ Net? Perineuronal Net Abnormalities in Neurological Disorders (2018) · cites this paper as Wang et al., 2017
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Metadata
- Authors
- Wang, J., Ethridge, L. E., Mosconi, M. W., White, S. P., Binder, D. K., Pedapati, E. V., Erickson, C. A., Byerly, M. J., & Sweeney, J. A.
- Affiliations
- Zhejiang Normal University (CN)
- University of Oklahoma Health Sciences Center (US)
- University of Kansas (US)
- The University of Texas Southwestern Medical Center (US)
- University of California, Riverside (US)
- Cincinnati Children's Hospital Medical Center (US)
- Montana State University (US)
- University of Cincinnati (US)
- Corresponding author
- Wang, Jun
- ORCID
- Venue
- Journal of Neurodevelopmental Disorders
- Published
- 2017-03-14
- Volume
- 9
- Issue
- 1
- Pages
- 11-11
- ISSN
- 1866-1947 (ISSN-L)
- 1866-1955
- PMC
- PMC5351111
- OpenAlex
- W2596913176
- Cited by
- 176
- References
- 63
- Citations by year
- 2026: 19 · 2025: 17 · 2024: 24 · 2023: 16 · 2022: 26 · 2021: 21 · 2020: 17 · 2019: 20 · 2018: 12 · 2017: 4
- FWCI
- 9.62
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Locations
- Journal of Neurodevelopmental Disorders (publishedVersion, cc-by)
- Europe PMC (PubMed Central) (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- KU ScholarWorks (University of Kansas) (submittedVersion, cc-by)
- Funders
- National Institute of Child Health and Human Development (U54 HD082008-01)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Life Sciences › Neuroscience › Cognitive Neuroscience › Autism Spectrum Disorder Research
- Life Sciences › Neuroscience › Cognitive Neuroscience › Neural dynamics and brain function
- Keywords
- fragile X syndrome, resting-state EEG, functional connectivity, gamma power, theta power, amplitude coupling, autism spectrum disorder, intellectual disability, biomarkers, neurodevelopmental disorders
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