Altered neocortical rhythmic activity states in Fmr1 KO mice are due to enhanced mGluR5 signaling and involve changes in excitatory circuitry

SA Hays, KM Huber, JR Gibson - Journal of Neuroscience, 2011 - Soc Neuroscience
Despite the pronounced neurological deficits associated with mental retardation and autism,
the degree to which neocortical circuit function is altered remains unknown. Here, we study …

Imbalance of neocortical excitation and inhibition and altered UP states reflect network hyperexcitability in the mouse model of fragile X syndrome

JR Gibson, AF Bartley, SA Hays… - Journal of …, 2008 - journals.physiology.org
Despite the pronounced neurological deficits associated with mental retardation and autism,
it is unknown if altered neocortical circuit function occurs in these prevalent disorders. Here …

Circuit level defects in the developing neocortex of Fragile X mice

JT Gonçalves, JE Anstey, P Golshani… - Nature …, 2013 - nature.com
Subtle alterations in how cortical network dynamics are modulated by different behavioral
states could disrupt normal brain function and underlie symptoms of neuropsychiatric …

Abnormal sleep architecture and hippocampal circuit dysfunction in a mouse model of fragile X syndrome

CE Boone, H Davoudi, JB Harrold, DJ Foster - Neuroscience, 2018 - Elsevier
Fragile X syndrome (FXS) is the most common heritable cause of intellectual disability and
single-gene cause of autism spectrum disorder. The Fmr1 null mouse models much of the …

Impaired inhibitory control of cortical synchronization in fragile X syndrome

SM Paluszkiewicz, JL Olmos-Serrano… - Journal of …, 2011 - journals.physiology.org
Fragile X syndrome (FXS) is a neurodevelopmental disorder characterized by severe
cognitive impairments, sensory hypersensitivity, and comorbidities with autism and epilepsy …

A resting EEG study of neocortical hyperexcitability and altered functional connectivity in fragile X syndrome

J Wang, LE Ethridge, MW Mosconi, SP White… - Journal of …, 2017 - Springer
Background Cortical hyperexcitability due to abnormal fast-spiking inhibitory interneuron
function has been documented in fmr1 KO mice, a mouse model of the fragile X syndrome …

Spontaneous Activity Patterns Are Altered in the Developing Visual Cortex of the Fmr1 Knockout Mouse

JE Cheyne, N Zabouri, D Baddeley… - Frontiers in Neural …, 2019 - frontiersin.org
Fragile X syndrome (FXS) is the most prevalent inherited cause of autism and is
accompanied by behavioral and sensory deficits. Errors in the wiring of the brain during …

Sensorimotor gating abnormalities in young males with fragile X syndrome and Fmr1-knockout mice

PW Frankland, Y Wang, B Rosner, T Shimizu… - Molecular …, 2004 - nature.com
Fragile X syndrome (FXS) is the most common single gene (FMR1) disorder affecting
cognitive and behavioral function in humans. This syndrome is characterized by a cluster of …

GABAB receptor‐mediated feed‐forward circuit dysfunction in the mouse model of fragile X syndrome

S Wahlstrom‐Helgren… - The Journal of …, 2015 - Wiley Online Library
Key points Cortico‐hippocampal feed‐forward circuits formed by the temporoammonic (TA)
pathway exhibit a marked increase in excitation/inhibition ratio and abnormal spike …

Translation-relevant EEG phenotypes in a mouse model of Fragile X Syndrome

JW Lovelace, IM Ethell, DK Binder, KA Razak - Neurobiology of disease, 2018 - Elsevier
Identification of comparable biomarkers in humans and validated animal models will
facilitate pre-clinical to clinical therapeutic pipelines to treat neurodevelopmental disorders …