Divergent dysregulation of gene expression in murine models of fragile X syndrome and tuberous sclerosis

SW Kong, M Sahin, CD Collins, MH Wertz… - Molecular Autism, 2014 - Springer
Background Fragile X syndrome and tuberous sclerosis are genetic syndromes that both
have a high rate of comorbidity with autism spectrum disorder (ASD). Several lines of …

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 …

Fragile X mice: Reduced long‐term potentiation and N‐Methyl‐D‐Aspartate receptor‐mediated neurotransmission in dentate gyrus

SH Yun, BL Trommer - Journal of neuroscience research, 2011 - Wiley Online Library
Fragile X syndrome (FXS) is a monogenic mental retardation syndrome that frequently
includes autism. The Fmr1‐knockout (Fmr1‐KO) mouse, like FXS‐affected individuals, lacks …

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 …

Alterations in the functional neural circuitry supporting flexible choice behavior in autism spectrum disorders

AM D'Cruz, MW Mosconi, ME Ragozzino… - Translational …, 2016 - nature.com
Restricted and repetitive behaviors, and a pronounced preference for behavioral and
environmental consistency, are distinctive characteristics of autism spectrum disorder (ASD) …

Altered frontal connectivity as a mechanism for executive function deficits in fragile X syndrome

LM Schmitt, J Li, R Liu, PS Horn, JA Sweeney… - Molecular Autism, 2022 - Springer
Abstract Background Fragile X syndrome (FXS) is the leading inherited monogenic cause of
intellectual disability and autism spectrum disorder. Executive function (EF), necessary for …

Fragile X syndrome: the GABAergic system and circuit dysfunction

SM Paluszkiewicz, BS Martin… - Developmental …, 2011 - karger.com
Fragile X syndrome (FXS) is a neurodevelopmental disorder characterized by intellectual
disability, sensory hypersensitivity, and high incidences of autism spectrum disorders and …

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 …

[HTML][HTML] Multiple drug treatments that increase cAMP signaling restore long-term memory and aberrant signaling in fragile X syndrome models

CH Choi, BP Schoenfeld, AJ Bell, J Hinchey… - Frontiers in behavioral …, 2016 - frontiersin.org
Fragile X is the most common monogenic disorder associated with intellectual disability (ID)
and autism spectrum disorders (ASD). Additionally, many patients are afflicted with …

Hypersensitivity to distractors in Fragile X syndrome from loss of modulation of cortical VIP interneurons

N Rahmatullah, LM Schmitt, L De Stefano… - Journal of …, 2023 - Soc Neuroscience
Attention deficit is one of the most prominent and disabling symptoms in Fragile X syndrome
(FXS). Hypersensitivity to sensory stimuli contributes to attention difficulties by overwhelming …