The role of the dorsal striatum in a mouse model for fragile X syndrome: Behavioral and dendritic spine assessment

JL Huebschman, CA Monterrey, DM Foster… - Brain Research, 2022 - Elsevier
Fragile X syndrome (FXS), a leading monogenic cause of autism spectrum disorders
(ASDs), typically occurs as the result of a mutation silencing the Fmr1 gene, preventing …

[HTML][HTML] Cell-type-specific disruption of cortico-striatal circuitry drives repetitive patterns of behavior in fragile X syndrome model mice

F Longo, S Aryal, PG Anastasiades, M Maltese… - Cell reports, 2023 - cell.com
Individuals with fragile X syndrome (FXS) are frequently diagnosed with autism spectrum
disorder (ASD), including increased risk for restricted and repetitive behaviors (RRBs) …

[HTML][HTML] Investigating cell-specific effects of FMRP deficiency on spiny projection neurons in a mouse model of Fragile X syndrome

G Giua, O Lassalle, L Makrini-Maleville… - Frontiers in Cellular …, 2023 - frontiersin.org
Introduction Fragile X syndrome (FXS), resulting from a mutation in the Fmr1 gene, is the
most common monogenic cause of autism and inherited intellectual disability. Fmr1 encodes …

Delayed stabilization of dendritic spines in fragile X mice

A Cruz-Martín, M Crespo… - Journal of …, 2010 - Soc Neuroscience
Fragile X syndrome (FXS) causes mental impairment and autism through transcriptional
silencing of the Fmr1 gene, resulting in the loss of the RNA-binding protein fragile X mental …

Cognitive dysfunction and prefrontal synaptic abnormalities in a mouse model of fragile X syndrome

DD Krueger, EK Osterweil, SP Chen… - Proceedings of the …, 2011 - National Acad Sciences
Among the hallmark phenotypes reported in individuals with fragile X syndrome (FXS) are
deficits in attentional function, inhibitory control, and cognitive flexibility, a set of cognitive …

Learning and behavioral deficits associated with the absence of the fragile X mental retardation protein: what a fly and mouse model can teach us

AR Santos, AK Kanellopoulos, C Bagni - Learning & memory, 2014 - learnmem.cshlp.org
The Fragile X syndrome (FXS) is the most frequent form of inherited mental disability and is
considered a monogenic cause of autism spectrum disorder. FXS is caused by a triplet …

[HTML][HTML] Cerebellar contribution to autism-relevant behaviors in fragile X syndrome models

JM Gibson, AH Vazquez, K Yamashiro, V Jakkamsetti… - Cell reports, 2023 - cell.com
Cerebellar dysfunction has been linked to autism spectrum disorders (ASDs). Although
cerebellar pathology has been observed in individuals with fragile X syndrome (FXS) and in …

[HTML][HTML] The fragile X mental retardation protein regulates striatal medium spiny neuron synapse density and dendritic spine morphology

JL Huebschman, KS Corona, Y Guo… - Frontiers in molecular …, 2020 - frontiersin.org
The fragile X mental retardation protein (FMRP), an RNA-binding protein that mediates the
transport, stability, and translation of hundreds of brain RNAs, is critically involved in …

Modelling Fragile X Syndrome in rats: New directions in translational research

A Asiminas - 2017 - era.ed.ac.uk
Fragile X syndrome (FXS) is the leading single gene cause of intellectual disability and
Autism Spectrum Disorder (ASD). It is caused by epigenetic silencing of the fragile X mental …

Abnormal presynaptic short-term plasticity and information processing in a mouse model of fragile X syndrome

PY Deng, D Sojka, VA Klyachko - Journal of neuroscience, 2011 - Soc Neuroscience
Fragile X syndrome (FXS) is the most common inherited form of intellectual disability and the
leading genetic cause of autism. It is associated with the lack of fragile X mental retardation …