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 …

Dysregulation of GABAA Receptor-Mediated Neurotransmission during the Auditory Cortex Critical Period in the Fragile X Syndrome Mouse Model

YJ Song, B Xing, AJ Barbour, C Zhou… - Cerebral cortex, 2022 - academic.oup.com
Fragile X syndrome (FXS) is the leading monogenic form of intellectual disability and autism,
with patients exhibiting numerous auditory-related phenotypes during their developmental …

A novel combination treatment for fragile X syndrome predicted using computational methods

W Chadwick, I Angulo-Herrera, P Cogram… - Brain …, 2024 - academic.oup.com
Fragile X syndrome is a neurodevelopmental disorder caused by silencing of the fragile X
messenger ribonucleotide gene. Patients display a wide spectrum of symptoms ranging from …

Defective GABAergic neurotransmission and pharmacological rescue of neuronal hyperexcitability in the amygdala in a mouse model of fragile X syndrome

JL Olmos-Serrano, SM Paluszkiewicz… - Journal of …, 2010 - Soc Neuroscience
Fragile X syndrome (FXS) is a neurodevelopmental disorder characterized by variable
cognitive impairment and behavioral disturbances such as exaggerated fear, anxiety and …

GABAergic abnormalities in the fragile X syndrome

N Van der Aa, RF Kooy - European Journal of Paediatric Neurology, 2020 - Elsevier
Many pathways have been involved in pathophysiology of the fragile X syndrome, one of the
more frequent genetic causes of intellectual disability and autism. This review highlights the …

The FXG: a presynaptic fragile X granule expressed in a subset of developing brain circuits

SB Christie, MR Akins, JE Schwob… - Journal of …, 2009 - Soc Neuroscience
The loss of Fragile X mental retardation protein (FMRP) causes Fragile X syndrome, the
most common inherited mental retardation and single gene cause of autism. Although …

[HTML][HTML] Genetic-Background Modulation of Core and Variable Autistic-Like Symptoms in Fmr1 Knock-Out Mice

S Pietropaolo, A Guilleminot, B Martin, FR d'Amato… - PloS one, 2011 - journals.plos.org
Background No animal models of autism spectrum disorders (ASD) with good construct
validity are currently available; using genetic models of pathologies characterized by ASD …

Altered striatal actin dynamics drives behavioral inflexibility in a mouse model of fragile X syndrome

V Mercaldo, B Vidimova, D Gastaldo, E Fernández… - Neuron, 2023 - cell.com
The proteome of glutamatergic synapses is diverse across the mammalian brain and
involved in neurodevelopmental disorders (NDDs). Among those is fragile X syndrome …

[HTML][HTML] Long-term behavioral effects of prenatal stress in the Fmr1-knock-out mouse model for fragile X syndrome

V Petroni, E Subashi, M Premoli, M Memo… - Frontiers in Cellular …, 2022 - frontiersin.org
Fragile X syndrome (FXS) is a major neurodevelopmental disorder and the most common
monogenic cause of autism spectrum disorder (ASD). FXS is caused by a mutation in the X …

Analysis of Fmr1 Deletion in a Subpopulation of Post‐Mitotic Neurons in Mouse Cortex and Hippocampus

A Amiri, E Sanchez‐Ortiz, W Cho… - Autism …, 2014 - Wiley Online Library
Fragile X syndrome (FXS) is the most common form of inherited mental retardation and the
leading cause of autism. FXS is caused by mutation in a single gene, FMR 1, which encodes …