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 …

Modeling fragile X syndrome in the Fmr1 knockout mouse

TM Kazdoba, PT Leach, JL Silverman… - Intractable & rare …, 2014 - jstage.jst.go.jp
Summary Fragile X Syndrome (FXS) is a commonly inherited form of intellectual disability
and one of the leading genetic causes for autism spectrum disorder. Clinical symptoms of …

Prefrontal cortex dysfunction in fragile X mice depends on the continued absence of fragile X mental retardation protein in the adult brain

JJ Siegel, RA Chitwood, JM Ding, C Payne… - Journal of …, 2017 - Soc Neuroscience
Fragile X Syndrome (FX) is generally considered a developmental disorder, arising from a
mutation that disrupts the transcription of Fragile X Mental Retardation Protein (FMRP) …

The molecular biology of FMRP: new insights into fragile X syndrome

JD Richter, X Zhao - Nature Reviews Neuroscience, 2021 - nature.com
Fragile X mental retardation protein (FMRP) is the product of the fragile X mental retardation
1 gene (FMR1), a gene that—when epigenetically inactivated by a triplet nucleotide repeat …

Fragile X syndrome and model organisms: identifying potential routes of therapeutic intervention

B Bhogal, TA Jongens - Disease models & mechanisms, 2010 - journals.biologists.com
Fragile X syndrome (FXS) is a cognitive disorder caused by silencing of the fragile X mental
retardation 1 gene (FMR1). Since the discovery of the gene almost two decades ago, most …

Neuroanatomical, molecular genetic, and behavioral correlates of fragile X syndrome

SD Koukoui, A Chaudhuri - Brain research reviews, 2007 - Elsevier
Fragile X syndrome (FXS) is a leading cause of inherited mental retardation. In the vast
majority of cases, this X-linked disorder is due to a CGG expansion in the 5′ untranslated …

Molecular and cellular aspects of mental retardation in the Fragile X syndrome: from gene mutation/s to spine dysmorphogenesis

S De Rubeis, E Fernández, A Buzzi… - Synaptic Plasticity …, 2012 - Springer
Abstract The Fragile X syndrome (FXS) is the most frequent form of inherited mental
retardation and also considered a monogenic cause of Autism Spectrum Disorder. FXS …

Increasing our understanding of human cognition through the study of Fragile X Syndrome

D Cook, E Nuro, KK Murai - Developmental neurobiology, 2014 - Wiley Online Library
ABSTRACT Fragile X Syndrome (FXS) is considered the most common form of inherited
intellectual disability. It is caused by reductions in the expression level or function of a single …

Learning and memory deficits consequent to reduction of the fragile X mental retardation protein result from metabotropic glutamate receptor-mediated inhibition of …

AK Kanellopoulos, O Semelidou, AG Kotini… - Journal of …, 2012 - Soc Neuroscience
Loss of the RNA-binding fragile X protein [fragile X mental retardation protein (FMRP)]
results in a spectrum of cognitive deficits, the fragile X syndrome (FXS), while aging …

Exaggerated behavioral phenotypes in Fmr1/Fxr2 double knockout mice reveal a functional genetic interaction between Fragile X-related proteins

CM Spencer, E Serysheva… - Human molecular …, 2006 - academic.oup.com
Individuals affected by Fragile X syndrome (FXS) experience cognitive impairment,
hyperactivity, attention deficits, social anxiety and autistic-like behaviors. FXS results from …