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 …

The cyclic AMP cascade is altered in the fragile X nervous system

DJ Kelley, RJ Davidson, JL Elliott, GP Lahvis, JCP Yin… - PloS one, 2007 - journals.plos.org
Fragile X syndrome (FX), the most common heritable cause of mental retardation and
autism, is a developmental disorder characterized by physical, cognitive, and behavioral …

STEP inhibition reverses behavioral, electrophysiologic, and synaptic abnormalities in Fmr1 KO mice

M Chatterjee, PK Kurup, CJ Lundbye, AKH Toft… - …, 2018 - Elsevier
Fragile X syndrome (FXS) is the leading cause of inherited intellectual disability, with
additional symptoms including attention deficit and hyperactivity, anxiety, impulsivity, and …

Targeted treatments for fragile X syndrome

E Berry-Kravis, A Knox, C Hervey - Journal of Neurodevelopmental …, 2011 - Springer
Fragile X syndrome (FXS) is the most common identifiable genetic cause of intellectual
disability and autistic spectrum disorders (ASD), with up to 50% of males and some females …

PDE-4 inhibition rescues aberrant synaptic plasticity in Drosophila and mouse models of fragile X syndrome

CH Choi, BP Schoenfeld, ED Weisz… - Journal of …, 2015 - Soc Neuroscience
Fragile X syndrome (FXS) is the leading cause of both intellectual disability and autism
resulting from a single gene mutation. Previously, we characterized cognitive impairments …

Multiple behavior phenotypes of the fragile-X syndrome mouse model respond to chronic inhibition of phosphodiesterase-4D (PDE4D)

ME Gurney, P Cogram, RM Deacon, C Rex… - Scientific reports, 2017 - nature.com
Fragile-X syndrome (FXS) patients display intellectual disability and autism spectrum
disorder due to silencing of the X-linked, fragile-X mental retardation-1 (FMR1) gene …

Pharmacological rescue of Ras signaling, GluA1-dependent synaptic plasticity, and learning deficits in a fragile X model

CS Lim, ET Hoang, KE Viar, RL Stornetta… - Genes & …, 2014 - genesdev.cshlp.org
Fragile X syndrome, caused by the loss of Fmr1 gene function, is the most common form of
inherited mental retardation, with no effective treatment. Using a tractable animal model, we …

Mechanism-based treatments in neurodevelopmental disorders: fragile X syndrome

E Berry-Kravis - Pediatric neurology, 2014 - Elsevier
Abstract Background Fragile X syndrome (FXS) is the most common identifiable genetic
cause of intellectual disability and autistic spectrum disorders. Recent major advances have …

Chronic metabotropic glutamate receptor 5 inhibition corrects local alterations of brain activity and improves cognitive performance in fragile X mice

A Michalon, A Bruns, C Risterucci, M Honer… - Biological …, 2014 - Elsevier
Background Fragile X syndrome (FXS) is the most common genetic cause for intellectual
disability. Fmr1 knockout (KO) mice are an established model of FXS. Chronic …

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 …