Mouse models of fragile X-related disorders

R Willemsen, RF Kooy - Disease Models & Mechanisms, 2023 - journals.biologists.com
The fragile X-related disorders are an important group of hereditary disorders that are
caused by expanded CGG repeats in the 5′ untranslated region of the FMR1 gene or by …

[HTML][HTML] A systematic review of brainstem contributions to autism spectrum disorder

A Seif, C Shea, S Schmid… - Frontiers in Integrative …, 2021 - frontiersin.org
Autism spectrum disorder (ASD) is a neurodevelopmental disorder that affects one in 66
children in Canada. The contributions of changes in the cortex and cerebellum to autism …

[HTML][HTML] Developmental delays in cortical auditory temporal processing in a mouse model of Fragile X syndrome

K Croom, JA Rumschlag, MA Erickson… - Journal of …, 2023 - Springer
Background Autism spectrum disorders (ASD) encompass a wide array of debilitating
symptoms, including sensory dysfunction and delayed language development. Auditory …

[HTML][HTML] Excessive proteostasis contributes to pathology in fragile X syndrome

SR Louros, SS Seo, B Maio, C Martinez-Gonzalez… - Neuron, 2023 - cell.com
In fragile X syndrome (FX), the leading monogenic cause of autism, excessive neuronal
protein synthesis is a core pathophysiology; however, an overall increase in protein …

[HTML][HTML] Auditory dysfunction in animal models of autism spectrum disorder

AC Castro, P Monteiro - Frontiers in Molecular Neuroscience, 2022 - frontiersin.org
Autism spectrum disorder (ASD) is a neurodevelopmental disorder mainly characterized by
social-communication impairments, repetitive behaviors and altered sensory perception …

[HTML][HTML] Neural correlates of auditory hypersensitivity in fragile X syndrome

KA Razak, DK Binder, IM Ethell - Frontiers in Psychiatry, 2021 - frontiersin.org
The mechanisms underlying the common association between autism spectrum disorders
(ASD) and sensory processing disorders (SPD) are unclear, and treatment options to reduce …

[HTML][HTML] Minocycline treatment reverses sound evoked EEG abnormalities in a mouse model of fragile X syndrome

JW Lovelace, IM Ethell, DK Binder… - Frontiers in …, 2020 - frontiersin.org
Fragile X Syndrome (FXS) is a leading known genetic cause of intellectual disability. Many
symptoms of FXS overlap with those in autism including repetitive behaviors, language …

[HTML][HTML] Auditory processing in rodent models of autism: a systematic review

M Wilde, L Constantin, PR Thorne… - Journal of …, 2022 - Springer
Autism is a complex condition with many traits, including differences in auditory sensitivity.
Studies in human autism are plagued by the difficulty of controlling for aetiology, whereas …

Axonal and presynaptic FMRP: Localization, signal, and functional implications

X Wang, D Sela-Donenfeld, Y Wang - Hearing research, 2023 - Elsevier
Fragile X mental retardation protein (FMRP) binds a selected set of mRNAs and proteins to
guide neural circuit assembly and regulate synaptic plasticity. Loss of FMRP is responsible …

Acute and repeated administration of NLX-101, a selective serotonin-1A receptor biased agonist, reduces audiogenic seizures in developing Fmr1 knockout mice

X Tao, A Newman-Tancredi, MA Varney, KA Razak - Neuroscience, 2023 - Elsevier
Abstract Fragile X Syndrome (FXS) is a leading known genetic cause of Autism Spectrum
Disorders (ASD) and intellectual disability. A consistent and debilitating phenotype of FXS is …