Cognitive reserve in model systems for mechanistic discovery: The importance of longitudinal studies

JA McQuail, AR Dunn, Y Stern, CA Barnes… - Frontiers in aging …, 2021 - frontiersin.org
The goal of this review article is to provide a resource for longitudinal studies, using animal
models, directed at understanding and modifying the relationship between cognition and …

Adaptive control of synaptic plasticity integrates micro-and macroscopic network function

DN Scott, MJ Frank - Neuropsychopharmacology, 2023 - nature.com
Synaptic plasticity configures interactions between neurons and is therefore likely to be a
primary driver of behavioral learning and development. How this microscopic-macroscopic …

Rescue of oxytocin response and social behaviour in a mouse model of autism

H Hörnberg, E Pérez-Garci, D Schreiner… - Nature, 2020 - nature.com
A fundamental challenge in developing treatments for autism spectrum disorders is the
heterogeneity of the condition. More than one hundred genetic mutations confer high risk for …

[HTML][HTML] Cell-type-specific profiling of human cellular models of fragile X syndrome reveal PI3K-dependent defects in translation and neurogenesis

N Raj, ZT McEachin, W Harousseau, Y Zhou, F Zhang… - Cell reports, 2021 - cell.com
Transcriptional silencing of the FMR1 gene in fragile X syndrome (FXS) leads to the loss of
the RNA-binding protein FMRP. In addition to regulating mRNA translation and protein …

Functional and molecular characterization of a non-human primate model of autism spectrum disorder shows similarity with the human disease

S Watanabe, T Kurotani, T Oga, J Noguchi… - Nature …, 2021 - nature.com
Autism spectrum disorder (ASD) is a multifactorial disorder with characteristic synaptic and
gene expression changes. Early intervention during childhood is thought to benefit …

Input-output relationship of CA1 pyramidal neurons reveals intact homeostatic mechanisms in a mouse model of fragile X syndrome

SA Booker, LS De Oliveira, NJ Anstey, Z Kozic… - Cell Reports, 2020 - cell.com
Cellular hyperexcitability is a salient feature of fragile X syndrome animal models. The
cellular basis of hyperexcitability and how it responds to changing activity states is not fully …

An iPSC-derived astrocyte model of fragile X syndrome exhibits dysregulated cholesterol homeostasis

K Talvio, VA Wagner, R Minkeviciene… - Communications …, 2023 - nature.com
Cholesterol is an essential membrane structural component and steroid hormone precursor,
and is involved in numerous signaling processes. Astrocytes regulate brain cholesterol …

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 …

Astroglial Kir4. 1 potassium channel deficit drives neuronal hyperexcitability and behavioral defects in Fragile X syndrome mouse model

D Bataveljic, H Pivonkova, V de Concini… - Nature …, 2024 - nature.com
Fragile X syndrome (FXS) is an inherited form of intellectual disability caused by the loss of
the mRNA-binding fragile X mental retardation protein (FMRP). FXS is characterized by …

Selective inhibition of glycogen synthase kinase 3α corrects pathophysiology in a mouse model of fragile X syndrome

PK McCamphill, LJ Stoppel, RK Senter… - Science translational …, 2020 - science.org
Fragile X syndrome is caused by FMR1 gene silencing and loss of the encoded fragile X
mental retardation protein (FMRP), which binds to mRNA and regulates translation. Studies …