Dysregulated Ca2+-Permeable AMPA Receptor Signaling in Neural Progenitors Modeling Fragile X Syndrome

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http://hdl.handle.net/10138/304489

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Danesi , C E , Keinänen , K P & Castren , M L 2019 , ' Dysregulated Ca2+-Permeable AMPA Receptor Signaling in Neural Progenitors Modeling Fragile X Syndrome ' , Frontiers in Synaptic Neuroscience , vol. 11 , 2 . https://doi.org/10.3389/fnsyn.2019.00002

Title: Dysregulated Ca2+-Permeable AMPA Receptor Signaling in Neural Progenitors Modeling Fragile X Syndrome
Author: Danesi, Claudia Elisabetta; Keinänen, Kari Pekka; Castren, Maija Liisa
Contributor: University of Helsinki, Department of Physiology
University of Helsinki, Molecular and Integrative Biosciences Research Programme
University of Helsinki, Department of Physiology
Date: 2019-02-08
Language: eng
Number of pages: 8
Belongs to series: Frontiers in Synaptic Neuroscience
ISSN: 1663-3563
URI: http://hdl.handle.net/10138/304489
Abstract: Fragile X syndrome (FXS) is a neurodevelopmental disorder that represents a common cause of intellectual disability and is a variant of autism spectrum disorder (ASD). Studies that have searched for similarities in syndromic and non-syndromic forms of ASD have paid special attention to alterations of maturation and function of glutamatergic synapses. Copy number variations (CNVs) in the loci containing genes encoding alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid receptors (AMPARs) subunits are associated with ASD in genetic studies. In FXS, dysregulated AMPAR subunit expression and trafficking affect neural progenitor differentiation and synapse formation and neuronal plasticity in the mature brain. Decreased expression of GluA2, the AMPAR subunit that critically controls Ca2+-permeability, and a concomitant increase in Ca2+-permeable AMPARs (CP-AMPARs) in human and mouse FXS neural progenitors parallels changes in expression of GluA2-targeting microRNAs (miRNAs). Thus, posttranscriptional regulation of GluA2 by miRNAs and subsequent alterations in calcium signaling may contribute to abnormal synaptic function in FXS and, by implication, in some forms of ASD.
Subject: AMPA
AUTISM SPECTRUM DISORDERS
DIFFERENTIATION
EXPRESSION
FMRP
GENE
GLUTAMATE RECEPTORS
GluA2
MENTAL-RETARDATION PROTEIN
MOUSE MODEL
SILENT SYNAPSES
SYNAPTIC PLASTICITY
autism
fragile X syndrome
miR-181
miRNA
neural progenitor
plasticity
3112 Neurosciences
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