Learning and behavioral deficits associated with the absence of the fragile X mental retardation protein: what a fly and mouse model can teach us.

Détails

ID Serval
serval:BIB_E5AB8DDD776F
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Synthèse (review): revue aussi complète que possible des connaissances sur un sujet, rédigée à partir de l'analyse exhaustive des travaux publiés.
Collection
Publications
Titre
Learning and behavioral deficits associated with the absence of the fragile X mental retardation protein: what a fly and mouse model can teach us.
Périodique
Learning & memory (Cold Spring Harbor, N.Y.)
Auteur(s)
Santos A.R., Kanellopoulos A.K., Bagni C.
ISSN
1549-5485 (Electronic)
ISSN-L
1072-0502
Statut éditorial
Publié
Date de publication
10/2014
Volume
21
Numéro
10
Pages
543-555
Langue
anglais
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't ; Review
Publication Status: epublish
Résumé
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 expansion that inhibits the expression of the FMR1 gene. The gene product, the Fragile X Mental Retardation Protein (FMRP), regulates mRNA metabolism in brain and nonneuronal cells. During brain development, FMRP controls the expression of key molecules involved in receptor signaling, cytoskeleton remodeling, protein synthesis and, ultimately, spine morphology. Symptoms associated with FXS include neurodevelopmental delay, cognitive impairment, anxiety, hyperactivity, and autistic-like behavior. Twenty years ago the first Fmr1 KO mouse to study FXS was generated, and several years later other key models including the mutant Drosophila melanogaster, dFmr1, have further helped the understanding of the cellular and molecular causes behind this complex syndrome. Here, we review to which extent these biological models are affected by the absence of FMRP, pointing out the similarities with the observed human dysfunction. Additionally, we discuss several potential treatments under study in animal models that are able to partially revert some of the FXS abnormalities.

Mots-clé
Animals, Anxiety/genetics, Circadian Rhythm/genetics, Disease Models, Animal, Drosophila Proteins/genetics, Drosophila melanogaster, Fragile X Mental Retardation Protein/genetics, Fragile X Syndrome/genetics, Fragile X Syndrome/physiopathology, Humans, Learning/physiology, Mice, Mice, Knockout, Sensory Gating/genetics, Signal Transduction/genetics, Social Behavior
Pubmed
Open Access
Oui
Création de la notice
06/03/2017 17:23
Dernière modification de la notice
20/08/2019 16:09
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