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Structural and functional abnormalities in the olfactory system of fragile x syndrome models

dc.contributor.authorBodaleo F.
dc.contributor.authorTapia-Monsalves C.
dc.contributor.authorCea-Del Rio C.
dc.contributor.authorGonzalez-Billault C.
dc.contributor.authorNunez-Parra A.
dc.date.accessioned2020-09-02T22:13:12Z
dc.date.available2020-09-02T22:13:12Z
dc.date.issued2019
dc.identifier10.3389/fnmol.2019.00135
dc.identifier.citation12, , -
dc.identifier.issn16625099
dc.identifier.urihttps://hdl.handle.net/20.500.12728/3769
dc.descriptionFragile X Syndrome (FXS) is the most common inherited form of intellectual disability. It is produced by mutation of the Fmr1 gene that encodes for the Fragile Mental Retardation Protein (FMRP), an important RNA-binding protein that regulates the expression of multiple proteins located in neuronal synapses. Individuals with FXS exhibit abnormal sensory information processing frequently leading to hypersensitivity across sensory modalities and consequently a wide array of behavioral symptoms. Insects and mammals engage primarily their sense of smell to create proper representations of the external world and guide adequate decision-making processes. This feature in combination with the exquisitely organized neuronal circuits found throughout the olfactory system (OS) and the wide expression of FMRP in brain regions that process olfactory information makes it an ideal model to study sensory alterations in FXS models. In the last decade several groups have taken advantage of these features and have used the OS of fruit fly and rodents to understand neuronal alteration giving rise to sensory perception issues. In this review article, we will discuss molecular, morphological and physiological aspects of the olfactory information processing in FXS models. We will highlight the decreased inhibitory/excitatory synaptic balance and the diminished synaptic plasticity found in this system resulting in behavioral alteration of individuals in the presence of odorant stimuli. © 2019 Bodaleo, Tapia-Monsalves, Cea-Del Rio, Gonzalez-Billault and Nunez-Parra.
dc.language.isoen
dc.publisherFrontiers Media S.A.
dc.subjectDfmr1
dc.subjectExcitation/inhibition balance
dc.subjectFmr1-KO
dc.subjectFMRP
dc.subjectOlfactory behavior
dc.subjectOlfactory coding
dc.subjectStructural plasticity
dc.subjectataxin 2
dc.subjectcalcium calmodulin dependent protein kinase II
dc.subjectDff related protein 2
dc.subjectdisks large homolog 4
dc.subjectfragile X mental retardation protein
dc.subjectFutsch protein
dc.subjectMAP1B protein
dc.subjectmetabotropic receptor
dc.subjectmicrotubule associated protein 5
dc.subjectprotein ZC3H14
dc.subjectRNA binding protein
dc.subjectShrub protein
dc.subjectunclassified drug
dc.subjectDrosophila
dc.subjectfragile X syndrome
dc.subjectgene mutation
dc.subjecthippocampus
dc.subjecthuman
dc.subjectinformation processing
dc.subjectintellectual impairment
dc.subjectmemory disorder
dc.subjectnerve cell plasticity
dc.subjectneuromuscular junction
dc.subjectnonhuman
dc.subjectolfactory associative learning test
dc.subjectolfactory cortex
dc.subjectolfactory discrimination
dc.subjectolfactory epithelium
dc.subjectolfactory memory
dc.subjectolfactory nerve disease
dc.subjectolfactory receptor neuron
dc.subjectolfactory system
dc.subjectprotein expression
dc.subjectprotein phosphorylation
dc.subjectReview
dc.subjectshort term memory
dc.subjectsmelling
dc.subjecttranslation regulation
dc.titleStructural and functional abnormalities in the olfactory system of fragile x syndrome models
dc.typeReview


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