Rett syndrome and environmental enrichment as a potential therapy for attenuating the pathology

Rett syndrome (RTT) is a neurological disorder affecting the development of the central nervous system and one of the leading causes of mental retardation among young women. RTT patients exhibit microcephaly, decreased neuronal size, shorter cortical dendrite, and a reduced dendritic spine density;...

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Autores principales: Tapia, Pablo, Kouro, Ricardo, Pérez, Marcos, Torres, Rodrigo, Espinoza, Sofía, Kerr, Bredford
Formato: Articulo Revision
Lenguaje:Inglés
Publicado: 2019
Materias:
Ciencias Médicas
Rett syndrome
RTT-like phenotype
Acceso en línea:http://sedici.unlp.edu.ar/handle/10915/126795
https://pmr.safisiol.org.ar/archive/id/114
Aporte de:
SEDICI (UNLP) de Universidad Nacional de La Plata
id I19-R120-10915-126795
record_format dspace
institution Universidad Nacional de La Plata
institution_str I-19
repository_str R-120
collection SEDICI (UNLP)
language Inglés
topic Ciencias Médicas
Rett syndrome
RTT-like phenotype
spellingShingle Ciencias Médicas
Rett syndrome
RTT-like phenotype
Tapia, Pablo
Kouro, Ricardo
Pérez, Marcos
Torres, Rodrigo
Espinoza, Sofía
Kerr, Bredford
Rett syndrome and environmental enrichment as a potential therapy for attenuating the pathology
topic_facet Ciencias Médicas
Rett syndrome
RTT-like phenotype
description Rett syndrome (RTT) is a neurological disorder affecting the development of the central nervous system and one of the leading causes of mental retardation among young women. RTT patients exhibit microcephaly, decreased neuronal size, shorter cortical dendrite, and a reduced dendritic spine density; evidence strongly suggesting that a synaptic disorder underlies the neurological RTT-associated phenotype. MECP2 is a transcription factor with multiple roles on gene expression, and mutations in its gene coding sequence have been identified as the major cause of RTT. The generation of transgenic mouse models lacking the expression of Mecp2 has allowed getting insight into the physiopathological events associated with the loss of a fully functional Mecp2 allele in RTT patients and it has been demonstrated that is possible to partially rescue, or reverse, the phenotype associated with RTT which opens a window to explore therapeutic approaches plausible to be utilized in RTT patients. Considering that RTT patients exhibit reduced neuronal plasticity and synaptic disorder, this mini-review is focused on studies demonstrating the positive effect of an enriched environment on the RTT-like phenotype exhibited by mouse models of the disease.
format Articulo
Revision
author Tapia, Pablo
Kouro, Ricardo
Pérez, Marcos
Torres, Rodrigo
Espinoza, Sofía
Kerr, Bredford
author_facet Tapia, Pablo
Kouro, Ricardo
Pérez, Marcos
Torres, Rodrigo
Espinoza, Sofía
Kerr, Bredford
author_sort Tapia, Pablo
title Rett syndrome and environmental enrichment as a potential therapy for attenuating the pathology
title_short Rett syndrome and environmental enrichment as a potential therapy for attenuating the pathology
title_full Rett syndrome and environmental enrichment as a potential therapy for attenuating the pathology
title_fullStr Rett syndrome and environmental enrichment as a potential therapy for attenuating the pathology
title_full_unstemmed Rett syndrome and environmental enrichment as a potential therapy for attenuating the pathology
title_sort rett syndrome and environmental enrichment as a potential therapy for attenuating the pathology
publishDate 2019
url http://sedici.unlp.edu.ar/handle/10915/126795
https://pmr.safisiol.org.ar/archive/id/114
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