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Glial Dysfunction in MeCP2 Deficiency Models: Implications for Rett Syndrome

Rett syndrome (RTT) is a rare, X-linked neurodevelopmental disorder typically affecting females, resulting in a range of symptoms including autistic features, intellectual impairment, motor deterioration, and autonomic abnormalities. RTT is primarily caused by the genetic mutation of the Mecp2 gene....

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Detalles Bibliográficos
Autores principales: Kahanovitch, Uri, Patterson, Kelsey C., Hernandez, Raymundo, Olsen, Michelle L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6696322/
https://www.ncbi.nlm.nih.gov/pubmed/31387202
http://dx.doi.org/10.3390/ijms20153813
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author Kahanovitch, Uri
Patterson, Kelsey C.
Hernandez, Raymundo
Olsen, Michelle L.
author_facet Kahanovitch, Uri
Patterson, Kelsey C.
Hernandez, Raymundo
Olsen, Michelle L.
author_sort Kahanovitch, Uri
collection PubMed
description Rett syndrome (RTT) is a rare, X-linked neurodevelopmental disorder typically affecting females, resulting in a range of symptoms including autistic features, intellectual impairment, motor deterioration, and autonomic abnormalities. RTT is primarily caused by the genetic mutation of the Mecp2 gene. Initially considered a neuronal disease, recent research shows that glial dysfunction contributes to the RTT disease phenotype. In the following manuscript, we review the evidence regarding glial dysfunction and its effects on disease etiology.
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spelling pubmed-66963222019-09-05 Glial Dysfunction in MeCP2 Deficiency Models: Implications for Rett Syndrome Kahanovitch, Uri Patterson, Kelsey C. Hernandez, Raymundo Olsen, Michelle L. Int J Mol Sci Review Rett syndrome (RTT) is a rare, X-linked neurodevelopmental disorder typically affecting females, resulting in a range of symptoms including autistic features, intellectual impairment, motor deterioration, and autonomic abnormalities. RTT is primarily caused by the genetic mutation of the Mecp2 gene. Initially considered a neuronal disease, recent research shows that glial dysfunction contributes to the RTT disease phenotype. In the following manuscript, we review the evidence regarding glial dysfunction and its effects on disease etiology. MDPI 2019-08-05 /pmc/articles/PMC6696322/ /pubmed/31387202 http://dx.doi.org/10.3390/ijms20153813 Text en © 2019 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Kahanovitch, Uri
Patterson, Kelsey C.
Hernandez, Raymundo
Olsen, Michelle L.
Glial Dysfunction in MeCP2 Deficiency Models: Implications for Rett Syndrome
title Glial Dysfunction in MeCP2 Deficiency Models: Implications for Rett Syndrome
title_full Glial Dysfunction in MeCP2 Deficiency Models: Implications for Rett Syndrome
title_fullStr Glial Dysfunction in MeCP2 Deficiency Models: Implications for Rett Syndrome
title_full_unstemmed Glial Dysfunction in MeCP2 Deficiency Models: Implications for Rett Syndrome
title_short Glial Dysfunction in MeCP2 Deficiency Models: Implications for Rett Syndrome
title_sort glial dysfunction in mecp2 deficiency models: implications for rett syndrome
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6696322/
https://www.ncbi.nlm.nih.gov/pubmed/31387202
http://dx.doi.org/10.3390/ijms20153813
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