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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....
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2019
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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. |
format | Online Article Text |
id | pubmed-6696322 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
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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