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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
Descripción
Sumario: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.