Glial Dysfunction in MeCP2 Deficiency Models: Implications for Rett Syndrome

TR Number
Date
2019-08-05
Journal Title
Journal ISSN
Volume Title
Publisher
MDPI
Abstract

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.

Description
Keywords
astrocytes, oligodendrocytes, microglia
Citation
Kahanovitch, U.; Patterson, K.C.; Hernandez, R.; Olsen, M.L. Glial Dysfunction in MeCP2 Deficiency Models: Implications for Rett Syndrome. Int. J. Mol. Sci. 2019, 20, 3813.