Role of DNA methyl-CpG-binding protein MeCP2 in Rett syndrome pathobiology and mechanism of disease

S Pejhan, M Rastegar - Biomolecules, 2021 - mdpi.com
Rett Syndrome (RTT) is a severe, rare, and progressive developmental disorder with
patients displaying neurological regression and autism spectrum features. The affected …

The epigenetic modification of DNA methylation in neurological diseases

L Li, R Chen, H Zhang, J Li, H Huang, J Weng… - Frontiers in …, 2024 - frontiersin.org
Methylation, a key epigenetic modification, is essential for regulating gene expression and
protein function without altering the DNA sequence, contributing to various biological …

Rett syndrome and CDKL5 deficiency disorder: from bench to clinic

SD Kadam, BJ Sullivan, A Goyal, ME Blue… - International journal of …, 2019 - mdpi.com
Rett syndrome (RTT) and CDKL5 deficiency disorder (CDD) are two rare X-linked
developmental brain disorders with overlapping but distinct phenotypic features. This review …

Dendrimer nanotherapy targeting of glial dysfunction improves inflammation and neurobehavioral phenotype in adult female Mecp2‐heterozygous mouse model of …

ES Khoury, RV Patel, C O'Ferrall… - Journal of …, 2023 - Wiley Online Library
Rett syndrome is an X‐linked neurodevelopmental disorder caused by mutation of Mecp2
gene and primarily affects females. Glial cell dysfunction has been implicated in in Rett …

MeCP2: an epigenetic regulator of critical periods

N Picard, M Fagiolini - Current opinion in neurobiology, 2019 - Elsevier
Highlights•MeCP2 regulates the timing of critical periods in sensory cortex.•MeCP2 binds
and drives expression of genes implicated in synaptic plasticity and the expression of …

Glial dysfunction in MeCP2 deficiency models: implications for Rett syndrome

U Kahanovitch, KC Patterson, R Hernandez… - International Journal of …, 2019 - mdpi.com
Rett syndrome (RTT) is a rare, X-linked neurodevelopmental disorder typically affecting
females, resulting in a range of symptoms including autistic features, intellectual impairment …

ANAVEX® 2-73 (blarcamesine), a Sigma-1 receptor agonist, ameliorates neurologic impairments in a mouse model of Rett syndrome

WE Kaufmann, J Sprouse, N Rebowe… - Pharmacology …, 2019 - Elsevier
Rett syndrome (RTT) is a severe neurodevelopmental disorder that is associated in most
cases with mutations in the transcriptional regulator MECP2. At present, there are no …

[HTML][HTML] Dendrimer-conjugated glutaminase inhibitor selectively targets microglial glutaminase in a mouse model of Rett syndrome

ES Khoury, A Sharma, RR Ramireddy, AG Thomas… - Theranostics, 2020 - ncbi.nlm.nih.gov
Background: Elevated glutamate production and release from glial cells is a common feature
of many CNS disorders. Inhibitors of glutaminase (GLS), the enzyme responsible for …

An early enriched experience drives targeted microglial engulfment of miswired neural circuitry during a restricted postnatal period

L Rogerson‐Wood, CS Goldsbury, A Sawatari… - Glia, 2024 - Wiley Online Library
Brain function is critically dependent on correct circuit assembly. Microglia are well‐known
for their important roles in immunological defense and neural plasticity, but whether they can …

Spatial and temporal gene function studies in rodents: towards gene-based therapies for autism spectrum disorder

IW Riemersma, R Havekes, MJH Kas - Genes, 2021 - mdpi.com
Autism spectrum disorder (ASD) is a complex neurodevelopmental condition that is
characterized by differences in social interaction, repetitive behaviors, restricted interests …