MeCP2_E1 N-terminal modifications affect its degradation rate and are disrupted by the Ala2Val Rett mutation

TI Sheikh, AM de Paz, S Akhtar, J Ausio… - Human molecular …, 2017 - academic.oup.com
Abstract Methyl CpG-binding protein 2 (MeCP2), the mutated protein in Rett syndrome
(RTT), is a crucial chromatin-modifying and gene-regulatory protein that has two main …

Rett syndrome: a surprising result of mutation in MECP2

J Dragich, I Houwink-Manville… - Human molecular …, 2000 - academic.oup.com
The identification of mutations in the gene encoding methyl CpG binding protein 2 (MeCP2)
in Rett syndrome represents a major advance in the field. The current model predicts that …

Methyl-CpG-binding protein 2 mutations in Rett syndrome

IB Van den Veyver, HY Zoghbi - Current opinion in genetics & development, 2000 - Elsevier
The X-linked methyl-CpG-binding protein 2 gene (MECP2) encodes a protein that links DNA
methylation to transcriptional repression mediated by histone deacetylases. Mutations in …

Sequence‐specific DNA binding by AT‐hook motifs in Me CP 2

MJ Lyst, J Connelly, C Merusi, A Bird - FEBS letters, 2016 - Wiley Online Library
Me CP 2 is a chromatin‐associated protein that is mutated in Rett syndrome. Its methyl‐CpG‐
binding domain interacts with DNA containing methylated cytosine, but other modes of …

MeCP2: the genetic driver of Rett syndrome epigenetics

KV Good, JB Vincent, J Ausió - Frontiers in Genetics, 2021 - frontiersin.org
Mutations in methyl CpG binding protein 2 (MeCP2) are the major cause of Rett syndrome
(RTT), a rare neurodevelopmental disorder with a notable period of developmental …

Rett syndrome: a complex disorder with simple roots

MJ Lyst, A Bird - Nature Reviews Genetics, 2015 - nature.com
Rett syndrome (RTT) is a severe neurological disorder caused by mutations in the X-linked
gene MECP2 (methyl-CpG-binding protein 2). Two decades of research have fostered the …

Paradoxical role of methyl-CpG-binding protein 2 in Rett syndrome

JM LaSalle - Current Topics in Developmental Biology, 2004 - Elsevier
Publisher Summary This chapter introduces Rett Syndrome (RTT), which is a
neurodevelopmental disorder representing one of the most frequent causes of severe …

MeCP2 dysfunction in Rett syndrome and related disorders

P Moretti, HY Zoghbi - Current opinion in genetics & development, 2006 - Elsevier
Rett syndrome, a neurodevelopmental disorder caused by mutations in the X-linked gene
encoding methyl-CpG-binding protein 2 (MeCP2), is a leading cause of mental retardation …

DNA binding selectivity of MeCP2 due to a requirement for A/T sequences adjacent to methyl-CpG

RJ Klose, SA Sarraf, L Schmiedeberg, SM McDermott… - Molecular cell, 2005 - cell.com
DNA methylation is interpreted by a family of methyl-CpG binding domain (MBD) proteins
that repress transcription through recruitment of corepressors that modify chromatin. To …

Molecular context-dependent effects induced by Rett syndrome-associated mutations in MeCP2

D Ortega-Alarcon, R Claveria-Gimeno, S Vega… - Biomolecules, 2020 - mdpi.com
Methyl-CpG binding protein 2 (MeCP2) is a transcriptional regulator and a chromatin-
binding protein involved in neuronal development and maturation. Loss-of-function …