Unusual characteristics of the DNA binding domain of epigenetic regulatory protein MeCP2 determine its binding specificity

S Khrapunov, C Warren, H Cheng, ER Berko… - Biochemistry, 2014 - ACS Publications
The protein MeCP2 mediates epigenetic regulation by binding methyl-CpG (mCpG) sites on
chromatin. MeCP2 consists of six domains of which one, the methyl binding domain (MBD) …

Partial rescue of MeCP2 deficiency by postnatal activation of MeCP2

E Giacometti, S Luikenhuis, C Beard… - Proceedings of the …, 2007 - National Acad Sciences
In humans, mutations in the X-linked MECP2 gene, are the cause of Rett syndrome (RTT), a
neurodevelopmental disorder that affects mainly girls. MeCP2 binds to methylated CpGs …

The role of MeCP2 in the brain

J Guy, H Cheval, J Selfridge… - Annual review of cell and …, 2011 - annualreviews.org
Methyl-CpG binding protein 2 (MeCP2) was first identified in 1992 as a protein that binds
specifically to methylated DNA. Mutations in the MECP2 gene were later found to be the …

[HTML][HTML] Solution structure of the methyl-CpG binding domain of human MBD1 in complex with methylated DNA

I Ohki, N Shimotake, N Fujita, JG Jee, T Ikegami… - Cell, 2001 - cell.com
In vertebrates, the biological consequences of DNA methylation are often mediated by
protein factors containing conserved methyl-CpG binding domains (MBDs). Mutations in the …

MeCP2 in Rett syndrome: transcriptional repressor or chromatin architectural protein?

LH Chadwick, PA Wade - Current opinion in genetics & development, 2007 - Elsevier
Rett syndrome is a progressive neurological disorder caused by mutations in the methyl-
DNA binding protein MeCP2. The longstanding model depicting MeCP2 as a transcriptional …

Regulation of RNA splicing by the methylation-dependent transcriptional repressor methyl-CpG binding protein 2

JI Young, EP Hong, JC Castle… - Proceedings of the …, 2005 - National Acad Sciences
Rett syndrome (RTT) is a postnatal neurodevelopmental disorder characterized by the loss
of acquired motor and language skills, autistic features, and unusual stereotyped …

[PDF][PDF] RETRACTED: A Mutant Form of MeCP2 Protein Associated with Human Rett Syndrome Cannot Be Displaced from Methylated DNA by Notch in Xenopus …

I Stancheva, AL Collins, IB Van den Veyver, H Zoghbi… - Molecular cell, 2003 - cell.com
MeCP2 is a DNA binding protein that represses transcription of methylated genes in vitro,
but the endogenous function of MeCP2 in vivo is unclear. Here, we demonstrate that in …

MeCP2 and Rett syndrome: reversibility and potential avenues for therapy

KKE Gadalla, MES Bailey, SR Cobb - Biochemical Journal, 2011 - portlandpress.com
Mutations in the X-linked gene MECP2 (methyl CpG-binding protein 2) are the primary
cause of the neurodevelopmental disorder RTT (Rett syndrome), and are also implicated in …

Rett syndrome: the complex nature of a monogenic disease

A Renieri, I Meloni, I Longo, F Ariani, F Mari… - Journal of molecular …, 2003 - Springer
Rett syndrome (RTT) is a severe neurodevelopmental disorder affecting almost exclusively
girls. It is currently considered a monogenic X-linked dominant disorder due to mutations in …

Heterogeneity in residual function of MeCP2 carrying missense mutations in the methyl CpG binding domain

S Kudo, Y Nomura, M Segawa, N Fujita… - Journal of medical …, 2003 - jmg.bmj.com
Rett syndrome is a neurodevelopmental disorder with severe mental retardation caused by
mutations in the MECP2 gene. Mutations in the MECP2 gene are also associated with other …