MECP2 (Methyl-CpG-binding protein 2) variants and mutations

MECP2 (also known as Methyl-CpG-binding protein 2) is a human protein-coding gene encoding a methyl-CpG-binding protein 2 protein. It interprets DNA methylation and organizes transcriptional and chromatin states that are especially important in mature neurons. Loss-of-function variants cause Rett syndrome, whereas increased dosage causes MECP2 duplication syndrome. This analysis covers 1,324 MECP2 variants and mutations. Of these, 81% have computational variant effect predictions. Disease context includes Rett syndrome, X-linked intellectual disability-psychosis-macroorchidism syndrome, and severe neonatal-onset encephalopathy with microcephaly. Example MECP2 variants include M1L, M5I, and G7E.

Variant analysis overview

Variant and mutation evidence

Clinical, disease, and population context

Protein structure and variant hotspots

Data sources

Evidence in this analysis draws on EBI Proteins Variation, UniProt, gnomAD v4, EuropePMC, 3D Hotspot Analysis, Interaction Network Analysis, Protein Data Bank, AlphaFold DB, gnomAD constraint, Open Targets, ClinGen, MaveDB, LitVar.

Notable MECP2 variants

Examples include M1L, M5I, G7E, L8P, R9S, R9T, R9X, E10*. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.