MECP2 Mouse Models

Why Model MECP2

Mecp2 is the mouse ortholog of human MECP2 and encodes methyl-CpG-binding protein 2, a transcriptional regulator that binds methylated DNA and is highly expressed in neurons. MeCP2 has been associated with regulation of gene expression in the nervous system.

MECP2 mutations cause Rett syndrome, and duplication of the gene causes MECP2 duplication syndrome; MECP2-directed gene therapies are under study. Mecp2-deficient and mutant mice reproduce many features of Rett syndrome.

Model Applications

Humanized MECP2 models, including those carrying disease-associated alleles, have been used to study MeCP2 biology and Rett syndrome and to evaluate MECP2-directed approaches in defined experimental settings.

Custom MECP2 Model Options

Engineered to your specifications, with molecular validation of the targeted allele and germline transmission guaranteed.

• Conventional knockout
• Conditional knockout
• Point mutation / knockin
• Reporter knockin
• Humanized
• Targeted transgenic

Sources

• Exploring the complexity of MECP2 function in Rett syndrome. Nat Rev Neurosci. 2025. doi:10.1038/s41583-025-00926-1
• Amir RE et al. Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2. Nat Genet. 1999. doi:10.1038/13810

Last reviewed: October 22, 2026 · Reviewed by: Paul Sheiffele

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