MECP2 (methyl CpG binding protein 2) is an X-linked transcriptional repressor that binds to methylated CpG dinucleotides. Loss of function causes the progressive neurological disorder Rett Syndrome (RTT). Conversely, duplication or triplication of the chromosomal region causes an equally wide-ranging progressive neurological disorder, MECP2 duplication syndrome, whose features overlap somewhat with RTT.

This transgenic strain expresses full-length, wildtype human MECP2 fused to EGFP. Immunofluorescent methods using anti-MECP2 and anti-GFP antibodies reveal expression patterns throughout the brain that parallel those of the endogenous gene. Transgene expression levels are similar to those of the endogenous X-linked mouse gene, thus double the total MECP2 level in these animals. 

When put on a Mecp2-/y background (see Stock No. <a href="https://www.jax.org/strain/005439">005439</a>), these mice rescue the Mecp2 null phenotype.

Mice hemizygous for human MECP2 tagged with EGFP are a model of MECP2 duplication syndrome.

Typically mice are recovered in 12-16 weeks. Contact Customer Service to place an order or for more information.

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