FVB/N-Tg(MECP2/EGFP)1Hzo/J

Stock number: 026043
Product name: MeCP2-EGFP
Research areas: Neurobiology Research, Research Tools

Description

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

Detailed description

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. 005439), these mice rescue the Mecp2 null phenotype.

Development

PAC (PAC671D9), containing the entire human MECP2 locus and its essential regulatory elements, was C-terminally tagged with EGFP. The 99kb PAC was linearized and separated from the backbone by pulse field gel electrophoresis before injection into FVB single cell zygotes. This strain was maintained on an FVB background by the donating lab.

Allele

Symbol: Tg(MECP2/EGFP)1Hzo
Name: transgene insertion 1, Huda Y Zoghbi
MGI accession ID: MGI:5588443
Generation method: Transgenic
Attributes: Reporter; Inserted expressed sequence; Humanized sequence
Marker symbol: Tg(MECP2/EGFP)1Hzo
Marker name: transgene insertion 1, Huda Y Zoghbi
Chromosome: UN
Strain of origin: FVB/N
Expressed genes: GFP,Green Fluorescent Protein,
Site of expression: MECP2 and GFP are expressed in the brain.
Molecular note: The transgenic construct contains a human PAC (PAC671D9) with the entire human MECP2 locus and its essential regulatory elements and a C-terminal GFP tag.