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Publication : Defective body-weight regulation, motor control and abnormal social interactions in Mecp2 hypomorphic mice.

First Author  Kerr B Year  2008
Journal  Hum Mol Genet Volume  17
Issue  12 Pages  1707-17
PubMed ID  18321865 Mgi Jnum  J:135824
Mgi Id  MGI:3794507 Doi  10.1093/hmg/ddn061
Citation  Kerr B, et al. (2008) Defective body-weight regulation, motor control and abnormal social interactions in Mecp2 hypomorphic mice. Hum Mol Genet 17(12):1707-17
abstractText  MeCP2 is an abundant protein that binds to methylated cytosine residues in DNA and regulates transcription. Mutations in MECP2 cause Rett syndrome, a severe neurological disorder that affects approximately 1:10 000 females. Mice lacking MeCP2 have been generated and constitute important models of Rett syndrome. However, it is yet unclear whether certain physiological events are sensitive to a decrease, rather than a complete lack of MeCP2. Here we report that a Mecp2 floxed allele (Mecp2(lox)) that was generated to allow conditional mutagenesis behaves as a hypomorph and the corresponding mutant mice exhibit phenotypical alterations including body weight gain, motor abnormalities and altered social behavior. Our data reinforce the view that the central nervous system is extremely sensitive to MeCP2 expression levels and suggest that the 3'-UTR of Mecp2 might contain important elements that contribute to the regulation of its stability or processing.
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