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

Bredford Kerr, Matías Alvarez-saavedra, Mauricio A. Sáez, Alexandra Saona, Juan I. Young

Resultado de la investigación: Contribución a una revistaArtículo

52 Citas (Scopus)

Resumen

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 (Mecp2lox) 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.

Idioma originalInglés
Páginas (desde-hasta)1707-1717
Número de páginas11
PublicaciónHuman Molecular Genetics
Volumen17
N.º12
DOI
EstadoPublicada - 15 jun 2008

Áreas temáticas de ASJC Scopus

  • Biología molecular
  • Genética
  • Genética (clínica)

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