B6.129P2(C)-Mecp2tm1.1Bird/J (Rett Syndrome Model)


The MeCP2 null mouse develops neurological deficits at 3-8 weeks that encompass aspects of the neurological and physical symptoms observed in Rett Syndrome. Clinical presentation manifests as a combination of hindlimb clasping, uneven breathing, tremor, gait, and general malaise that are scored to assess phenotypic severity. The average lifespan of MeCP2-/- mice is 50-60 days. The efficacy of candidate therapeutics for Rett Syndrome can be assessed using this model in combination with plethysmography, grip strength, voluntary wheel, and foot slip functional assessments

Related Information

Guy, J., Gan, J., Selfridge, J., Cobb, S. & Bird, A. Reversal of neurological defects in a mouse model of Rett syndrome. Sci. 315, 1143–7 (2007).

Guy, J., Hendrich, B., Holmes, M., Martin, J. E. & Bird, A. A mouse Mecp2-null mutation causes neurological symptoms that mimic Rett syndrome. Nat. Genet. 27, 322–326 (2001).