MeCP2 deficiency results in robust Rett-like behavioural and motor deficits in male and female rats.

ABSTRACT: Since the identification of MECP2 as the causative gene in the majority of Rett Syndrome (RTT) cases, transgenic mouse models have played a critical role in our understanding of this disease. The use of additional mammalian RTT models offers the promise of further elucidating critical early mechanisms of disease as well as providing new avenues for translational studies. We have identified significant abnormalities in growth as well as motor and behavioural function in a novel zinc-finger nuclease model of RTT utilizing both male and female rats throughout development. Male rats lacking MeCP2 (Mecp2^ZFN/y) were noticeably symptomatic as early as postnatal day 21, with most dying by postnatal day 55, while females lacking one copy of Mecp2 (Mecp2^ZFN/+) displayed a more

SUBMITTER: Patterson KC 

PROVIDER: S-EPMC5179928 | biostudies-literature | 2016 Aug

REPOSITORIES: biostudies-literature