In order to understand the effects of the behavioral and biological phenotypes in RTT relevant to the MeCP2 mutations, researchers have experimentally manipulated the MeCP2 gene for RTT in animal models (Calfa et al, 2011). Animal paradigms that are utilized for clinical research are usually developed to meticulously study the core mechanism associated with a particular syndrome (Chahrour et al, 2011). The significance of animal models, in turn, is reliant upon its resemblance to the syndrome, so knowing this, researchers have developed three different mouse models of RTT, each with different mutation type and phenotypic features. (Young & Zoghbi, 2004). These mouse models of RTT include the MeCP2 knockout, models expressing high levels of MeCP2, and …show more content…
The C57BL/6J mouse is the perfect strain to evaluate the effect of RTT because of easy breeding and its frequent usage for genetic modifications (Young & Zoghbi, 2004).
The MeCP2 knockout (KO) strain is a common mouse model used to study human RTT. These KO models are created by knocking out exon 3 or both exons 3 and 4 of the targeted gene on the X chromosome which then leads to a deletion of the MeCP2 gene. These MeCP2 (KO) male mouse displays a number of motor problems and a range of RTT-like characteristics, which makes them useful in studying RTT (Calfa et al, 2011). The symptoms seen in this model are reflective of what is seen in human RTT. These MeCP2 KO models seem typical developing at birth, but severe neurological dysfunction such as poor coordination and seizures are detectable at 3 weeks and gets worse with age (Chen et al, 2001). Hind limb clasping and irregularities in respiratory function is also noted (Chen et al, 2001). KO mice usually live for approximately 8-10 weeks, and experience decreased weight and prolonged motor problems when compared to their