Despite the fact that several mouse models have already been generated for DYT1 dystonia, findings are inconsistent and the role(s) of mutant torsinA in the development of DYT1 dystonia and the physiological function(s) of this protein in its active or native state are still not well understood (Dang et al., 2005; Goodchild and Dauer, 2004; Goodchild et al., 2005; Grundmann et al., 2007; Sharma et al., 2005; Shashidharan et al., 2005). This evidence concerns the gene TOR1A and early-onset generalized limb-onset dystonia.