In this framework, it was shown that the loss of the Mecp2 gene in a Rett syndrome mouse model led to the impairment of the nigrostriatal pathways and motor deficits, which include reduced dopamine release after striatal stimulation and defects in the D2 dopamine receptor-dependent electrophysiological activity of DA neurons [207]. This evidence concerns the gene MECP2 and atypical Rett syndrome.