ATP1A3 is highly expressed in neurons of the adult rodent brain (McGrail et al., 1991; Pietrini et al., 1992; Bottger et al., 2011) and mutations in the gene have been implicated in three autosomal dominant Mendelian diseases, including alternating hemiplegia of childhood (AHC) type 2 (Rosewich et al., 2012), CAPOS syndrome (Demos et al., 2014), and Dystonia-12 (Anselm et al., 2009). This evidence concerns the gene ATP1A3 and Rapid-onset dystonia-parkinsonism.