Huntington's disease and dentatorubral-pallidoluysian atrophy (DLPRA) are related polyQ disorders with increased IP3R1 sensitivity and reduced IP3R1 abundance (Liu et al., 2009; Datta et al., 2011; Euler et al., 2012; Suzuki et al., 2012). This evidence concerns the gene ITPR1 and juvenile Huntington disease.