ITPR1 and Dentatorubral pallidoluysian atrophy: In fact, Liu et al. reported that the mutant Ataxin-1 protein and polyQ-expanded atrophin-1, the protein mutated in Dentatorubral-pallidoluysian atrophy (DRPLA), both associate with the C-terminal of IP3R1 [10], though IP3R1 supersensitivity has not yet been assessed experimentally in either disease.