Previous data have shown that the mammalian target of rapamycin inhibitor temsirolimus and n-Butylidenephthalide, through the upregulation of autophagy, reduced polyQ disease-causing protein aggregates in brain neurons and alleviated motor impairment in mouse models of Huntington’s disease and SCA3 [10,11,28]. Here, ATXN3 is linked to Huntington disease.