To directly examine the impact of activating autophagy by inhibiting GPCR signaling on the accumulation of expanded polyglutamine repeats and disease progression in animal models of expanded polyglutamine disorders, we used N171-82Q transgenic mice, a mouse model of Huntington's disease that expresses the N-terminal 171 amino acids of human htt with 82Q repeat under the control of the prion promoter (Schilling et al., 1999). Here, HTT is linked to juvenile Huntington disease.