Interestingly, investigations performed in R6/2 and 140 CAG knock-in HD mouse models revealed that the Golgi protein acyl-CoA binding domain containing 3 (ACBD3) and the huntingtin-associated protein 1 (Hap1) oppositely modulated Rhes E3 ligase activity, either increasing or reducing Rhes-mediated SUMOylation of mHtt [38,39]. The gene discussed is ACBD3; the disease is Huntington disease.