Anna Pluciennik et al. discovered that the deubiquitinase USP7 selectively interacts with polyQ-expanded androgen receptor (AR) in spinal and bulbar muscular atrophy (SBMA) and that decreasing usP7 levels reduced mutant AR aggregation and cytotoxicity in cell models of SBMA, suppressed disease phenotypes in SBMA and spinocerebellar ataxia type 3 (SCA3) fly models, and improved several motor deficiencies in transgenic SBMA mice, highlighting the critical role of USP7 in the pathophysiology of SBMA and suggesting a similar role in SCA3 and HD [82]. The gene discussed is USP7; the disease is Spinocerebellar ataxia type 3.