Studies have shown PGC-1α/SOD2/CYCS and NRF2/GCLC/NQO1 pathways are involved in pathogenesis of polyQ-mediated diseases including HD and SCA3 [18,20,23,24], and impaired NFYA/HSPA5 expression has been shown in SCA17, including cellular and animal models and lymphoblasts from patients [6,7,9]. Here, SOD2 is linked to spinocerebellar ataxia type 17.