The levels of the autophagy marker proteins SQSTM1 and LC3B were altered in SCA2 neuronal cells and lentiviral mouse models, and abnormal accumulation of SQSTM1 and LC3B was observed in the cerebellum and striatum of patients with SCA2, indicating that impaired autophagy may play an important role in the pathogenesis of SCA2 (Marcelo et al., 2021). Here, SQSTM1 is linked to spinocerebellar ataxia type 2.