ATXN2 and frontotemporal dementia: We had previously found that autophagy is abnormally inhibited in SCA2 patient fibroblasts and cerebellar and spinal cord tissues of SCA2 mouse models, as well as ALS and frontotemporal dementia (FTD) fibroblasts and mouse models associated with overabundance of the RNA binding protein STAU1 (Paul et al., 2018, 2021, 2023).