STAU1 becomes highly overabundant in multiple human neurological diseases, including amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), Parkinson’s disease, Alzheimer’s disease and spinocerebellar ataxia type 2 (SCA2) [14, 16–24]; and also increases in response to acute stress [17–20, 22, 23]. Here, STAU1 is linked to spinocerebellar ataxia type 2.