Until recently, the function of ATXN2 was unknown, but its involvement in neurodegeneration through genetic variants leading to spinocerebellar ataxia type 2 and ALS, led to several discoveries highlighting its function in RNA processing, mRNA stability, and translation.[67, 68] In addition, loss‐of‐function variants revealed their importance in the stress‐related induction of stress granules that protect vulnerable mRNA from decay.[69] Further, STAU2 was found to be decreased in elderly individuals. Here, ATXN2 is linked to spinocerebellar ataxia type 2.