In some cases, mutations in specific RBPs are the cause of neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), which can be caused by mutations in several RBPs, including TIA1 cytotoxic granule-associated RNA binding protein (TIA1), FUS RNA binding protein (FUS), TAR DNA binding protein (TARDBP/TPD-43), and others [24,25,26,27,28], spinocerebellar ataxia type 2 (SCA2), which is caused by a triplet expansion in Ataxin 2 (ATXN2) gene [29,30,31], and Fronto-temporal dementia (FTD), which is caused by mutations in TARDBP [32,33]. Here, SRRM2 is linked to spinocerebellar ataxia type 2.