Although the toxicity of a SCA2 pathogenic variant with a long expanded CAG repeat has not been tested in zebrafish yet, its variant ATXN2[30Q] carrying the intermediate CAG length of 30Q was examined in zebrafish especially in the paradigm of amyotrophic lateral sclerosis and frontotemporal dementia (ALS-FTD) [97]. Here, ATXN2 is linked to frontotemporal dementia.