Disease-associated oligodendrocyte signatures have been described broadly throughout both polygenic and monogenic neurodegenerative diseases, including SCA3 (Ramani et al., 2017; Haas et al., 2021), other polyQ diseases such as Huntington’s disease (HD) (Yamada et al., 2001; Huang et al., 2015; Ferrari Bardile et al., 2019), and more common neurodegenerative diseases, including Alzheimer’s disease (AD), Parkinson’s disease (PD), and Amyotrophic Lateral Sclerosis (ALS) (Philips et al., 2013; Ferrari Bardile et al., 2019; Errea and Rodriguez-Oroz, 2021; Kenigsbuch et al., 2022). This evidence concerns the gene ATXN3 and amyotrophic lateral sclerosis.