Since mutations in the human orthologs of anc‐1, SYNE1/SYNE2, underlie the development of the adult‐onset neurodegenerative disorder spinocerebellar ataxia autosomal recessive 8 (SCAR8) (Beaudin, Gamache, Gros‐Louis, & Dupré, 1993), we focused our efforts on uncovering the mechanism by which ANC‐1 regulates proteostasis. Here, SYNE2 is linked to autosomal recessive ataxia, Beauce type.