Mutations in SYNE1 and SYNE2, that code for the mammalian orthologs of ANC‐1 (nesprin 1 and nesprin 2), are accountable for the development of two diseases: the neurodegenerative disorder SCAR8, which shares similarities with ALS and Emery‐Dreifuss Muscular Dystrophy (Janin et al., 2017). The gene discussed is SYNE1; the disease is autosomal recessive ataxia, Beauce type.