Future elucidation of Nesprin-1 isoforms specific functions, not only in muscle but also in other cell types including neurons, will allow understanding the mechanisms underlying the numerous Nesprin-1-associated pathologies including Emery Dreifuss muscular dystrophy and autosomal recessive cerebelar ataxia. Here, SYNE1 is linked to Emery-Dreifuss muscular dystrophy.