Myotonic dystrophy type 2 (DM2, OMIM 602668) is a multi-systemic autosomal dominant disease that displays a wide spectrum of clinical manifestations, including proximal myotonia, degeneration of muscle fibers, cataracts, defective cardiac conduction, insulin resistance, and other endocrine disorders [1,2]. Here, INS is linked to myotonic dystrophy type 2.