Mutations in genes encoding LINC complex proteins are known to cause skeletal myopathy and cardiomyopathy, called Emery–Dreifuss muscular dystrophy (Heller et al., 2020), and defects in nuclear structure are often observed in mice with mutations of LINC and LINC-associated molecules such as lamin A (Lmna), nesprin, ezrin, and Des (Nikolova et al., 2004; Banerjee et al., 2014; Wada et al., 2019; Heffler et al., 2020). The gene discussed is DES; the disease is Skeletal myopathy.