For example, MYH7 protein is located in sarcomere and pathogenic variants in MYH7 gene are responsible for Distal Myopathy 1 (MPD1, also known as Laing distal myopathy), whereas, A-type lamins are located at the inner nuclear membrane and pathogenic variants in LMNA gene are responsible for Emery-Dreifuss Muscular Dystrophy 2 (EDMD2) that has a proximal onset. The gene discussed is MYH7; the disease is Emery-Dreifuss muscular dystrophy 2, autosomal dominant.