Several missense and in-frame indel mutations in FLNC [8,22,28], disrupting the normal organization of myofibrils and often leading to abnormal cytoplasmic filamin C aggregates, were described in a particular form of myofibrillar myopathy, consisting in late onset skeletal myopathy, with or without poorly characterized CMP. The gene discussed is FLNC; the disease is skeletal muscle disorder.