Early studies have shown elevated NFκB levels in skeletal muscle of mdx mice, the mouse model for Duchenne muscular dystrophy (DMD), and in inflammatory myopathies.17, 18, 19, 20, 21 NFκB activation is thought to contribute to the deterioration of skeletal muscle pathology and muscle loss in DMD.18 However, NFκB seems to have a multifaceted regulatory role and may show protective activity in different disorders. Here, NFKB1 is linked to myopathy.