FSHD patients manifest many of the clinicopathological features common to LGMD2B and DMD, including lobulated (trabeculated) fibers with abnormal distributions of intramyofibrillar mitochondria, oxidative stress, and reductions in anti-oxidant defense mechanisms, abnormal calcium homeostasis, and mitochondrial dysfunction [19,20,54,55,56,57]. Here, DYSF is linked to facioscapulohumeral muscular dystrophy.