CAV3 and Qualitative or quantitative defects of caveolin-3: 13, 34 Whether skeletal muscle of LGMD1C patients also exhibit similar changes in mitochondrial biology is currently unknown, but testing this would be instructive not only for establishing the utility and relevance of myoblasts as a disease model but also for assessing whether impaired mitochondrial energetics/ATP production might contribute to muscle atrophy and the decline in muscle strength and exercise tolerance that has been reported in patients with caveolinopathies.69