PLEC and myopathy: The importance of this interplay is supported by the observation that aside from DES mutations [30,52], mutations in genes encoding other essential extrasarcomeric intermediate filament cytoskeleton components such as the cytoskeletal linker protein plectin (PLEC) [53,54] or the small heat shock protein alphaB-crystallin (CRYAB) [55,56] also cause cardiomyopathies and myopathies with morphological and biochemical evidence of mitochondrial dysfunction [30,57,58].