DES and Desminopathy: In the context of desminopathies, a direct functional link between desmin pathology and mitochondrial dysfunction can be formulated based on the observations that mitochondria co-localize with the desmin intermediate filament network [63] and that desmin knock-out mice display defects in the morphology and positioning as well as in the respiratory enzyme function of mitochondria in striated muscle tissue [25, 44, 54].