Wredenberg et al. [14] created a mouse model of mitochondrial myopathy (MITO) by disrupting the gene for mitochondrial transcription factor A (Tfam), which leads to the development of many of the hallmarks of mitochondrial myopathies, including ragged red muscle fibers, the accumulation of abnormal mitochondria, and deteriorating respiratory chain function. Here, TFAM is linked to Mitochondrial myopathy.