Modeling Mitochondrial Myopathy in Mice

Mitochondria constitute the major energy plant of the cell; genetic defects of mitochondrial bioenergetics cause an astonishingly wide spectrum of disorders. Mitochondrial disorders are genetic conditions characterized by faulty oxidative phosphorylation and usually manifests in organs that are highly dependent on energy, such as skeletal muscle. Mitochondrial myopathies (MMs) are a subset of mitochondrial diseases, primary prognosis of which are prominent muscular dysfunction.