2.8. MOUSE MODELS OF DEGENERATIVE DISEASES AND AGING
To learn more about the pathophysiology of mitochondrial degenerative diseases and aging, mice in which genes for mitochondrial energy production and ROS detoxification had been inactivated were analysed. Inhibition of mitochondrial energy production was accomplished by genetically inactivating the heart-muscle isoform of the adenine nucleotide translocator (ANT1). In tissues where this gene was expressed, this effectively blocked export of mitochondrial ATP to the cytosol and the reciprocal import of cytosolic ADP into the mitochondrial matrix. Inactivating the mitochondrial MnSOD increased Mitochondrial ROS production.