Mitochondrial diseases in man and mouse

Over the past 10 years, mitochondrial defects have been implicated in a wid e variety of degenerative diseases, aging, and cancer. Studies on patients with these diseases have revealed much about the complexities of mitochondr ial genetics, which involves an interplay between mutations in the mitochon drial and nuclear genomes. However, the pathophysiology of mitochondrial di seases has remained perplexing. The essential role of mitochondrial oxidati ve phosphorylation in cellular energy production, the generation of reactiv e oxygen species, and the initiation of apoptosis has suggested a number of novel mechanisms for mitochondrial pathology. The importance and interrela tionship of these functions are now being studied in mouse models of mitoch ondrial disease.