Lactic acidosis and other mitochondrial disorders.

The ability of mitochondria to oxidize substrates and generate energy is integral to normal homeostasis and to the ability of cells to survive in the face of impending energy failure. Lactic acidosis is a common and readily apparent biochemical marker for mitochondrial dysfunction. However, lactic acidosis represents only the most obvious example in which acquired or congenital abnormalities of mitochondrial oxidative phosphorylating capacity contribute to the pathobiology and phenotypic expression of a broad spectrum of clinical disorders. Consequently, interventions that improve mitochondrial function or prevent mitochondrial energy failure may have widespread therapeutic implications.