Mitochondria, free radicals, neurodegeneration and aging

The discovery that defects of the mitochondrial respiratory chain can result in human diseases is relatively recent (Schapira and DiMauro, 1994; Wallace, 1992). Over the same period evidence has also been presented which suggests that mutations of mitochondrial DNA (mtDNA) and a decline in respiratory chain function contribute to the aging process. This chapter will review the lessons that can be learned from our study of inborn metabolic defects of the respiratory chain in understanding the functional importance of cumulative mtDNA mutations. The potential role of mitochondrial toxins in human disease will also be discussed. These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.