ABSTRACT
Human mitochondrial DNA (mtDNA) deletion mutations have been found in a number of neurodegenerative diseases, including Kearns-Sayre syndrome, Leber’s hereditary optic neuropathy, Alzheimer’s disease (AD), and Parkinson’s disease (PD), and with aging. Abnormalities in mitochondrial metabolism likewise have been associated with neurodegenerative disease, e.g., Huntington’s disease (HD). In addition, a decline of aerobic energy metabolism in the affected tissue is often associated with the progression of these neurodegenerative diseases. A decline in aerobic metabolism has been observed in aging tissues.
