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The human mitochondrial genome was completely sequenced in 1981 by Anderson and co-workers (
1
) and consists of a closed circular supercoiled
DNA
molecule of 16,569 base pairs. Mammalian cells characteristically contain a few hundred to several thousand mitochondria, each with 2-10 copies of the genome. The mitochondrial genome encodes 13 polypeptides, 22 transfer RNAs (tRNAs), and 2 rRNA. The 13 polypeptides encoded by the mitochondrial DNA (mtDNA) are essential subunits of the electron transport chain (ETC) and ATP synthase; cells lacking mtDNA are completely dependent on glycolysis for survival (
2
).