Mitochondria contain the respiratory chain enzyme complexes that carry out
oxidative phosphorylation and produce the main part of cellular energy in t
he form of ATP. Mitochondrial DNA (mtDNA) encodes essential subunits of the
respiratory chain and is thus critical for maintaining cellular energy pro
duction. The first pathogenic mtDNA mutations were reported in 1988, and to
day more than 50 disease-causing mtDNA mutations have been identified. In a
ddition, mtDNA mutations have been implicated in ageing and in common disor
ders such as diabetes mellitus, heart failure and Parkinson's disease, This
review will summarize recent advances in the rapidly expanding field of mi
tochondrial medicine.