Mitochondrial disease – a review

Mitochondria are the source of cellular energy. Genetically they depend on mitochondrial genes (mtDNA) as well as nuclear genes. MtDNA inheritance differs from Mendalian inheritance in many respects. As mitochondria are found in all cells, mitochondrial disease has an exceptionally wide clinical spectrum. This review summarizes the features of the classical mitochondrial disorders including the classical syndromes. The investigation and management is also discussed.