mtDNA loses more than 99% of its original genes and most autonomy in its symbiosis with nDNA. Its function depends on the basic functions of nuclear genes, including replication, translation, synthesis of most respiratory chain subunits, and assembly of respiratory chain complexes. And the synthesis of phospholipids that constitute the inner mitochondrial membrane (IMM). Mutations of respiratory chain complex subunit are induced by mitochondrial self DNA mutations and mutations in related nuclear genes. These mutations are mainly in complexes I and II, it indicates genetic mutations that are deleterious in complexes III, IV and V are rare or fatal. Most mutations in the nuclear gene of complex I or complex II subunit will cause LS, a hallmark neuropathic lesion in infancy or early childhood neurodegenerative disease. Studies have found that mtDNA mutations can also cause LS, a large number of mtDNA mutations will damage oxidative phosphorylation in the early life.