Besides the production of ATP, there is a complex relationship between the mitochondrial genome (mtDNA) and the nuclear genome (nDNA). Unlike nuclear DNA which resides in the nucleus, mtDNA is inherited from the mother. Upon fertilization, the mtDNA of the sperm is typically lost through ubiquitination, and only the egg contributes to the mtDNA of the zygote, due to the significant difference in mtDNA copy number between the sperm (about 100 copies) and the egg (about 100,000 copies).

Mitochondrial