Endonuclease G, Mitochondrial (ENDOG)

Endonuclease G, mitochondrial is a nuclear encoded endonuclease that is localized in the mitochondrion. The encoded protein is widely distributed among animals and cleaves DNA at GC tracts. This protein is capable of generating the RNA primers required by DNA polymerase gamma to initiate replication of mitochondrial DNA. Tiranti et al. (1995) mapped 3 human housekeeping genes involved in mitochondrial biogenesis. One of these was the gene encoding a mitochondrion-specific endonuclease, designated ENDOG, that preferentially cleaves DNA stretches rich in C and G residues. The authors hypothesized that this endonuclease could play a role in the maturation of RNA complementary stretches serving as primers for mtDNA replication, in the splicing process of polycistronic transcripts, or in mtDNA repair.