A missense mutation in OGG1, reported in kidney cancer, leads to substitution of glycine at position 12 with glutamic acid residue, disrupting the mitochondrial targeting sequence at the N-terminus of OGG1. OGG1beta G12E mutant is unable to translocate to the mitochondrion (Audebert et al. 2002). It is uncertain whether OGG1beta participates in the repair of 8-oxoguanine lesions in mitochondrial DNA. While some studies have reported DNA glycosylase activity of OGG1 beta (reviewed by Furihata 2015) and preservation of this activity in OGG1beta G12E mutant (Audebert et al. 2002), other studies have reported lack of DNA glycosylase activity in OGG1beta (Hashiguchi et al. 2004).