The genes MutS homolog 2 (MSH2), MutL Homolog 1 (MLH1) and PMS1 Homolog 2 (PMS2) are involved in this type of repair mechanism [39], with MSH2 and MLH1 mutations having the greatest contribution to the development of LS-associated malignancies [40]. This evidence concerns the gene MLH1 and Leigh syndrome.