DNA mismatch repair (MMR) is essential for maintaining the integrity of the genome during replication. This process is highly conserved across bacterial and eukaryotic systems, as many of the genes expressed in bacteria are closely related to the yeast and mammalian homologs. In bacteria two proteins, MutS and MutL, form homodimeric complexes that are responsible for recognizing and facilitating MMR. Human homologs of these proteins include MSH2 and MSH3 (MutS homolog 2 and 3), and the corresponding human homologs of MutL are MLH1, PMS1, PMS2 and MLH3. MSH2 and MSH3 form heterodimers that cooperatively mediate MMR. MLH3 preferentially dimerizes with MLH1 to repair DNA mismatches and restore the stability to the genome.