For example, it was shown that when the mismatch repair (MMR) proteins MSH2, MSH3 and MSH6 are downregulated upon differentiation, repeat instability is considerably reduced in hESC/iPSCs with myotonic dystrophy (DM1; CTG repeats), Huntington’s disease (HD; CAG repeats) and Friedrich’s ataxia (FRDA; GAA repeats) mutations [95,96]. This evidence concerns the gene MSH6 and Huntington disease.