Mutations in mt-tRNA genes are associated with maternally inherited diabetes and deafness (MIDD) and insulin resistance [86,87,88,89,90] and mutations in the tRNA methyltransferase TRMT10A cause young onset diabetes and microcephaly [53,91,92,93,94,95,96]. The gene discussed is TRMT10A; the disease is maternally-inherited diabetes and deafness.