A rare form of diabetes associated with thiamine-responsive megaloblastic anemia (TRMA) is caused by a defect in SLC19A2 gene, encoding for THTR1 [28,29], while THTR2 truncated proteins as a result of mutations in SLC19A3 gene provoke biotin-responsive basal ganglial disease [30] and thiamine-responsive encephalopathy [31]. Here, SLC19A3 is linked to Thiamine-responsive encephalopathy.