In addition, rare MODY cases [accounting for up to 6% of all MODY forms (49)] have been diagnosed with mutations in PDX1 (59), HNF1B (60), NEUROD1 (61, 62), KLF11 (63), CEL (64), PAX4 (65), INS (66), BLK (67), ABCC8 (68), KCNJ11 (69), and APPL1 (70) (known as MODY4-14). This evidence concerns the gene HNF1B and MODY.