MODY Sub-Type Gene Affected Protein Affected Locus Gene Function Primary Defect
Type 1 HNF4 alpha Hepatocyte nuclear factor 4 alpha 20q Transcription factor (Nuclear factor) Pancreas
Type 2 GCK Glucokinase 7p15-p13 Hexokinase IV Pancreas/Liver
Type 3 TCF1 Hepatocyte nuclear factor 1 alpha 12q24.2 Transcription factor (Homeodomain) Pancreas/Kidney
Type 4 IPF1 insulin promoter factor-1 13q12.1 Transcription factor (Homeodomain) Pancreas
Type 5 TCF2 Hepatocyte nuclear factor 1 beta 17q12 Transcription factor (Homeodomain) Kidney/Pancreas
Type 6 Neuro D1 Neurogenic differentiation factor 1 2q Transcription factor (bHLH) Pancreas
Type 7 KLF11 Kruppel-like factor 11 2p25 Transforming growth factor-beta inducible- early growth response 2 Pancreas
Type 8 CEL Bile salt dependent lipase 9q34.3 Hydrolyze cholesterol esters as well as a variety of other dietary esters (OMIM 114840) Pancreas
Type 9 PAX4 Paired domain gene 4 7q32 Transcription factor (paired domain gene 4) Pancreas
Type 10 INS Insulin 11p15.5 Beta cells of the islets of Langerhans NF-kappa-B
Type 11 BLK Tyrosine kinase B-Lymphocyte specific 8p23-p22 Tyrosine kinase (B lymphocytes) MIN6 beta cells
Table 1: Sub-types of MODY.