| 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 |
