Table 2.
Maturity-onset diabetes of the young subtypes, tissue distribution, and extra-pancreatic features
| MODY type | Tissue distribution | Extra-pancreatic features |
|---|---|---|
| MODY1 | Liver, kidney, intestine, pancreatic islets and insulinoma cells | Macrosomia with hyperinsulinemic hypoglycemia; low levels apolipoproteins and triglycerides; atypical Fanconi syndrome with hypercalciuria and nephrocalcinosis in carriers of the p.Arg76Trp (R76W) mutation |
| MODY2 | Liver and pancreatic beta-cells | Not described |
| MODY3 | Liver, pancreatic islets, kidney | Renal glycosuria |
| MODY4 | Pancreatic beta-cells | Overweight/obesity in some patients |
| MODY5 | Kidney, liver, bile ducts, thymus, genital tract, pancreas, lung, and gut | Renal cysts; progressive renal dysfunction, leading to chronic renal insufficiency and failure; internal genital abnormalities (in female carriers); pancreatic hypoplasia, pancreatic cysts |
| MODY6 | Neurons in the central and peripheral nervous systems, pancreatic endocrine cells, intestine | Overweight/obesity in some patients; intellectual disabilities with brain abnormalities (uncommon) |
| MODY7 | Ubiquitously expressed | Not described |
| MODY8 | Lactating mammary gland and pancreas | Pancreatic atrophy with exocrine pancreatic insufficiency; fibrosis and lipomatosis causing hyperglycemia |
| MODY9 | Germ layers in the mammalian embryo | Not described |
| MODY10 | Pancreas, eye, limb | Not described |
| MODY11 | Lymphoblastoid cell lines, spleen, pancreatic islets, liver, leukocytes, ovary, muscle, testis | Overweight/obesity in some patients |
| MODY12 | Pancreatic beta-cells | Not described |
| MODY13 | Pancreatic beta-cells, neurons, muscle cells | Not described |
| MODY14 | Highly expressed in skeletal muscle, heart, ovary, and pancreas | Overweight/obesity in some patients |