Table 1.  Maturity‐onset diabetes of the young.

	Gene	Protein	Clinical features
MODY1	HNF4A	Hepatocyte nuclear factor 4‐alpha	Sensitive to sulfonylureas
MODY2	GCK	Glucokinase	Stable, non‐progressive elevated fasting blood glucose; typically does not require treatment
MODY3	HNF1A	Hepatocyte nuclear factor 1‐alpha	Sensitive to sulfonylureas
MODY4	PDX1 (IPF1)	Pancreatic duodenal homeobox 1 (insulin promoter factor 1)	Rare, diabetes appears to be mild
MODY5	HNF1B	Hepatocyte nuclear factor 1‐beta	Renal and genitourinary abnormalities; atrophy of the pancreas
MODY6	NEUROD1	Neurogenic differentiation 1
MODY7	CEL	Carboxyl‐ester lipase	Atrophy of the pancreas
MODY8	INS	Insulin

MODY, maturity‐onset diabetes of the young.