The epidemiology, molecular pathogenesis, diagnosis, and treatment of maturity-onset diabetes of the young (MODY)

Table 3 Underlying molecular pathogenesis and treatment of MODY

MODY subtype	Pathophysiology	Treatment options	References
Transcriptional regulation disorders
HNF4A (MODY1)	Beta cell dysfunction	Diet, sulfonylureas, insulin	[21, 69, 70]
HNF1A (MODY3)	Beta cell dysfunction	Diet, sulfonylureas, insulin, GLP-1 RAs	[69]
PDX1/IPF1 (MODY4)	Beta cell dysfunction	Diet, OADs, insulin	[21, 69]
HNF1B (MODY5)	Beta cell dysfunction	Insulin	[21, 69, 72]
NEUROD1 (MODY6)	Beta cell dysfunction	Diet, OADs, insulin	[21, 69]
KLF11 (MODY7)	Beta cell dysfunction	OADs, insulin	[21, 69]
PAX4 (MODY9)	Beta cell dysfunction	Diet, OADs, insulin	[21, 69]
BLK (MODY 11)	Insulin secretion defect	Diet, OADs, insulin	[21, 69]
Enzyme disorders
GCK (MODY2)	Glucose sensing defect	Usually not treated. Insulin may be used during pregnancy	[69, 73, 74]
Protein misfolding disorders
CEL (MODY8)	Pancreatic exocrine and endocrine dysfunction	OADs, insulin	[21, 69]
INS (MODY10)	Insulin biosynthesis defect	Diet, OADs, insulin	[21, 69]
Ion channel disorders
ABCC8 (MODY12)	Insulin secretion defect	Sulfonylureas	[21, 69]
KCNJ11 (MODY13)	Insulin secretion defect	Sulfonylureas	[21, 69]
Signal transduction disorders
APPL1 (MODY14)	Insulin secretion defect	Diet, OADs, insulin	[21, 69]

OADs oral antidiabetic drugs, GLP-1 RAs glucagon-like peptide-1 receptor agonists

ISSN: 2055-8260