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Table 1 Different types of monogenic diabetes and the genes implicated [52]

From: Challenges in diagnosis and management of diabetes in the young

Historical name Gene Locus Clinical features
MODY 1 HNF4A 20q12–q13.1 Mild-severe fasting and postprandial plasma glucose (PG) Respond well to sulphonylurea agents
MODY 2 GCK 7p15–p13 Mild fasting hyperglycemia. Less than 50 % of carriers have overt diabetes, and microvascular complications of diabetes are rare. Treatment not needed except in pregnancy (see below)
MODY 3 HNF1A 12q24.2 Same as MODY 1
MODY 4 IPF1/ PDX1 13q12.1 Pancreatic agenesis.
MODY 5 HNF1B 17cen–q21.3 Overt diabetes in association with renal and genito-urinary abnormalities.
MODY 6 NEUROD1 2q32 Rare, with phenotype characterized by obesity and insulin resistance.
MODY 7 KLF11 2p25 Very rare; phenotype ranges from impaired glucose tolerance or impaired fasting glucose to overt diabetes.
MODY 8 CEL 9q34.3 Very rare; associated with both exocrine and endocrine pancreatic deficiency and with demyelinating peripheral neuropathy.
MODY 9 PAX4 7q32 Very rare. Crucial transcription factor for beta cells development
MODY 10 INS 11p15.5 Very rare. Usually associated with neonatal diabetes. < 1 % cases.
MODY 11 BLK 8p23–p22 These adapter proteins’ nucleate formation contributes to the qualitative and quantitative control of beta cell signaling.
MODY 12 ABCC8 11p15.1 Very rare. Usually associated with neonatal diabetes.  < 1 % cases.
MODY 13 KCNJ11 11p15.1 Very rare. Usually associated with neonatal diabetes.  < 1 % cases.
MODY 14 WFS 4p16.1 Rare. Usually associated with DIDMOAD syndrome. Also, seen with early onset diabetes.< 1 % cases.