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Table 1 Some forms of monogenic or maturity-onset diabetes of the young

From: Stem cell approaches for diabetes: towards beta cell replacement

Type Protein Description
MODY 1 HNF4A Loss-of-function mutations
MODY 2 Glucokinase Many forms, most often mild diabetes, can cause hypoglycemia
MODY 3 HNF1A Loss-of-function mutations
MODY 4 PDX1 Pancreas atrophy and beta cell impairment
MODY 5 HNF1B Pancreas atrophy and renal disease
MODY 6 NeuroD1 Transcription factor important for beta cell development
Permanent neonatal diabetes KCNJ11, ABCC8, neurogenin 3 Can be associated with hypoglycemia or diabetes. Some forms can be treated with sulfonylureas
Transient neonatal diabetes ABCC8 Some forms remit with time
  1. ABCC8, ATP-binding cassette, sub-family C, member 8; HNF1A, hepatocyte nuclear factor 1 homeobox A; HNF1B, hepatocyte nuclear factor 1 homeobox B; HNF4A, hepatocyte nuclear factor 4 alpha; KCNJ11, potassium channel J11; MODY, maturity-onset diabetes of the young; NeuroD1, neurogenic differentiation factor 1; PDX1, pancreatic duodenal homeobox.