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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.