Table 1 MODY—genes and relative prevalence

GCK

Glucokinase

Common (30-70% of MODY)

[4]

HNF1A

Hepatocyte nuclear factor-1 hohomeobox A

Common (30-70% of MODY)

[5]

HNF4A

Hepatocyte nuclear factor-4 homeobox A

5-10% of MODY

[6]

HNF1B

Hepatocyte nuclear factor-1 homeobox B

5-10% of MODY

[8]

ABCC8

ATP-binding cassette C8

Rare: < 1% of MODY

[90]

KCNJ11

Inward-rectifying potassium channel J11

Rare: < 1% of MODY

[98]

INS

Insulin

Rare: < 1% of MODY

[99]

PDX1

Pancreas/duodenum homeobox-1

Very rare

[100]

NEUROD1

Neurogenic differentiation-1

Very rare

[101]

CEL

Cholesteryl-ester lipase

Very rare

[102]

KLF11

Krüpell-like factor 11

Very rare

[103]

PAX 4

Paired homeobox 4

Very rare

[104]

BLK

B-lymphoid tyrosine kinase

Very rare

[105]

APPL1

Adaptor protein, phosphotyrosine interaction, PH Domain, and leucine zipper-containing 1

Very rare

[106]