Classification of Maturity-Onset Diabetes of the Young, U.S., 2022
| GENE (CHROMOSOME) | NAME AND MODY DESIGNATION | PREFERRED TREATMENT | RELATIVE INCIDENCE/ FREQUENCY (PERCENT) |
|---|---|---|---|
| Most common forms | |||
|
GCK
(7p15-p13) | GCK-MODY (MODY 2) | None | >70 |
|
HNF1A
(12q24.2) | HNF1A-MODY (MODY 3) | Low-dose sulfonylurea / insulin | 20–30 |
|
HNF4A
(20q12-q13.1) | HNF4A-MODY (MODY 1) | Sulfonylurea / insulin | ~5 |
|
HNF1B
(17q12) | HNF1B-MODY (MODY 5) | Insulin | <2 |
| Rarer forms | |||
|
INS
(11p15.5) | MODY 10 | Insulin | <1 |
|
ABCC8
(11p15.1) | MODY 12 | High-dose Sulfonylurea | <1 |
|
KCNJ11
(11p15.1) | MODY 13 | High-dose Sulfonylurea | <1 |
|
PDX1
(13q12.2) | MODY 4 | Insulin | <1 |
|
NEUROD1
(2q32) | MODY 6 | Insulin | <1 |
|
CEL
(9q34.3) | MODY 8 | Insulin | <1 |
| Refuted/disputed forms | |||
|
KLF11
(2p25.1) | MODY 7 | -- | |
|
PAX4
(7q32.1) | MODY 9 | -- | |
|
BLK
(8p23.1) | MODY 11 | -- | |
GCK, HNF1A, and HNF4A together constitute approximately 95% of all known MODY patients. Gene names and preferred treatment for each MODY form are listed according to prevalence. ABCC8, ATP-binding cassette transporter subfamily C member 8; BLK, B lymphocyte kinase; CEL, carboxyl ester lipase; GCK, glucokinase; HNF, hepatocyte nuclear factor; INS, insulin; KCNJ11, potassium inwardly rectifying channel subfamily J member 11; KLF11, Kruppel-like factor 11; MODY, maturity-onset diabetes of the young; NEUROD1, neurogenic differentiation 1; PAX4, paired box 4; PDX1, pancreatic and duodenal homeobox 1.
SOURCE: Reference 14
From: Monogenic Forms of Diabetes
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