MODY
MCID: MTR004
MIFTS: 49

Maturity-Onset Diabetes of the Young (MODY) malady

Summaries for Maturity-Onset Diabetes of the Young

Sources:
8Disease Ontology, 64Wikipedia, 47OMIM, 33MalaCards
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Wikipedia:64 Maturity onset diabetes of the young (MODY) refers to any of several hereditary forms of diabetes caused... more...

MalaCards: Maturity-Onset Diabetes of the Young, also known as MODY, is related to hypoglycemia and type 1 diabetes. An important gene associated with Maturity-Onset Diabetes of the Young is GCK (glucokinase (hexokinase 4)), and among its related pathways are Regulation of beta-cell development and Type II diabetes mellitus. The drugs acetohexamide and chlorpropamide and the compounds glucose and c-peptide have been mentioned in the context of this disorder. Related mouse phenotypes are growth/size and homeostasis/metabolism.

Disease Ontology:8 A genetic disease that has material basis in mutations in the mody genes disrupting insulin production.

Description from OMIM:47 610508, 125850, 613375, 612225, 606394 606392, 137920, 609812, 613370, 125851, 600496 more

Aliases & Classifications for Maturity-Onset Diabetes of the Young

Sources:
8Disease Ontology, 43NIH Rare Diseases, 20GeneTests, 10DISEASES, 61UMLS, 47OMIM
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Aliases & Descriptions:

maturity-onset diabetes of the young 8 43 20 10
mody 8 43
maturity onset diabetes mellitus in young 61
diabetes mellitus, non-insulin-dependent 61
mason type diabetes 43
mason-type diabetes 8


External Ids:

Disease Ontology8 DOID:0050524

Related Diseases for Maturity-Onset Diabetes of the Young

Sources:
17GeneCards, 18GeneDecks
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Diseases in the maturity-onset diabetes of the young, type 1 family:

maturity-onset diabetes of the young maturity-onset diabetes of the young, type 8
maturity-onset diabetes of the young, type 2 maturity-onset diabetes of the young, type 3
maturity-onset diabetes of the young, type 4 maturity-onset diabetes of the young, type 6
maturity-onset diabetes of the young, type 7 maturity-onset diabetes of the young, type 9
maturity-onset diabetes of the young, type 5 maturity-onset diabetes of the young, type 11
maturity-onset diabetes of the young, type 10

Diseases related to Maturity-Onset Diabetes of the Young via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 64)
idRelated DiseaseScoreTop Affiliating Genes
1hypoglycemia31.0GCKR, GCK, HNF4A, INS
2type 1 diabetes30.9INS, HNF4A, HNF1A, GCK
3type 2 diabetes mellitus30.8FOXA2, ISL1, PDX1, HNF1B, HNF4A, HNF1A
4hyperinsulinemic hypoglycemia30.8INS, GCK
5insulin resistance30.6GCK, HNF1A, HNF4A, HNF1B, FOXA2, INS
6adenoma30.6INS, HNF4A, HNF1A
7hyperinsulinism30.4GCK, INS
8gestational diabetes30.4GCK, HNF1A, HNF4A, INS
9familial hyperinsulinism30.4GCK
10permanent neonatal diabetes mellitus30.4INS, GCK
11neonatal diabetes mellitus30.1GCK, HNF1B, PDX1, INS
12pancreatic agenesis29.9PDX1
13maturity-onset diabetes of the young, type 810.7
14maturity-onset diabetes of the young, type 110.7
15maturity-onset diabetes of the young, type 610.7
16maturity-onset diabetes of the young, type 710.7
17maturity-onset diabetes of the young, type 910.7
18maturity-onset diabetes of the young, type 310.7
19maturity-onset diabetes of the young, type 410.7
20maturity-onset diabetes of the young, type 210.6
21renal cysts and diabetes syndrome10.5
22maturity-onset diabetes of the young type1010.5
23young syndrome10.4
24maturity-onset diabetes of the young, type 510.4
25monogenic diabetes10.4
26maturity-onset diabetes of the young type1110.4
27maturity-onset diabetes of the young, type 1110.4
28maturity-onset diabetes of the young, type 1010.4
29diabetes, permanent neonatal10.4
30mody, type ii10.4
31diabetes, type 210.3
32rh-mod syndrome10.3
33diabetic nephropathy10.3
34proliferative diabetic retinopathy10.3
35diabetic ketoacidosis10.3
36necrobiosis lipoidica10.3
37thyroid malformation10.3
38n syndrome10.3
39granuloma annulare10.3
40colorectal cancer10.3
41transient neonatal diabetes mellitus10.3
42lip disease10.3
43renal dysplasia10.3
44diabetes mellitus10.0GCKR, GCK, HNF1A, HNF4A, HNF1B, PDX1
45hyperglycemia10.0GCKR, GCK, HNF1A, HNF4A, HNF1B, PDX1
46insulinoma10.0GCK, HNF1A, HNF4A, PDX1, INS, PAX4
47obesity10.0GCKR, GCK, HNF1A, HNF4A, ISL1, FOXA2
48pancreatic cancer10.0KLF11, HNF4A, PDX1, INS, ACVR2A, CELA3B
49pancreatitis10.0INS, CELA3B, CEL
50glucose intolerance10.0GCK, HNF1A, HNF4A, HNF1B, INS

Graphical network of the top 20 diseases related to Maturity-Onset Diabetes of the Young:



Diseases related to maturity-onset diabetes of the young

Clinical Features for Maturity-Onset Diabetes of the Young

Sources:
47OMIM
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Clinical features from OMIM:

610508, 125850, 613375, 612225, 606394, 606392, 137920, 609812, 613370, 125851 600496 more

Drugs & Therapeutics for Maturity-Onset Diabetes of the Young

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
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Genetic Tests for Maturity-Onset Diabetes of the Young

Sources:
20GeneTests
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Genetic tests related to Maturity-Onset Diabetes of the Young:

id Genetic test Affiliating Genes
1 Maturity-onset Diabetes Of The Young Multi-gene Panels20

Anatomical Context for Maturity-Onset Diabetes of the Young

Animal Models for Maturity-Onset Diabetes of the Young or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
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Publications for Maturity-Onset Diabetes of the Young

Genetic Variations for Maturity-Onset Diabetes of the Young

Expression for genes affiliated with Maturity-Onset Diabetes of the Young

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Maturity-Onset Diabetes of the Young

Search GEO for disease gene expression data for Maturity-Onset Diabetes of the Young.

Pathways for genes affiliated with Maturity-Onset Diabetes of the Young

Sources:
54Reactome, 30KEGG, 50PharmGKB, 38NCBI BioSystems Database, 4Cell Signaling Technology, 52QIAGEN, 12EMD Millipore
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Pathways related to Maturity-Onset Diabetes of the Young according to GeneCards/GeneDecks:

(show all 14)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
10.6PAX4, GCK, INS, NEUROD1, FOXA2, PDX1
2
Hide members
10.6GCK, HNF1A, HNF4A, HNF1B, PDX1, ISL1
310.6GCK, HNF1A, HNF4A, PDX1, FOXA2, INS
410.6HNF1A, HNF4A, PDX1, FOXA2, NEUROD1, INS
5
Hide members
10.6KCNRG, KCTD4, GCK, HNF4A, FOXA2, NEUROD1
6
Hide members
10.6GCK, HNF4A, HNF1B, PDX1, NEUROD1
710.5INS, PDX1, HNF1B, HNF1A
810.5INS, PDX1, GCK
910.5INS, FOXA2, ISL1
1010.5NEUROD1, ISL1, HNF4A
11
Dichloroethylene metabolism
Hide members
10.5HNF1A, HNF4A
12
Hide members
10.5PAX4, ACVR2A, ACVR2B
1310.5NEUROD1, PAX4
14
Hide members
10.5ACVR2A, ACVR2B

Compounds for genes affiliated with Maturity-Onset Diabetes of the Young

Sources:
45Novoseek, 24HMDB, 50PharmGKB, 11DrugBank, 29IUPHAR
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Compounds related to Maturity-Onset Diabetes of the Young according to GeneCards/GeneDecks:

(show all 31)
idCompoundScoreTop Affiliating Genes
1glucose4511.1PAX4, KLF11, GCKR, GCK, HNF1A, HNF4A
2c-peptide4511.0GCK, HNF1A, HNF4A, PDX1, FOXA2, INS
3glycogen45 2411.9GCKR, GCK, HNF4A, FOXA2, PPP1R3B, INS
4sulfonylurea4510.9INS, PDX1, HNF1A, GCK
5katp4510.9GCK, PDX1, FOXA2, INS
6fatty acid4510.9GCK, HNF1A, HNF4A, FOXA2, INS, CEL
7retinoic acid45 2411.9GCK, HNF1A, HNF4A, HNF1B, PDX1, NEUROD1
8preproinsulin4510.9INS, PDX1, GCK
9i-app4510.9GCK, PDX1, INS
10incretin4510.9INS, PDX1, GCK
11proglucagon4510.9ISL1, PDX1, GCK
12repaglinide45 50 1112.9NEUROD1, INS, PAX4
13cholesterol45 29 11 2413.9GCK, HNF1A, HNF4A, HNF1B, INS, CEL
14oligonucleotide4510.8GCK, HNF1A, HNF4A, HNF1B, PDX1, CEL
15nnmt4510.8HNF1A, HNF1B
16mannitol-1-phosphate4510.8GCKR, GCK
17tolbutamide45 50 29 1113.8INS, HNF1A, GCK
18mannoheptulose4510.8GCKR, GCK
19glycerol45 11 2412.8GCK, HNF4A, INS, CEL
20chromium picolinate4510.8INS, GCK
21nicotinamide45 1111.8HNF1B, PDX1, INS
22streptozotocin4510.8GCK, PDX1, INS
23pyruvate4510.7PDX1, HNF4A, HNF1A, GCK
24fructose 1-phosphate45 2411.7GCKR, GCK
25threonine4510.7GCK, HNF4A, PDX1, ACVR2B, ACVR2A, CEL
26phosphoenolpyruvate45 1111.6HNF4A, HNF1A, GCK
27myristic acid45 11 2412.6HNF4A, INS, CEL
28uric acid45 2411.5INS, HNF1B, GCKR
29digitonin4510.5BLK, GCK
30fructose-6-phosphate45 1111.4GCK, GCKR
31serine4510.3GCK, HNF4A, PDX1, INS, ACVR2B, ACVR2A

GO Terms for genes affiliated with Maturity-Onset Diabetes of the Young

Sources:
16Gene Ontology
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Biological processes related to Maturity-Onset Diabetes of the Young according to GeneCards/GeneDecks:

(show all 31)
idNameGO IDScoreTop Affiliating Genes
1endocrine pancreas developmentGO:03101811.0PAX4, GCK, HNF1A, HNF4A, HNF1B, PDX1
2glucose homeostasisGO:04259311.0GCK, HNF1A, HNF4A, PDX1, FOXA2, NEUROD1
3negative regulation of transcription from RNA polymerase II promoterGO:00012211.0KLF11, HNF1A, HNF1B, PDX1, ISL1, FOXA2
4positive regulation of transcription from RNA polymerase II promoterGO:04594410.9HNF1A, HNF4A, HNF1B, PDX1, ISL1, FOXA2
5regulation of insulin secretionGO:05079610.9INS, NEUROD1, HNF4A, HNF1A, GCK
6insulin secretionGO:03007310.9HNF1A, HNF1B, PDX1, NEUROD1, ACVR2B
7response to glucose stimulusGO:00974910.9HNF1A, HNF4A, HNF1B, NEUROD1, ACVR2B
8positive regulation of insulin secretionGO:03202410.9GCK, PDX1, ISL1, BLK
9signal transduction involved in regulation of gene expressionGO:02301910.9NEUROD1, FOXA2, HNF4A
10anterior/posterior pattern specificationGO:00995210.9HNF1B, NEUROD1, ACVR2B, ACVR2A
11positive regulation of cell differentiationGO:04559710.9PAX4, INS, NEUROD1
12regulation of pronephros sizeGO:03556510.8HNF1A, HNF1B
13negative regulation of glucokinase activityGO:03313210.8GCKR, FOXA2
14detection of glucoseGO:05159410.8PDX1, GCK
15positive regulation of transcription, DNA-dependentGO:04589310.8HNF1A, HNF4A, HNF1B, FOXA2, NEUROD1
16glucose transportGO:01575810.8GCKR, GCK, INS
17positive regulation of activin receptor signaling pathwayGO:03292710.8ACVR2A, ACVR2B
18positive regulation of transcription initiation from RNA polymerase II promoterGO:06026110.8HNF1B, HNF1A
19negative regulation of gluconeogenesisGO:04572110.8INS, GCK
20transmembrane receptor protein serine/threonine kinase signaling pathwayGO:00717810.8ACVR2A, ACVR2B
21positive regulation of glycolysisGO:04582110.8GCK, INS
22cellular glucose homeostasisGO:00167810.7GCK, GCKR
23regulation of Wnt receptor signaling pathwayGO:03011110.7HNF1B, HNF1A
24neuron fate specificationGO:04866510.7FOXA2, ISL1
25positive regulation of glycogen biosynthetic processGO:04572510.7GCK, INS
26nitric oxide mediated signal transductionGO:00726310.6NEUROD1, PDX1
27SMAD protein signal transductionGO:06039510.6HNF4A, HNF1A
28triglyceride homeostasisGO:07032810.5GCKR, HNF4A
29gastrulation with mouth forming secondGO:00170210.5ACVR2A, ACVR2B
30pancreas developmentGO:03101610.4ISL1, ACVR2B
31positive regulation of DNA bindingGO:04338810.2ISL1, PDX1

Molecular functions related to Maturity-Onset Diabetes of the Young according to GeneCards/GeneDecks:

(show all 13)
idNameGO IDScoreTop Affiliating Genes
1sequence-specific DNA binding transcription factor activityGO:00370010.9PAX4, KLF11, HNF1A, HNF4A, HNF1B, PDX1
2transcription regulatory region DNA bindingGO:04421210.8KLF11, HNF1A, HNF4A, HNF1B, FOXA2
3double-stranded DNA bindingGO:00369010.8PAX4, NEUROD1, FOXA2, HNF1A
4RNA polymerase II activating transcription factor bindingGO:00110210.7HNF4A, ISL1, NEUROD1
5sequence-specific DNA bindingGO:04356510.7HNF1A, HNF4A, HNF1B, NEUROD1, PAX4
6inhibin bindingGO:03471110.7ACVR2A, ACVR2B
7transcription factor bindingGO:00813410.7NEUROD1, FOXA2, PDX1, HNF1A
8receptor signaling protein serine/threonine kinase activityGO:00470210.6ACVR2A, ACVR2B
9RNA polymerase II distal enhancer sequence-specific DNA binding transcription factor activityGO:00370510.6HNF4A, HNF1B, FOXA2
10RNA polymerase II transcription coactivator activityGO:00110510.5NEUROD1, ISL1
11activin bindingGO:04818510.5ACVR2A, ACVR2B
12transforming growth factor beta-activated receptor activityGO:00502410.4ACVR2A, ACVR2B
13protein heterodimerization activityGO:04698210.2HNF1A, HNF1B, PDX1, NEUROD1

Products for genes affiliated with Maturity-Onset Diabetes of the Young

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Sources for Maturity-Onset Diabetes of the Young

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet