Maturity-onset Diabetes of the Young malady

Wikipedia: Maturity onset diabetes of the young (MODY) refers to any of several hereditary forms of diabetes caused...⁴⁴ more...

MalaCards: Maturity-onset Diabetes of the Young, also known as MODY, is related to maturity-onset diabetes of the young, type 2 and maturity-onset diabetes of the young, type 3. An important gene associated with Maturity-onset Diabetes of the Young is GCK (glucokinase (hexokinase 4)), and among its related pathways are Regulation of gene expression in endocrine-committed (NEUROG3+) progenitor cells and Gene Expression. The drugs glipizide and repaglinide and the compounds i-app and katp have been mentioned in the context of this disorder. Affiliated tissues include kidney, liver and thyroid, and related mouse phenotypes are integument and endocrine/exocrine gland.

Disease Ontology: A genetic disease that has material basis in mutations in the mody genes disrupting insulin production.⁶

Aliases & Descriptions:

maturity-onset diabetes of the young 6 30 8 mody 6 7 30 maturity onset diabetes mellitus in young 43

diabetes mellitus, non-insulin-dependent 43 mason type diabetes 30 mason-type diabetes 6

Disease types for maturity-onset diabetes of the young family:

maturity-onset diabetes of the young, type 1	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 5	maturity-onset diabetes of the young, type 6
maturity-onset diabetes of the young, type 7	maturity-onset diabetes of the young, type 8
maturity-onset diabetes of the young, type 9	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 by text searches and GeneDecks gene sharing:

(show top 50)    (show all 170)

id	Related Disease	Score	Top Affiliating Genes
1	maturity-onset diabetes of the young, type 2	37.7	GCK, ACVR2B, ACVR2A
2	maturity-onset diabetes of the young, type 3	37.2	CEL, CELA3B, INS, HNF1A, HNF4A
3	hypoglycemia	33.1	PCK2, PCK1, GCKR, GCK, GCG, KCNJ11
4	type 2 diabetes mellitus	32.2	KCNJ11, TCF7L2
5	hyperinsulinemic hypoglycemia	32.1	GCK, GCG, KCNJ11, FOXA2, INS, HNF4A
6	diabetic ketoacidosis	31.8	INS, HNF4A, HLA-DQB1, GAD1
7	hepatitis a	30.9	HNF1B, DPP4, ADA, GAPDH, GGT1
8	type 1 diabetes mellitus	30.9	GCK, GCG, VDR, PAX4, CELA3B, INS
9	diabetes, type 2	30.6	GCG, KCNJ11, INS, NR1H2, TCF7L2
10	gestational diabetes	30.3	GCK, GCG, KCNJ11, IRS1, INS, IAPP
11	neonatal diabetes mellitus	28.4	GCK, KCNJ11, INS, HNF1B, ONECUT1, PDX1
12	polyposis	28.0	PCBD1, VDR, HNF1A, HNF4A, HLA-DQB1, PPARA
13	hyperglycemia	26.8	PCK2, PCK1, GCKR, GCK, GCG, PAX4
14	diabetes mellitus	25.4	PCBD1, PCK2, PCK1, GCKR, GCK, GCG
15	colorectal cancer	24.5	PCBD1, RBP1, VDR, BMP3, PAX6, FOXM1
16	cystic fibrosis	23.5	VDR, KCNJ11, CEL, CELA3B, INS, IGFBP2
17	pancreatitis	22.7	KLF11, PCBD1, GCKR, GCK, GCG, RBP1
18	neuroblastoma	22.3	VDR, FOXA1, FOXM1, IRS1, INS, IGFBP2
19	thyroiditis	22.2	PCK2, PCK1, GCK, GCG, VDR, FOXA1
20	fibrosis	21.8	VDR, BMP3, KCNJ11, CEL, CELA3B, INS
21	hepatitis	21.6	PCBD1, PCK2, PCK1, GCKR, GCK, GCG
22	nephropathy	21.1	GCK, GCG, VDR, KCNJ11, CELA3B, IRS1
23	alzheimer's disease	20.3	PCBD1, PCK1, GCG, VDR, IRS1, INS
24	adenoma	19.9	GCK, GCG, VDR, BMP3, IRS1, INS
25	obesity	19.4	PCBD1, PCK2, PCK1, GCKR, GCK, GCG
26	hepatocellular carcinoma	19.2	PCK2, RBP1, VDR, FOXA2, FOXM1, IRS1
27	leukemia	19.0	RBP1, VDR, BLK, PAX4, PAX6, MNX1
28	carcinoma	15.1	PCBD1, PCK2, GCK, GCG, RBP1, VDR
29	glomerulocystic kidney disease	13.9	GCK, HNF1A, HNF1B
30	pancreatic agenesis	13.8	GCK, KCNJ11, INS, PDX1
31	permanent neonatal diabetes mellitus	13.8	GCK, KCNJ11, INS, PDX1
32	persistent hyperinsulinemic hypoglycemia of infancy	13.8	GCK, KCNJ11, INS, PDX1
33	hypoglycemia of infancy	13.8	GCK, KCNJ11, INS, PDX1
34	diabetes mellitus, noninsulin-dependent	13.5	IRS1, HNF1B, HNF4A, SLC2A2, NEUROD1
35	pancreatic endocrine tumors	13.5	GCG, ISL1, PDX1, HES1, NEUROD1, NEUROG3
36	prediabetes syndrome	13.5	INS, DPP4, GAD1
37	acute porphyria	13.4	PCK2, NR1I2, NR1I3
38	gastrinoma	13.3	GCG, INS, IAPP, GLP1R, NR1I3, PDX1
39	hyperuricemia	13.3	GCK, INS, FABP2, HNF1B, ADA, GGT1
40	pepck 1 deficiency	13.3	PCK2, PCK1
41	neuroendocrine tumor	13.2	GCG, INS, IAPP, GLP1R, PDX1, NKX2-2
42	familial hyperlipidemia	13.2	INS, APOC2, APOC3, PPARA
43	xanthomatosis	13.1	INS, NR1H2, NR1H4, NR1I2
44	hyperlipoproteinemia type iii	13.1	INS, APOC2, APOC3, PPARA
45	androgen insensitivity syndrome	13.1	INS, IGFBP2, NR1H2, NR1I2
46	carbohydrate metabolic disorder	13.1	IRS1, INS, PPARA
47	neuroectodermal endocrine syndrome	13.1	INS, IAPP, NR1H2, GAD1
48	biliary tract disease	13.0	INS, NR1H2, NR1H4, NR1I2, NR1I3
49	common wart	13.0	HLA-DQB1, NFIC, NFIX
50	jaundice	13.0	CEL, HNF1B, HLA-DQB1, NR1I3, PKLR, GGT1

Approved drugs:

Drug clinical trials:

Inferred drug relations via UMLS/NDF-RT:

^43 28 acarbose, acetohexamide, chlorpropamide, exenatide, glimepiride, glipizide, glyburide, insulin, insulin,aspart protamine, insulin,aspart,human/rdna, insulin,detemir,human, insulin,glargine,human/rdna, insulin,glulisine,human, insulin,lente,beef, insulin,lente,beef/pork, insulin,lente,beef/purified, insulin,lente,human/rdna, insulin,lente,human/semisynthetic, insulin,lente,pork/purified, insulin,lispro,human/rdna, insulin,lispro,protamine/rdna, insulin,nph,beef, insulin,nph,beef/pork, insulin,nph,beef/purified, insulin,nph,human/rdna, insulin,nph,human/semisynthetic, insulin,nph,pork/purified, insulin,protamine zinc,beef/pork, insulin,protamine zinc,beef/purified, insulin,protamine zinc,pork/purified, insulin,regular,beef/pork, insulin,regular,beef/purified, insulin,regular,human buffered, insulin,regular,human/rdna, insulin,regular,human/semisynthetic, insulin,regular,pork, insulin,regular,pork/purified, insulin,semilente,beef, insulin,semilente,beef/pork, insulin,semilente,pork/purified, insulin,ultralente,beef, insulin,ultralente,beef/pork, insulin,ultralente,beef/purified, insulin,ultralente,human, insulin,ultralente,human/rdna, metformin, metformin hydrochloride, miglitol, nateglinide, pioglitazone, pioglitazone hcl, pioglitazone hydrochloride, pramlintide, repaglinide, rosiglitazone, rosiglitazone maleate, sitagliptin, sitagliptin phosphate, tolazamide, tolbutamide, tolbutamide sodium, troglitazone

MalaCards organs/tissues related to maturity-onset diabetes of the young:

²²

MGI Mouse Phenotypes related to maturity-onset diabetes of the young:

²⁵ (show all 23)

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	integument phenotype	MP:0010771	9.7	PDX1, NR4A3, NR4A1, ISL1, FOXA2, FOXA1
2	endocrine/exocrine gland phenotype	MP:0005379	9.6	GGT1, NEUROG3, NEUROD1, NKX2-2, DPP4, ACVR2A
3	renal/urinary system phenotype	MP:0005367	9.2	TMEM27, NFIA, ACVR2B, NEUROD1, NEUROG3, GAPDH
4	respiratory system phenotype	MP:0005388	9.2	HHEX, ADA, NEUROD1, ACVR2A, ACVR2B, NFIB
5	no phenotypic analysis	MP:0003012	9.1	NR1H4, NR1I2, NR2F2, HES1, NKX6-1, HNF1A
6	hematopoietic system phenotype	MP:0005397	9.0	GAPDH, HHEX, ADA, HES1, ACVR2B, NR4A3
7	vision/eye phenotype	MP:0005391	8.8	NFIX, ACVR2A, HES1, PRKAB1, NEUROD1, HHEX
8	adipose tissue phenotype	MP:0005375	8.5	TMEM27, CYB5R4, PPARA, TCF7L2, PRKAB1, NEUROG3
9	craniofacial phenotype	MP:0005382	8.1	NFIX, ACVR2A, HES1, TCF7L2, HHEX, GAD1
10	digestive/alimentary phenotype	MP:0005381	8.0	NR2F2, NR5A2, NFIX, PDX1, ACVR2A, HES1
11	immune system phenotype	MP:0005387	7.8	PKLR, NR5A2, NR4A3, NR2F2, PDX1, ACVR2B
12	skeleton phenotype	MP:0005390	7.7	HMX1, NR1H2, NFIC, ACVR2B, ADA, GAD1
13	normal phenotype	MP:0002873	7.5	PKLR, NR1H3, NR1H2, HLA-DQB1, NFIX, PDX1
14	liver/biliary system phenotype	MP:0005370	7.4	HHEX, ONECUT1, HNF1B, HNF1A, INS, IRS1
15	muscle phenotype	MP:0005369	7.1	PPARA, NR1H2, NR1H3, NR2F2, PDX1, TCF7L2
16	reproductive system phenotype	MP:0005389	6.4	NR1I3, NR2F2, NR5A2, NFIA, NFIC, ACVR2A
17	cardiovascular system phenotype	MP:0005385	6.4	NR1H2, PPARA, ONECUT1, HLA-DQB1, NR1H3, NR2F2
18	nervous system phenotype	MP:0003631	6.3	NFIA, NFIB, NFIC, NFIX, ACVR2A, HES1
19	behavior/neurological phenotype	MP:0005386	5.7	NFASC, NR4A3, NR4A2, NR4A1, NR1I2, NR1H3
20	cellular phenotype	MP:0005384	5.1	ACVR2B, PDX1, NFIA, NR4A3, NR4A2, NR1I3
21	growth/size phenotype	MP:0005378	3.7	GAD1, NFIC, NFIB, NFIA, NFASC, NR5A2
22	homeostasis/metabolism phenotype	MP:0005376	2.4	GGT1, GAPDH, GAD1, HHEX, ADA, NEUROG3
23	mortality/aging	MP:0010768	2.2	HES1, NR2F2, NR1I3, NR1I2, NR1H4, NR1H3

Articles related to maturity-onset diabetes of the young:

(show top 50)    (show all 135)

id	Title	Authors	Year	Affiliating Genes
1	Bone morphogenetic protein 3 controls insulin gene ex pression and is down-regulated in INS-1 cells inducibly expressing a hepatocyte nuclear factor 1A-maturity-onset diabetes of the young mutation. (21628466)	Bonner C.... Prehn J.H.	2011	BMP3
2	Diabetes and pancreatic exocrine dysfunction due to m utations in the carboxyl ester lipase gene-maturity onset diabetes of the young (CEL-MODY): a protein misfolding disease. (21784842)	Johansson B.B.... RA	2011	CEL
3	Characterization of glucokinase polymorphisms associa ted with maturity-onset diabetes of the young (MODY2) in Jordanian population. (20458967)	Al-Sheyab F.... Khaul R.W.	2009	GCK
4	Maturity-onset diabetes of the young in children with incidental hyperglycemia: a multicenter Italian study of 172 families. (19564454)	Lorini R.... Barbetti F.	2009	GCK, HNF1A
5	Pancreatic exocrine dysfunction in maturity-onset diabetes of the young type 3. (17989309)	Vesterhus M.... Njolstad P.R.	2008	CEL, CELA3B
6	A case of new mutation in maturity-onset diabetes of the young type 3 (MODY 3) responsive to a low dose of sulphonylurea. (18433912)	Jesic M.D.... Necic S.	2008	HNF1A
7	Mutations of maturity-onset diabetes of the young (MODY) genes in Thais with early-onset type 2 diabetes mellitus. (18811724)	Plengvidhya N.... Yenchitsomanus P.T.	2008	GCK, HNF4A, NEUROD1
8	Biochemical characterization of novel glucokinase mutations isolated from Spanish maturity-onset diabetes of the young (MODY2) patients. (18322640)	Estalella I.... Sanz P.	2008	GCK
9	Phenotype of a patient with a de novo mutation in the hepatocyte nuclear factor 1beta/maturity-onset diabetes of the young type 5 gene. (18249217)	Mayer C.... Stumvoll M.	2008	HNF1B
10	Novel glucokinase mutation in a boy with maturity-onset diabetes of the young (19069349)	MilenkoviA8 T.... MitroviA8 K.	2008	GCK
11	The type and the position of HNF1A mutation modulate age at diagnosis of diabetes in patients with maturity-onset diabetes of the young (MODY)-3. (18003757)	Bellanne-Chantelot C.... Timsit J.	2008	HNF1A
12	Clinical application of 1,5-anhydroglucitol measurements in patients with hepatocyte nuclear factor-1alpha maturity-onset diabetes of the young. (18492944)	Skupien J.... Malecki M.T.	2008	HNF1A
13	Diabetic ketoacidosis with a fatal issue: is it a MO DY3 (maturity-onset diabetes of the young type 3)? (18390429)	Dehan C.... Gardet V.	2008	HNF4A
14	Partial and whole gene deletion mutations of the GCK and HNF1A genes in maturity-onset diabetes of the young. (17828387)	Ellard S.... Hattersley A.T.	2007	GCK, HNF4A, GCKR
15	No major contribution of TCF7L2 sequence variants to maturity onset of diabetes of the young (MODY) or neonatal diabetes mellitus in French white subjects. (17093940)	Cauchi S.... Froguel P.	2007	TCF7L2
16	Exonic duplication of the hepatocyte nuclear factor-1beta gene (transcription factor 2, hepatic) as a cause of maturity onset diabetes of the young type 5. (17440011)	Carette C.... Bellanne-Chantelot C.	2007	HNF1B
17	Evaluation of common variants in the six known maturity-onset diabetes of the young (MODY) genes for association with type 2 diabetes. (17327436)	Winckler W.... Altshuler D.	2007	GCK, HNF4A, NEUROD1
18	Identification of novel and recurrent glucokinase mut ations in Belgian and Luxembourg maturity onset diabetes of the young patients. (16965331)	Vits L.... Wuyts W.	2006	GCK
19	Mutations in the genes encoding the transcription factors hepatocyte nuclear factor 1 alpha (HNF1A) and 4 alpha (HNF4A) in maturity-onset diabetes of the young. (16917892)	Ellard S.... Colclough K.	2006	HNF4A, HNF1A
20	Conditional expression of hepatocyte nuclear factor-1beta, the maturity-onset diabetes of the young-5 gene product, influences the viability and functional competence of pancreatic beta-cells. (16837621)	Welters H.J.... Morgan N.G.	2006	HNF1B
21	A prevalent amino acid polymorphism at codon 98 (Ala98Val) of the hepatocyte nuclear factor-1alpha is associated with maturity-onset diabetes of the young and younger age at onset of type 2 diabetes in Asian Indians. (16186275)	Anuradha S.... Mohan V.	2005	HNF1A
22	Genetic testing for maturity onset diabetes of the young: uptake, attitudes and comparison with hereditary non-polyposis colorectal cancer. (15660263)	Liljestrom B.... Tuomi T.	2005	PCBD1
23	Genetic and clinical characteristics of maturity-onset diabetes of the young in Chinese patients. (15657605)	Xu J.Y.... Lam K.S.	2005	GCK, HNF1A
24	The G115S mutation associated with maturity-onset diabetes of the young impairs hepatocyte nuclear factor 4alpha activities and introduces a PKA phosphorylation site in its DNA-binding domain. (15233628)	Oxombre B.... Laine B.	2004	HNF4A
25	'I don't feel like a diabetic any more': the impact of stopping insulin in patients with maturity onset diabetes of the young following genetic testing. (15139733)	Shepherd M.... Hattersley A.T.	2004	HNF1A
26	Identification of 21 novel glucokinase (GCK) mutations in UK and European Caucasians with maturity-onset diabetes of the young (MODY). (14517956)	Thomson K.L.... Ellard S.	2003	GCK
27	Glucokinase (GCK) mutations in hyper- and hypoglycemia: maturity-onset diabetes of the young, permanent neonatal diabetes, and hyperinsulinemia of infancy. (14517946)	Gloyn A.L.	2003	GCK
28	Determinants of the development of diabetes (maturity-onset diabetes of the young-3) in carriers of HNF-1alpha mutations: evidence for parent-of-origin effect. (12453976)	Klupa T.... Krolewski A.S.	2002	HNF1A
29	GCK and HNF1A mutations in Canadian families with maturity onset diabetes of the young (MODY). (12442280)	Cao H.... Hegele R.A.	2002	GCK, HNF1A
30	Maturity-onset diabetes of the young caused by a balanced translocation where the 20q12 break point results in disruption upstream of the coding region of hepatocyte nuclear factor-4alpha (HNF4A) gene. (12086970)	Gloyn A.L.... Hattersley A.T.	2002	HNF4A
31	Different genes, different diabetes: lessons from maturity-onset diabetes of the young. (12173691)	Stride A.... Hattersley A.T.	2002	HNF4A, PDX1, HNF1B
32	Nine novel mutations in maturity-onset diabetes of the young (MODY) candidate genes in 22 Spanish families. (12050210)	Barrio R.... Mustieles C.	2002	GCK, HNF4A, HNF1A
33	Abnormal splicing of hepatocyte nuclear factor 1 alpha in maturity-onset diabetes of the young. (12378390)	Bulman M.P.... Ellard S.	2002	HNF1A
34	Molecular mechanisms and clinical pathophysiology of maturity-onset diabetes of the young. (11575290)	Fajans S.S.... Polonsky K.S.	2001	NEUROD1
35	Single-strand conformation polymorphism analysis of the glucose transporter gene GLUT1 in maturity-onset diabetes of the young. (11485019)	Baroni M.G.... Barbetti F.	2001	SLC2A2
36	Genetic variation in the hepatocyte nuclear factor (HNF)-3alpha gene does not contribute to maturity-onset diabetes of the young in Japanese. (11355750)	Yu L.... Takeda J.	2001	FOXA1
37	Mutations in the coding region of the neurogenin 3 gene (NEUROG3) are not a common cause of maturity-onset diabetes of the young in Japanese subjects. (11246894)	Del Bosque-Plata L.... Bell G.I.	2001	NEUROG3
38	Identification of three missense mutations in the peroxisome proliferator-activated receptor alpha gene in Japanese subjects with maturity-onset diabetes of the young. (11355020)	Hara M.... Bell G.I.	2001	PPARA
39	Genetic variation in the hepatocyte nuclear factor-3beta gene (HNF3B) does not contribute to maturity-onset diabetes of the young in French Caucasians. (10868949)	Abderrahmani A.... Froguel P.	2000	HNF4A, FOXA2
40	Missense mutations in the human insulin promoter factor-1 gene and their relation to maturity-onset diabetes of the young and late-onset type 2 diabetes mellitus in caucasians. (10720084)	Hansen L.... Pedersen O.	2000	HNF4A, PDX1, HNF1B
41	Hepatocyte nuclear factor 1 alpha (HNF-1 alpha) mutations in maturity-onset diabetes of the young. (11058894)	Ellard S.	2000	HNF1A
42	Phenotypic characteristics of early-onset autosomal-dominant type 2 diabetes unlinked to known maturity-onset diabetes of the young (MODY) genes. (10333942)	Doria A.... Krolewski A.S.	1999	HNF4A, HNF1A
43	Insulin secretion and insulin sensitivity in diabetic and non-diabetic subjects with hepatic nuclear factor-1alpha (maturity-onset diabetes of the young-3) mutations. (10601964)	Vaxillaire M.... Velho G.	1999	PCBD1, HNF1A
44	A novel missense mutation in the homeodomain of the hepatocyte nuclear factor-1alpha/maturity-onset diabetes of the young 3 in a Japanese early-onset type 2 diabetic patient and time-course of glucose-stimulated insulin secretion. (9727913)	Awata T.... Katayama S.	1998	HNF1A
45	Liver and kidney function in Japanese patients with maturity-onset diabetes of the young. (9839108)	Iwasaki N.... Iwamoto Y.	1998	GGT1, HNF4A, HNF1A
46	Mutations in the hepatocyte nuclear factor-1alpha gene are a common cause of maturity-onset diabetes of the young in the U.K. (9075818)	Frayling T.M.... Hattersley A.T.	1997	HNF1A
47	A rapid screening method for hepatocyte nuclear factor 1 alpha frameshift mutations; prevalence in maturity-onset diabetes of the young and late-onset non-insulin dependent diabetes. (9439666)	Frayling T.M.... Ellard S.	1997	HNF1A
48	Mutations in the hepatocyte nuclear factor-4alpha gene in maturity- onset diabetes of the young (MODY1). (8945471)	Yamagata K.... Bell G.I.	1996	GCK, HNF4A, HNF1A
49	cDNA sequence and localization of polymorphic human cytosolic phosphoenolpyruvate carboxykinase gene (PCK1) to chromosome 20, band q13.31: PCK1 is not tightly linked to maturity-onset diabetes of the young. (8490617)	Stoffel M.... Bell G.I.	1993	PCK1, PCK2
50	Missense glucokinase mutation in maturity-onset diabetes of the young and mutation screening in late-onset diabetes. (1303265)	Stoffel M.... Wainscoat J.S.	1992	GCK

Genes related to maturity-onset diabetes of the young according to GeneCards:

(show top 50)    (show all 84)

id	Symbol	Description	Score	GeneCards Section Context
1	GCK	glucokinase (hexokinase 4)	11.1	Pathways & Interactions, Aliases & Descriptions, Orthologs, Publications (show all 5 sections) Summaries
2	KLF11	Kruppel-like factor 11	11.1	Summaries
3	HNF1A	HNF1 homeobox A	11.1	Publications, Summaries, Pathways & Interactions
4	PDX1	pancreatic and duodenal homeobox 1	11.1	Publications, Pathways & Interactions, Summaries
5	GCKR	glucokinase (hexokinase 4) regulator	11.1	Publications, Summaries
6	FOXA2	forkhead box A2	11.1	Publications, Pathways & Interactions, Summaries
7	ISL1	ISL LIM homeobox 1	11.1	Summaries
8	CEL	carboxyl ester lipase (bile salt-stimulated lipase)	11.1	Publications
9	INS	insulin	11.1	Publications, Pathways & Interactions
10	NEUROD1	neuronal differentiation 1	11.1	Publications, Pathways & Interactions
11	HNF4A	hepatocyte nuclear factor 4, alpha	11.1	Pathways & Interactions, Publications
12	PAX4	paired box 4	11.1	Pathways & Interactions, Publications
13	BLK	B lymphoid tyrosine kinase	11.1
14	HNF1B	HNF1 homeobox B	11.0	Publications, Pathways & Interactions
15	CELA3B	chymotrypsin-like elastase family, member 3B	11.0	Publications
16	ACVR2B	activin A receptor, type IIB	11.0	Publications
17	ACVR2A	activin A receptor, type IIA	11.0	Publications
18	PPP1R3B	protein phosphatase 1, regulatory subunit 3B	11.0	Summaries
19	MIR1273D	microRNA 1273d	11.0
20	BHLHA15	basic helix-loop-helix family, member a15	11.0	Pathways & Interactions
21	PCBD1	pterin-4 alpha-carbinolamine dehydratase/dimerization cofactor of hepatocyte nuclear factor 1 alpha	11.0	Publications
22	NKX6-1	NK6 homeobox 1	11.0	Pathways & Interactions
23	NKX2-2	NK2 homeobox 2	11.0	Pathways & Interactions, Publications
24	MAFA	v-maf musculoaponeurotic fibrosarcoma oncogene homolog A (avian)	11.0	Pathways & Interactions
25	NEUROG3	neurogenin 3	11.0	Publications, Pathways & Interactions
26	MNX1	motor neuron and pancreas homeobox 1	11.0	Pathways & Interactions
27	HMX1	H6 family homeobox 1	11.0
28	FOXA3	forkhead box A3	11.0	Pathways & Interactions
29	FABP12	fatty acid binding protein 12	11.0	Publications
30	FOXA1	forkhead box A1	11.0	Publications
31	NR4A2	nuclear receptor subfamily 4, group A, member 2	11.0	Orthologs
32	FOXM1	forkhead box M1	11.0	Publications
33	ANKS4B	ankyrin repeat and sterile alpha motif domain containing 4B	11.0	Disorders
34	PCK1	phosphoenolpyruvate carboxykinase 1 (soluble)	11.0	Publications
35	APOC2	apolipoprotein C-II	11.0	Publications
36	NR4A3	nuclear receptor subfamily 4, group A, member 3	11.0	Orthologs
37	BMP3	bone morphogenetic protein 3	11.0	Publications
38	CYB5R4	cytochrome b5 reductase 4	11.0	Publications
39	ONECUT1	one cut homeobox 1	11.0	Pathways & Interactions
40	PCK2	phosphoenolpyruvate carboxykinase 2 (mitochondrial)	11.0	Publications
41	HNF4G	hepatocyte nuclear factor 4, gamma	11.0	Pathways & Interactions
42	SLC2A2	solute carrier family 2 (facilitated glucose transporter), member 2	11.0	Publications, Pathways & Interactions
43	TAF6	TAF6 RNA polymerase II, TATA box binding protein (TBP)-associated factor, 80kDa	11.0
44	GLP1R	glucagon-like peptide 1 receptor	11.0	Publications
45	HHEX	hematopoietically expressed homeobox	11.0	Pathways & Interactions
46	HES1	hairy and enhancer of split 1, (Drosophila)	11.0	Pathways & Interactions
47	RBP1	retinol binding protein 1, cellular	11.0	Publications
48	TMEM27	transmembrane protein 27	11.0
49	ALDOB	aldolase B, fructose-bisphosphate	11.0	Publications
50	PKLR	pyruvate kinase, liver and RBC	11.0	Publications, Pathways & Interactions

Pathways related to maturity-onset diabetes of the young according to GeneDecks:

(show all 19)

id	Pathway	Score	Top Affiliating Genes
1	Regulation of gene expression in endocrine-committed (NEUROG3+) progenitor cells38	10.4	NEUROD1, NKX2-2, PAX4
2	Gene Expression38	10.4	TAF6, NR4A3, NR1I2, HNF4A
3	Glucose transport38	10.4	SLC2A2, HK2, GCK, GCKR
4	Regulation of gene expression in early pancreatic precursor cells38	10.3	ONECUT1, PDX1, NKX6-1
5	Regulation of gene expression in late stage (branching morphogenesis) pancreatic bud precursor cells38	10.3	HES1, NEUROG3
6	Transcriptional Regulatory Network in Embryonic Stem Cell36	10.3	PAX6, ISL1, HNF4A, ONECUT1, NEUROD1
7	Transcription factors in neurogenesis10	10.0	NEUROG3, NEUROD1, NKX2-2, HES1, PAX6
8	MODY (Maturity-Onset Diabetes of Young)36	10.0	KCNU1, NEUROD1, SLC2A2, HNF4A, INS, FOXA2
9	Glycolysis and gluconeogenesis (short map)10	9.9	GAPDH, HK2, PKLR, ALDOB, GCK, PCK1
10	Glycolysis and gluconeogenesis (short map)41	9.9	GAPDH, HK2, PKLR, ALDOB, GCK, PCK1
11	Glycolysis / Gluconeogenesis20	9.8	GAPDH, HK2, PKLR, ALDOB, GCK, PCK1
12	PPAR signaling pathway20	9.8	NR1H3, PPARA, FABP2, APOC3, PCK1, PCK2
13	Anti-diabetic Drug Potassium Channel Inhibitors Pathway, Pharmacodynamics34	9.5	PDX1, HNF4A, HNF1B, HNF1A, MAFA, INS
14	Selected targets of HNF110	9.4	SLC2A2, DPP4, PDX1, NR5A2, NR1I2, NR1H4
15	Type II diabetes mellitus20	9.3	SLC2A2, HK2, PDX1, PKLR, MAFA, INS
16	Insulin signaling pathway20	9.3	PRKAB1, HK2, PKLR, PPP1R3B, INS, IRS1
17	Maturity onset diabetes of the young20	9.2	HHEX, GCK, HNF1A, IAPP, MAFA, INS
18	Adipocytokine signaling pathway20	8.8	PCK2, PCK1, IRS1, PPARA, PRKAB1
19	Glucose / Energy Metabolism3	8.7	PDX1, HK2, PRKAB1, NEUROD1, GAPDH, HNF4A

Compounds related to maturity-onset diabetes of the young according to GeneDecks:

(show top 50)    (show all 136)

id	Compound	Score	Top Affiliating Genes
1	i-app32	10.1	PDX1, IAPP, INS, GCG, GCK
2	katp32	10.1	GCG, SLC2A2, PDX1, INS, FOXA2, KCNJ11
3	proglucagon32	10.0	PDX1, TCF7L2, GCK, PAX6, GLP1R, ISL1
4	tolbutamide32 34 9 9	12.8	HK2, HNF1A, INS, KCNJ11, GCG, GCK
5	vildagliptin32 9 9	11.8	GCG, INS, GLP1R, DPP4
6	incretin32	9.7	GLP1R, INS, PDX1, GCG, DPP4, GCK
7	fructose32 9 9	11.7	PCK2, GCK, GCG, NR1H4, SLC2A2, ALDOB
8	liraglutide32 9 9	11.7	INS, IAPP, DPP4, GLP1R, GCG
9	exenatide32 9 9	11.7	DPP4, IAPP, INS, GCG, GLP1R
10	sitagliptin32 9 9	11.7	INS, DPP4, GCG, GLP1R, IAPP
11	triacylglycerol32	9.7	CEL, PCK2, GCG, INS, APOC2, APOC3
12	pyruvate32	9.6	GAPDH, SLC2A2, HK2, PKLR, HNF4A, HNF1A
13	glucose32	9.3	SLC2A2, HK2, PDX1, ONECUT1, ALDOB, MAFA
14	repaglinide32 42 9 9	12.3	NR1H2, GCG, KCNJ11, INS, IAPP
15	androstane32	9.1	NR1H4, NR1I2, NR1I3, PPARA, HNF4A, HNF1A
16	phosphoenolpyruvate32 9 9	11.0	SLC2A2, HK2, PCK2, PCK1, GCK, FOXM1
17	chenodeoxycholic acid32 9 18 9	12.0	HNF4A, VDR, NR1H2, NR1H4, NR1I2, NR1I3
18	rifampicin32	8.9	NR1I2, GAPDH, IAPP, HNF4A, PPARA, NR1H2
19	sulfonylurea32	8.8	GCG, KCNJ11, IRS1, INS, IAPP, GLP1R
20	2-deoxyglucose32	8.7	GAPDH, HK2, GCK, GCG, IRS1, INS
21	streptozotocin32	8.6	GCK, IRS1, GAD1, SLC2A2, DPP4, PDX1
22	oligonucleotide32	8.6	HNF1A, HNF1B, HNF4A, HLA-DQB1, PCK2, IAPP
23	retinoid32	8.5	GAPDH, NR4A2, NR4A1, NR1I2, PCK2, VDR
24	thiazolidinedione32	8.4	PPARA, PCK2, NR1H2, INS, IRS1, DPP4
25	c-peptide32	8.3	SLC2A2, GAD1, GCK, GCG, FOXA2, IRS1
26	vitamin a32 9 18 9	11.3	VDR, GGT1, FABP12, NR4A1, NR1I2, NR1H2
27	cysteine32	8.2	VDR, APOC2, IAPP, GLP1R, GCK, GCG
28	lactate32	8.2	GGT1, PRKAB1, ADA, DPP4, HK2, INS
29	ibmx32	8.2	ADA, INS, IAPP, PPARA, NR1H2, SLC2A2
30	rosiglitazone32 9 18 9	11.0	INS, PCK1, IRS1, PPARA, NR1H2, NR1I2
31	glutamine32	8.0	KCNJ3, GCG, GCK, PCK2, KCNJ11, GAPDH
32	testosterone32 9 18 9	11.0	NR4A1, NR1I3, NR1I2, NR1H4, NR1H2, HNF4A
33	glycerol32 9 18 9	10.9	INS, PCK2, GAPDH, ADA, DPP4, NR1H2
34	creatinine32	7.7	APOC3, PPARA, HK2, DPP4, ADA, GAD1
35	sterol32	7.7	FOXM1, IRS1, HNF1A, HNF4A, PPARA, NR1H2
36	metformin32 34 9 9	10.6	NR1H2, GCK, GCG, KCNJ11, IRS1, INS
37	steroid32	7.6	NR1H2, PPARA, HNF4A, HNF1A, IGFBP2, APOC3
38	atp32	7.5	ADA, PKLR, GAD1, GAPDH, SLC2A2, PCK2
39	glycogen32 18	8.4	PCK2, PCK1, GCKR, HES1, PKLR, PPP1R3B
40	dexamethasone32 42 34 9 9	11.1	NR1H2, HNF4A, NR1H4, NR1I2, NR1I3, NR4A1
41	fatty acid32	7.1	PPARA, HNF4A, HNF1A, GLP1R, FABP2, FABP12
42	cholesterol32 9 18 9	9.9	GGT1, GAD1, PRKAB1, DPP4, NR5A2, FABP12
43	glutamate32	6.8	GCG, VDR, KCNJ11, PCK2, IRS1, INS
44	arginine32	6.7	KCNJ11, ADA, SLC2A2, NR1I3, NR1H4, NR1H2
45	estrogen32	6.5	HNF4A, HES1, GAPDH, ADA, NR5A2, APOC3
46	alanine32	6.2	HNF4A, PPARA, NR1H2, NR1I3, NR4A1, ACVR2A
47	tyrosine32	6.2	NR4A3, NR1H4, DPP4, PCK2, BLK, INS
48	lipid32	6.1	GCKR, PCK1, PCK2, TCF7L2, HNF4A, HNF1A
49	serine32	5.7	IAPP, IGFBP2, APOC2, IRS1, CEL, PAX6
50	retinoic acid32 42 18	7.1	GAPDH, ADA, NEUROD1, DPP4, HES1, ACVR2B

Cellular components related to maturity-onset diabetes of the young according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	nucleoplasm	GO:005654	7.6	NR1I3, NR4A1, NR4A2, NR4A3, NR5A2, TCF7L2
2	nucleus	GO:005634	4.1	GAPDH, NFIB, NFIA, NR5A2, NR4A3, NR4A2

Biological processes related to maturity-onset diabetes of the young according to GeneDecks:

(show top 50)    (show all 52)

id	Name	GO ID	Score	Top Affiliating Genes
1	detection of glucose	GO:051594	10.8	GCK, PDX1, NKX6-1
2	cellular glucose homeostasis	GO:001678	10.7	FOXA3, GCK, GCKR
3	positive regulation of cell differentiation	GO:045597	10.7	NEUROD1, GLP1R, INS, PAX4
4	signal transduction involved in regulation of gene expression	GO:023019	10.7	NEUROD1, HNF4A, FOXA2, PAX6
5	insulin secretion	GO:030073	10.6	NEUROD1, ACVR2B, PDX1, CYB5R4, HNF1B, HNF1A
6	nitric oxide mediated signal transduction	GO:007263	10.6	NEUROD1, PDX1, MAFA
7	positive regulation of sequence-specific DNA binding transcription factor activity	GO:051091	10.6	NEUROG3, NEUROD1, NKX6-1, NKX2-2, FOXA1
8	anterior/posterior pattern specification	GO:009952	10.5	HHEX, NEUROD1, ACVR2A, ACVR2B, NR2F2, HNF1B
9	glucose transport	GO:015758	10.5	GCKR, GCK, INS, HK2, SLC2A2
10	triglyceride homeostasis	GO:070328	10.5	NR1H3, HNF4A, APOC3, APOC2, GCKR
11	smoothened signaling pathway	GO:007224	10.5	NKX6-1, NKX2-2, HES1, PAX6
12	positive regulation of insulin secretion	GO:032024	10.4	NKX6-1, TCF7L2, GLP1R, ISL1, BLK, GCK
13	pancreas development	GO:031016	10.4	ISL1, ACVR2B, TCF7L2, NKX6-1, HHEX
14	hepatoblast differentiation	GO:061017	10.4	HNF1B, HHEX
15	hexose transport	GO:008645	10.4	SLC2A2, HK2, GCK, GCKR
16	pancreatic PP cell fate commitment	GO:003329	10.4	NEUROD1, NKX2-2
17	response to cAMP	GO:051591	10.3	PAX4, ALDOB, HNF4A, PKLR
18	regulation of pronephros size	GO:035565	10.3	HNF1B, HNF1A
19	liver development	GO:001889	10.3	ADA, HES1, PDX1, ONECUT1, ALDOB
20	organ morphogenesis	GO:009887	10.2	NKX6-1, PDX1, PAX6, PAX4, VDR
21	regulation of transcription from RNA polymerase II promoter	GO:006357	10.2	TCF7L2, NR2F2, HNF4G, HNF4A, HNF1A, FOXA2
22	regulation of insulin secretion	GO:050796	10.2	NEUROD1, SLC2A2, HNF4A, GLP1R, INS, KCNJ11
23	glycolysis	GO:006096	10.1	GAPDH, HK2, PKLR, ALDOB, GCK
24	negative regulation of glucokinase activity	GO:033132	10.1	FOXA2, GCKR
25	lipid metabolic process	GO:006629	10.1	NR2F2, PPARA, HNF4A, APOC3, APOC2, CEL
26	gluconeogenesis	GO:006094	10.1	PCK2, PCK1, ALDOB, GAPDH
27	energy reserve metabolic process	GO:006112	10.1	SLC2A2, PKLR, GLP1R, INS, KCNJ11, GCG
28	response to glucose stimulus	GO:009749	10.0	NEUROD1, SLC2A2, TCF7L2, ACVR2B, PKLR, NR1H4
29	positive regulation of fatty acid biosynthetic process	GO:045723	10.0	NR1H3, NR1H2, HNF4A, APOC2
30	response to drug	GO:042493	10.0	GAD1, NEUROD1, NKX6-1, HNF4A, ALDOB, IGFBP2
31	positive regulation of lipoprotein lipase activity	GO:051006	9.9	APOC2, NR1H2, NR1H3
32	positive regulation of cellular protein metabolic process	GO:032270	9.9	INS, NR1H2, NR1H3
33	glucose metabolic process	GO:006006	9.9	GAPDH, PDX1, PKLR, ONECUT1, ALDOB, INS
34	carbohydrate metabolic process	GO:005975	9.9	GAPDH, SLC2A2, HK2, PKLR, ALDOB, GCK
35	endocrine pancreas development	GO:031018	9.8	HES1, PDX1, PKLR, ONECUT1, HNF4G, HNF4A
36	negative regulation of cholesterol storage	GO:010887	9.8	PPARA, NR1H2, NR1H3
37	positive regulation of glycogen biosynthetic process	GO:045725	9.8	INS, IRS1, GCK
38	intracellular receptor mediated signaling pathway	GO:030522	9.8	NR5A2, NR4A2, NR4A1, NR2F2, NR1I3, NR1I2
39	negative regulation of macrophage derived foam cell differentiation	GO:010745	9.7	NR1H3, NR1H2, PPARA
40	transcription from RNA polymerase II promoter	GO:006366	9.7	TAF6, PDX1, NFIX, NFIC, NFIB, FOXM1
41	negative regulation of pinocytosis	GO:048550	9.7	NR1H3, NR1H2
42	positive regulation of transcription, DNA-dependent	GO:045893	9.6	PPARA, NR1H3, NR1I2, NR2F2, NR5A2, NEUROD1
43	glucose homeostasis	GO:042593	9.4	NEUROD1, TCF7L2, PDX1, CYB5R4, HNF4A, HNF1A
44	negative regulation of transcription, DNA-dependent	GO:045892	9.0	HHEX, TCF7L2, HES1, NR2F2, NR1I2, NR1H2
45	transcription initiation from RNA polymerase II promoter	GO:006367	8.5	NR1I3, NR4A1, NR4A2, NR4A3, NR5A2, TAF6
46	gene expression	GO:010467	8.5	TAF6, NR5A2, NR4A3, NR4A2, NR4A1, NR1I3
47	negative regulation of transcription from RNA polymerase II promoter	GO:000122	8.5	NFIB, NFIC, NFIX, PDX1, HES1, TCF7L2
48	small molecule metabolic process	GO:044281	8.4	ALDOB, GLP1R, PPARA, PKLR, HK2, SLC2A2
49	signal transduction	GO:007165	7.9	NR2F2, NR4A1, NR4A2, ACVR2B, PRKAB1, NR1I3
50	positive regulation of transcription from RNA polymerase II promoter	GO:045944	6.8	VDR, HNF4A, HNF1B, HNF1A, GLP1R, MAFA

Molecular functions related to maturity-onset diabetes of the young according to GeneDecks:

(show all 15)

id	Name	GO ID	Score	Top Affiliating Genes
1	RNA polymerase II core promoter proximal region sequence-specific DNA binding transcription factor activity involved in positive	GO:001077	10.3	NR4A3, ONECUT1, HNF4A, HNF1A, FOXA1
2	phosphoenolpyruvate carboxykinase (GTP) activity	GO:004613	10.3	PCK2, PCK1
3	thyroid hormone receptor activity	GO:004887	10.1	NR1H4, NR1I3, NR4A3
4	RNA polymerase II distal enhancer sequence-specific DNA binding transcription factor activity	GO:003705	10.0	NFIX, NR5A2, NR1H4, HNF4A, HNF1B
5	transcription regulatory region DNA binding	GO:044212	9.8	HHEX, TCF7L2, NR5A2, NR1H3, HNF4A, HNF1B
6	transcription coactivator activity	GO:003713	9.8	NEUROG3, NEUROD1, NKX2-2, NR1I3, NR1I2, NR1H4
7	retinoid X receptor binding	GO:046965	9.4	NR1H4, NR1H3, NR1H2, VDR
8	transcription factor binding	GO:008134	9.3	HHEX, NEUROD1, NKX2-2, TCF7L2, HES1, NFIA
9	protein homodimerization activity	GO:042803	9.2	HHEX, DPP4, HES1, NR2F2, HNF4A, HNF1B
10	ligand-activated sequence-specific DNA binding RNA polymerase II transcription factor activity	GO:004879	8.7	PPARA, NR1H2, NR1H3, NR1H4, NR1I2, NR1I3
11	steroid hormone receptor activity	GO:003707	8.5	VDR, HNF4A, HNF4G, PPARA, NR1H2, NR1H3
12	sequence-specific DNA binding transcription factor activity	GO:003700	7.4	KLF11, HNF1B, HNF4A, HNF4G, HMX1, PPARA
13	sequence-specific DNA binding	GO:043565	7.3	HMX1, NR5A2, PDX1, HES1, TCF7L2, NKX2-2
14	DNA binding	GO:003677	7.0	NR4A3, NR4A1, NR1I3, NR1H3, NR5A2, NFIA
15	protein binding	GO:005515	4.6	HES1, ACVR2A, ACVR2B, NR5A2, NR4A2, NR4A1