MCID: MTR004
MIFTS: 57

Maturity-Onset Diabetes of the Young

Categories: Rare diseases, Genetic diseases, Endocrine diseases, Neuronal diseases

Aliases & Classifications for Maturity-Onset Diabetes of the Young

MalaCards integrated aliases for Maturity-Onset Diabetes of the Young:

Name: Maturity-Onset Diabetes of the Young 12 50 24 56 29 14
Mody 12 50 56
Maturity Onset Diabetes Mellitus in Young 29 69
Mason-Type Diabetes 12
Mason Type Diabetes 50

Characteristics:

Orphanet epidemiological data:

56
mody
Inheritance: Autosomal dominant,Not applicable; Prevalence: 1-5/10000 (Europe),1-5/10000 (United Kingdom); Age of onset: Adolescent,Adult,Childhood;

Classifications:

Orphanet: 56  
Rare endocrine diseases


External Ids:

Disease Ontology 12 DOID:0050524
ICD10 33 E11.8
Orphanet 56 ORPHA552
UMLS via Orphanet 70 C0342276
ICD10 via Orphanet 34 E11.9

Summaries for Maturity-Onset Diabetes of the Young

NIH Rare Diseases : 50 maturity-onset diabetes of the young (mody) is a form of diabetes that is characterized by an early onset diabetes.  mody represents about 2% of all diabetes cases and is commonly misdiagnosed as type 1 or type 2 diabetes mellitus. it is due to a primary defect in pancreatic β-cell function. there are several mody subtypes with distinct genetic causes: mody1, caused by mutations in the hnf4a gene; mody2, caused by mutations in gck gene; mody3 caused by mutations in the hnfa1 (the most common type); mody4 caused by mutations in the pdx1 gene; mody5 caused by mutations in the hnf1b gene; mody6 caused by mutations in the neurod1 gene; mody7 caused by mutations in the klf11 gene; mody8 caused by mutations in the cel gene; mody9 caused by mutations in the pax4 gene; mody10 caused by mutations in the ins gene; mody11 caused by mutations in the blk gene; mody12 caused by mutations in the abcc8 gene; mody13  caused by heterozygous mutation in the kcnj11 gene; and mody14 caused by mutations in the appl1 gene. it is inherited in an autosomal dominant pattern. treatment depends on the subtype and may include sulfonylureas, insulin or diet and exercise. last updated: 4/20/2016

MalaCards based summary : Maturity-Onset Diabetes of the Young, also known as mody, is related to maturity-onset diabetes of the young 6 and maturity-onset diabetes of the young, type 13. An important gene associated with Maturity-Onset Diabetes of the Young is GCK (Glucokinase), and among its related pathways/superpathways are Maturity onset diabetes of the young and Developmental Biology. The drugs Moxonidine and Metformin have been mentioned in the context of this disorder. Affiliated tissues include breast, testes and brain, and related phenotypes are Decreased viability and homeostasis/metabolism

Disease Ontology : 12 A genetic disease that has material basis in mutations in the MODY genes disrupting insulin production.

Wikipedia : 72 \"Maturity onset diabetes of the young\" (MODY) refers to any of several hereditary forms of diabetes... more...

Related Diseases for Maturity-Onset Diabetes of the Young

Diseases in the Maturity-Onset Diabetes of the Young family:

Maturity-Onset Diabetes of the Young, Type Vii Maturity-Onset Diabetes of the Young 6
Maturity-Onset Diabetes of the Young, Type 14 Maturity-Onset Diabetes of the Young, Type Ix
Maturity-Onset Diabetes of the Young, Type 11 Maturity-Onset Diabetes of the Young, Type Viii
Maturity-Onset Diabetes of the Young, Type 10 Maturity-Onset Diabetes of the Young, Type 13

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

(show top 50) (show all 66)
id Related Disease Score Top Affiliating Genes
1 maturity-onset diabetes of the young 6 33.1 ABCC8 GCK GCKR HNF1A HNF1B HNF4A
2 maturity-onset diabetes of the young, type 13 12.7
3 maturity-onset diabetes of the young, type viii 12.7
4 maturity-onset diabetes of the young, type ix 12.7
5 maturity-onset diabetes of the young, type vii 12.7
6 maturity-onset diabetes of the young, type 11 12.7
7 maturity-onset diabetes of the young, type 10 12.6
8 maturity-onset diabetes of the young, type 14 12.6
9 fanconi renotubular syndrome 4, with maturity-onset diabetes of the young 12.6
10 renal cysts and diabetes syndrome 12.3
11 mody, type i 12.1
12 mody, type ii 12.1
13 mody, type iii 12.1
14 mody, type iv 12.1
15 17q12 deletion syndrome 11.0
16 hyperinsulinism due to glucokinase deficiency 11.0
17 hemochromatosis, type 4 10.9 HNF1A NEUROD1
18 prostate cancer, hereditary, 11 10.8 HNF1B HNF4A
19 antidepressant type abuse 10.8 GCK HNF1A INS KCNJ11
20 survival motor neuron spinal muscular atrophy 10.8 GCK INS KCNJ11 SLC2A2
21 adrenal cortical hypofunction 10.7 GCK PDX1
22 persistent hyperplastic primary vitreous, autosomal recessive 10.7 NEUROD1 NEUROG3
23 paroxysmal nonkinesigenic dyskinesia 2 10.7 INS NEUROD1
24 neuropathy, hereditary sensory and autonomic, type ic 10.6 HNF1A INS
25 pancreatitis 10.3
26 hypoglycemia 10.2
27 familial papillary thyroid carcinoma 10.2 ABCC8 HNF4A
28 writing disorder 10.2 ABCC8 GCK KCNJ11
29 hepatitis 10.2
30 diabetes mellitus, transient neonatal, 3 10.2 ABCC8 HNF4A KCNJ11
31 diabetes mellitus, permanent neonatal 10.2 ABCC8 GCK
32 retinitis pigmentosa 68 10.1 ABCC8 INS SLC2A2
33 labyrinthine unilateral reactive loss 10.1 ABCC8 GCK INS KCNJ11
34 acute insulin response 10.1 ABCC8 INS KCNJ11
35 neurogenic arthropathy 10.1 ABCC8 GCK INS KCNJ11
36 nephronophthisis 19 10.1 ABCC8 INS KCNJ11
37 chondroma 10.1 ABCC8 INS KCNJ11
38 inclusion body myopathy with paget disease of bone and frontotemporal dementia 10.1 ABCC8 GCK INS KCNJ11 PDX1
39 diabetic cataract 10.0 ABCC8 GCK HNF4A INS KCNJ11
40 atrial fibrillation, familial, 12 10.0 ABCC8 KCNJ11
41 diaphanospondylodysostosis 10.0 ABCC8 GCK INS KCNJ11 PDX1
42 triple x syndrome 10.0 ABCC8 GCK INS KCNJ11
43 duodenitis 10.0
44 hyperinsulinemic hypoglycemia 10.0
45 exostosis 10.0 ABCC8 GCK HNF4A INS KCNJ11
46 hypertrichosis 10.0 ABCC8 GCK INS KCNJ11 SLC2A2
47 intraocular lymphoma 9.9 ABCC8 GCK INS NEUROD1 PDX1 SLC2A2
48 hyperglycemia 9.9
49 kidney disease 9.9
50 obesity 9.9

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



Diseases related to Maturity-Onset Diabetes of the Young

Symptoms & Phenotypes for Maturity-Onset Diabetes of the Young

GenomeRNAi Phenotypes related to Maturity-Onset Diabetes of the Young according to GeneCards Suite gene sharing:

26
id Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability GR00221-A-1 9.86 GCK GCKR PKLR
2 Decreased viability GR00221-A-2 9.86 GCK PKLR
3 Decreased viability GR00221-A-3 9.86 APPL1 GCK
4 Decreased viability GR00221-A-4 9.86 APPL1 BLK GCK GCKR PKLR
5 Decreased viability GR00231-A 9.86 GCK
6 Decreased viability GR00301-A 9.86 PKLR
7 Decreased viability GR00342-S-3 9.86 PKLR
8 Decreased viability GR00381-A-1 9.86 APPL1
9 Decreased free cholesterol GR00340-A-2 9.33 GCKR HNF1A HNF4A
10 shRNA abundance <= 50% GR00343-S 9.32 BLK CEL GCK HNF1A HNF4A KCNJ11

MGI Mouse Phenotypes related to Maturity-Onset Diabetes of the Young:

44 (show all 11)
id Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.36 APPL1 CEL FOXA2 GCK GCKR HNF1A
2 growth/size/body region MP:0005378 10.31 APPL1 CEL FOXA2 GCK HNF1A HNF1B
3 endocrine/exocrine gland MP:0005379 10.3 PDX1 SLC2A2 ABCC8 BLK FOXA2 GCK
4 mortality/aging MP:0010768 10.2 NEUROG3 NKX2-2 PAX4 PDX1 PKLR SLC2A2
5 adipose tissue MP:0005375 10.04 APPL1 CEL FOXA2 HNF1A INS KCNJ11
6 digestive/alimentary MP:0005381 10.02 NEUROD1 NEUROG3 PDX1 CEL FOXA2 HNF1A
7 liver/biliary system MP:0005370 10.02 CEL FOXA2 GCK HNF1A HNF1B HNF4A
8 muscle MP:0005369 9.8 PDX1 SLC2A2 INS KCNJ11 APPL1 HNF1A
9 no phenotypic analysis MP:0003012 9.7 ABCC8 APPL1 HNF1A INS KCNJ11 NEUROG3
10 normal MP:0002873 9.65 NKX2-2 PAX4 PDX1 PKLR BLK FOXA2
11 renal/urinary system MP:0005367 9.17 GCK HNF1A HNF1B INS NEUROD1 NEUROG3

Drugs & Therapeutics for Maturity-Onset Diabetes of the Young

Drugs for Maturity-Onset Diabetes of the Young (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 218)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Moxonidine Approved Phase 4 75438-57-2 4810
2
Metformin Approved Phase 4,Phase 3,Phase 2 657-24-9 14219 4091
3
Cilostazol Approved Phase 4 73963-72-1 2754
4
Pitavastatin Approved Phase 4 147511-69-1, 147526-32-7 6366718 5282452
5
Insulin Aspart Approved Phase 4 116094-23-6 16132418
6
Insulin Detemir Approved Phase 4 169148-63-4 5311023
7
Zinc Approved Phase 4,Phase 1 7440-66-6 32051 23994
8
Glimepiride Approved Phase 4,Phase 2,Phase 3 93479-97-1 3476
9
Insulin Glargine Approved Phase 4 160337-95-1
10
Exenatide Approved, Investigational Phase 4,Phase 3 141758-74-9 15991534
11
Ethanol Approved Phase 4 64-17-5 702
12 Mirabegron Approved Phase 4 223673-61-8
13
Menthol Approved Phase 4,Phase 3 2216-51-5 16666
14
Aspirin Approved, Vet_approved Phase 4 50-78-2 2244
15 Hormones Phase 4,Phase 2,Phase 3,Phase 1
16 insulin Phase 4,Phase 3,Phase 2,Phase 1
17 Insulin, Globin Zinc Phase 4,Phase 3,Phase 2,Phase 1
18 Antihypertensive Agents Phase 4,Phase 3
19 Dipeptidyl-Peptidase IV Inhibitors Phase 4,Phase 3
20 HIV Protease Inhibitors Phase 4,Phase 3
21 Hormone Antagonists Phase 4,Phase 2,Phase 3,Phase 1
22 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2,Phase 3,Phase 1
23 Hypoglycemic Agents Phase 4,Phase 2,Phase 3,Phase 1
24 Incretins Phase 4,Phase 2,Phase 3,Phase 1
25
protease inhibitors Phase 4,Phase 3
26 Sitagliptin Phosphate Phase 4,Phase 3
27 Anti-Asthmatic Agents Phase 4,Phase 1
28 Autonomic Agents Phase 4
29 Bronchodilator Agents Phase 4
30 Fibrinolytic Agents Phase 4
31 Neuroprotective Agents Phase 4
32 Peripheral Nervous System Agents Phase 4,Phase 1
33 Phosphodiesterase 3 Inhibitors Phase 4
34 Phosphodiesterase Inhibitors Phase 4
35 Platelet Aggregation Inhibitors Phase 4
36 Protective Agents Phase 4,Phase 1
37 Respiratory System Agents Phase 4,Phase 1
38 Vasodilator Agents Phase 4
39 Anticholesteremic Agents Phase 4
40 Antimetabolites Phase 4,Phase 2
41 Atorvastatin Calcium Phase 4 134523-03-8
42 Calcium, Dietary Phase 4,Phase 3
43 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 4
44 Hypolipidemic Agents Phase 4,Phase 2
45 Lipid Regulating Agents Phase 4,Phase 2
46 Anti-Arrhythmia Agents Phase 4,Phase 2,Phase 3
47 Immunosuppressive Agents Phase 4,Phase 2,Phase 3,Phase 1
48 Neurotransmitter Agents Phase 4,Phase 3,Phase 1
49 Liver Extracts Phase 4,Phase 3
50 Analgesics Phase 4,Phase 1

Interventional clinical trials:

(show top 50) (show all 140)

id Name Status NCT ID Phase Drugs
1 Thyroid Study Type 2 Diabetes Mellitus (T2DM) Unknown status NCT01379170 Phase 4 Euthyrox (levothyroxine)
2 Study of the Effect of Moxonidine and Diet on Sympathetic Functions in Young Adults With Obesity Unknown status NCT01180231 Phase 4 Moxonidine (Physiotens)
3 Exploratory Study to Compare the Effects of Tenelia® or Januvia® on Glucose Variability in add-on to Metformin (CGMS Study) Unknown status NCT02512523 Phase 4 Teneligliptin;Sitagliptin
4 Effects of Cilostazol on Plasma Adipocytokine and Arterial Stiffness Unknown status NCT00573950 Phase 4 cilostazol;Placebo
5 Phenotypic and Genetic Correlates of Diabetes (Non-Type 1) in Young Non-Obese Asian Indians in North India and A Study to Evaluate the Efficacy of Sitagliptin (DPP-4 Inhibitor) in a Sub-group of the Study Population Completed NCT00964184 Phase 4 Metformin
6 Efficacy and Safety Study of Pitavastatin Compared to atoRvastatin in Type 2 dIabeTes Mellitus With Hypercholesterolemia Completed NCT00889226 Phase 4 Pitavastatin;Atorvastatin
7 Management of Type-2 Diabetic Patients Treated With Insulin During the Ramadan Completed NCT01354925 Phase 4 Insulin treatment during Ramadan;Standard of care
8 Optimal Oral Hypoglycaemic Agents (OHA) for Combination With Insulin Glargine (Sulfonylurea vs. Metformin) Completed NCT00708578 Phase 4 Glimepiride;Metformin;Insulin Glargine
9 Insulin Glargine (Lantus) vs Sulfonylurea (SU) for BETA Cell Function (BETA Study) Completed NCT00562172 Phase 4 Insulin glargine;Glimepiride;Metformin
10 Exenatide and Brown Adipose Tissue Recruiting NCT03002675 Phase 4 Bydureon
11 Mirabegron and Brown Adipose Tissue Recruiting NCT03012113 Phase 4 Mirabegron;Placebo Oral Capsule
12 A Study of Methoxy Polyethylene Glycol-Epoetin Beta (Mircera) for the Treatment of Chronic Renal Anemia in Participants With Diabetic Nephropathy Recruiting NCT01191983 Phase 4 Methoxy polyethylene glycol-epoetin beta [Mircera]
13 Effects of Dapagliflozin on Epicardial Fat in Subjects With Type 2 Diabetes Recruiting NCT02235298 Phase 4 Dapagliflozin;Metformin
14 Metformin for Ectopic Fat Deposition and Metabolic Markers in Polycystic Ovary Syndrome (PCOS) Recruiting NCT02500147 Phase 4 Metformin;Placebo
15 Diabetes Interventional Assessment of Slimming or Training to Lessen Inconspicuous Cardiovascular Dysfunction Recruiting NCT02590822 Phase 4
16 Metformin in Longevity Study Active, not recruiting NCT02432287 Phase 4 Metformin;Placebo
17 Effect of aSpirin Versus CilOstazol for Inhibition of Antiplatelet aggRegaTion in Type 2 DM Patients Not yet recruiting NCT02933788 Phase 4 Cilostazol;Acetylsalicylic acid
18 Carboplatin With Either Paclitaxel Poliglumex or Paclitaxel in Treating Women With Stage IIIB, Stage IV, or Recurrent Non-Small Cell Lung Cancer Unknown status NCT00551733 Phase 3 carboplatin;paclitaxel;paclitaxel poliglumex
19 The Effects of GLP-1 in Maturity-Onset Diabetes of The Young (MODY) Completed NCT01610934 Phase 2, Phase 3 liraglutide;Glimepiride
20 Safety and Efficacy of OSTEOFORM (rhPTH [1-34]) in Increasing Bone Mineral Density in Osteoporosis Completed NCT00500409 Phase 3 Osteoform;SHELCAL
21 Extension Study for 2993-112 Completed NCT01789957 Phase 3 AC2993
22 Evaluation of the Effect on Glucose Control of AC2993 in Patients With Type 2 Diabetes Mellitus Treated With a Sulfonylurea Completed NCT00039026 Phase 3 AC2993;AC2993;Placebo;Placebo
23 Type 2 Diabetes Primary Prevention for At Risk Girls Completed NCT00063674 Phase 2, Phase 3
24 Phase III Study for Glimepiride + Metformin Hydrochloride (Amaryl M) Slow Release (SR) Completed NCT00437554 Phase 3 Glimepiride
25 Effects of Liraglutide in Young Adults With Type 2 DIAbetes (LYDIA) Recruiting NCT02043054 Phase 3 Liraglutide;Sitagliptin
26 Safety and Efficacy of Bimatoprost Sustained-Release (SR) in Patients With Open-Angle Glaucoma or Ocular Hypertension Recruiting NCT02250651 Phase 3 Bimatoprost sustained-release;Timolol;Timolol Vehicle (placebo)
27 An Open-Label, Long-Term Study of Oral Treprostinil in Subjects With Pulmonary Arterial Hypertension Recruiting NCT01560637 Phase 3 UT-15C (treprostinil diethanolamine)
28 Trial of the Early Combination of Oral Treprostinil With Background Oral Monotherapy in Subjects With Pulmonary Arterial Hypertension Recruiting NCT01560624 Phase 3 UT-15C (treprostinil diethanolamine);Placebo
29 Randomized Global Phase 3 Study to Evaluate the Impact on NASH With Fibrosis of Obeticholic Acid Treatment Recruiting NCT02548351 Phase 3 Obeticholic Acid;Placebo
30 Effect of a Continuous Glucose Monitoring on Maternal and Neonatal Outcomes in Gestational Diabetes Mellitus Not yet recruiting NCT02838147 Phase 3
31 Efficacy of Foot Orthotics in Veterans With Chronic Lower Back Pain Terminated NCT01865539 Phase 2, Phase 3
32 A Study to Determine the Safety & Efficacy of Autologous Human Platelet Lysate (HPL) in Treatment of Dark Circles Unknown status NCT01644448 Phase 1, Phase 2
33 Clinical Trial of YH14618 in Patients With Degenerative Disc Disease Completed NCT02320019 Phase 2 Placebo;YH14618
34 Open-label Study of STA-9090 for Patients With Metastatic Breast Cancer Completed NCT01273896 Phase 2 STA-9090
35 RTA 408 Ophthalmic Suspension for the Prevention of Corneal Endothelial Cell Loss Following Cataract Surgery - GUARD Completed NCT02128113 Phase 2 Placebo;0.5% Omaveloxolone Ophthalmic Suspension;1% Omaveloxolone Ophthalmic Suspension
36 A Clinical Trial to Evaluate the Safety and Efficacy of ZYH7 Compared to Fenofibrate in Patients With Dyslipidemia Completed NCT01539616 Phase 2 ZYH7;ZYH7;ZYH7;Fenofibrate
37 Partners for Better Health in Adolescent Type 2 Diabetes: The Buddy Study Completed NCT01007266 Phase 2
38 Stem Cell Educator Therapy in Type 2 Diabetes Completed NCT01415726 Phase 1, Phase 2
39 Mobile Device Outcomes-based Rehabilitation Program Recruiting NCT02891707 Phase 1, Phase 2
40 Bone Marrow Transplantation vs Standard of Care in Patients With Severe Sickle Cell Disease (BMT CTN 1503) Recruiting NCT02766465 Phase 2 Busulfan;Fludarabine;r-ATG;Tacrolimus;Methotrexate
41 Hypogonadism in Young Men With Type 2 Diabetes Terminated NCT01155518 Phase 2 testosterone;clomiphene;placebo;placebo
42 Evaluate Safety and Effectiveness of MBX-102 in Type 2 Diabetes Patients With Poor Glycemic Control on Metformin Terminated NCT00814372 Phase 2 MBX-102;Placebo;Actos;Metformin
43 Bioequivalence Study of Zafirlukast Tablets 20 mg of Dr. Reddy's Laboratories Limited Under Fasting Condition Completed NCT01283061 Phase 1 Zafirlukast
44 Bioavailability Study of Anastrozole Tablets 1 mg of Dr.Reddy's Under Fed Conditions Completed NCT01155960 Phase 1 Anastrozole
45 Bioavailability Study of Duloxetine Delayed-Relase Capsules 60 mg Under Fed Condition Completed NCT02291341 Phase 1 Duloxetine;Cymbalta®
46 Bioequivalence Study of Eszopiclone Tablets, 3 mg Under Fed Conditions Completed NCT02322658 Phase 1 Eszopiclone Tablets 3 mg
47 Bioequivalence Study of Eszopiclone Tablets, 3 mg Under Fasting Conditions Completed NCT02322645 Phase 1 Eszopiclone Tablets 3 mg
48 Comparative Bioavailability - Gender Effect - Single and Multiple Ascending Dose Safety and Pharmacokinetic Study of GFT505 Completed NCT01474161 Phase 1 GFT505 120mg - old formulation;GFT505 120mg - new formulation;GFT505 180mg - new formulation;GFT505 240mg - new formulation;GFT505 300mg - new formulation;Placebo
49 A Study of MK-0893 on Glucagon-Induced Glycemic Excursion in Healthy Male Participants Following Intravenous Administration of Glucagon, Sandostatine® and Insulin (MK-0893-002) Completed NCT02012166 Phase 1 MK-0893 10 mg;MK-0893 40 mg;MK-0893 200 mg;MK-0893 1000 mg;Placebo
50 Contribution of Hyperinsulinemia vs. Hyperglycemia to Insulin Resistance in Type 1 Diabetes and Maturity Onset Diabetes of the Young, Type 2 (MODY2) Recruiting NCT02971202 Phase 1 Hyperinsulinemic, euglycemic clamp;20% dextrose

Search NIH Clinical Center for Maturity-Onset Diabetes of the Young

Genetic Tests for Maturity-Onset Diabetes of the Young

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

id Genetic test Affiliating Genes
1 Maturity Onset Diabetes Mellitus in Young 29
2 Maturity-Onset Diabetes of the Young 29 24

Anatomical Context for Maturity-Onset Diabetes of the Young

MalaCards organs/tissues related to Maturity-Onset Diabetes of the Young:

39
Breast, Testes, Brain, Bone, Liver, Lung, Endothelial

Publications for Maturity-Onset Diabetes of the Young

Articles related to Maturity-Onset Diabetes of the Young:

(show top 50) (show all 319)
id Title Authors Year
1
Generation of an induced pluripotent stem cell (iPSC) line from a patient with maturity-onset diabetes of the young type 13 (MODY13) with a the potassium inwardly-rectifying channel, subfamily J, member 11 (KCNJ11) mutation. ( 28925365 )
2017
2
Hepatocyte nuclear factor 1I^ maturity-onset diabetes of the young in a Chinese child presenting with hyperglycemic hyperosmolar state. ( 28593362 )
2017
3
Phenotype Heterogeneity in Glucokinase-Maturity-Onset Diabetes of the Young (GCK-MODY) Patients. ( 28663157 )
2017
4
Efficacy and safety of alogliptin in a pediatric patient with maturity-onset diabetes of the young type 1. ( 28804210 )
2017
5
Identification of Maturity-Onset Diabetes of the Young Caused by Glucokinase Mutations Detected Using Whole-Exome Sequencing. ( 28555465 )
2017
6
A novel mutation in the hepatocyte nuclear factor-1I^ gene in maturity onset diabetes of the young 5 with multiple renal cysts and pancreas hypogenesis: A case report. ( 28912863 )
2017
7
Variation in Maturity-Onset Diabetes of the Young Genes Influence Response to Interventions for Diabetes Prevention. ( 28453780 )
2017
8
Incremental cost-effectiveness of algorithm-driven genetic testing versus no testing for Maturity Onset Diabetes of the Young (MODY) in Singapore. ( 28835481 )
2017
9
An analysis of the sequence of the BAD gene among patients with maturity-onset diabetes of the young (MODY). ( 27935851 )
2017
10
Maturity-onset diabetes of the young (MODY) in Brazil: Establishment of a national registry and appraisal of available genetic and clinical data. ( 28012402 )
2017
11
A case of a novel mutation in HNF1I^-related maturity-onset diabetes of the young type 5 with diabetic kidney disease complication in a Chinese family. ( 28502589 )
2017
12
Use of oral antidiabetic drugs in the treatment of maturity-onset diabetes of the young: A mini review. ( 28840639 )
2017
13
Comprehensive Maturity Onset Diabetes of the Young (MODY) Gene Screening in Pregnant Women with Diabetes in India. ( 28095440 )
2017
14
A Single Nucleotide Variant in HNF-1I^ is Associated with Maturity-Onset Diabetes of the Young in a Large Chinese Family. ( 27114981 )
2016
15
Genetic Confirmation Rate in Clinically Suspected Maturity-Onset Diabetes of the Young. ( 27634015 )
2016
16
A three-step programmed method for the identification of causative gene mutations of maturity onset diabetes of the young (MODY). ( 27185633 )
2016
17
Studies of genetic variability of the hepatocyte nuclear factor-1I+ gene in an Indian maturity-onset diabetes of the young family. ( 27148439 )
2016
18
ABCC8-Related Maturity-Onset Diabetes of the Young (MODY12): Clinical Features and Treatment Perspective. ( 27538677 )
2016
19
GCK gene mutations are a common cause of childhood-onset MODY (maturity-onset diabetes of the young) in Turkey. ( 27256595 )
2016
20
Maturity-Onset Diabetes of the Young (MODY): Making the Right Diagnosis to Optimize Treatment. ( 27130141 )
2016
21
A review of maturity onset diabetes of the young (MODY) and challenges in the management of glucokinase-MODY. ( 27852188 )
2016
22
Maturity onset diabetes of the young 5 accompanied by duodenal cysts. ( 26676964 )
2016
23
Maturity-Onset Diabetes of the Young: A Genetic Form of Diabetes in Children. ( 27955956 )
2016
24
Maturity onset diabetes of the young: Seek and you will find. ( 27323672 )
2016
25
Divergent phenotypes in siblings with identical novel mutations in the HNF-1I+ gene leading to maturity onset diabetes of the young type 3. ( 27142837 )
2016
26
Searching for Maturity-Onset Diabetes of the Young (MODY): When and What for? ( 27103109 )
2016
27
Characteristics of maturity onset diabetes of the young in a large diabetes center. ( 26059258 )
2015
28
Establishment of maturity-onset diabetes of the young-induced pluripotent stem cells from a Japanese patient. ( 26417411 )
2015
29
Frameshift mutations in the insulin gene leading to prolonged molecule of insulin in two families with Maturity-Onset Diabetes of the Young. ( 25721872 )
2015
30
Molecular and phenotypic characteristics of maturity-onset diabetes of the young compared with early onset type 2 diabetes in China. ( 25588466 )
2015
31
A genetic diagnosis of maturity-onset diabetes of the young (MODY): experiences of patients and family members. ( 25763774 )
2015
32
Hepatocyte nuclear factor 1I+-inactivated hepatocellular adenomatosis in a patient with maturity-onset diabetes of the young type 3: case report and literature review. ( 26346360 )
2015
33
Maturity-onset diabetes of the young (MODY): an update. ( 25581748 )
2015
34
Urinary C-Peptide/Creatinine Ratio Can Distinguish Maturity-Onset Diabetes of the Young from Type 1 Diabetes in Children and Adolescents: A Single-Center Experience. ( 25792383 )
2015
35
Postprandial incretin and islet hormone responses and dipeptidyl-peptidase 4 enzymatic activity in patients with maturity onset diabetes of the young. ( 25953829 )
2015
36
Maturity-Onset Diabetes of the Young: What Do Clinicians Need to Know? ( 26706916 )
2015
37
Aberrant mRNA splicing of paired box 4 (PAX4) IVS7-1G>A mutation causing maturity-onset diabetes of the young, type 9. ( 25951767 )
2015
38
Hepatocyte nuclear factor-I+ genetic mutation in a Chinese pedigree with maturity-onset diabetes of the young (MODY3). ( 26436572 )
2015
39
Incretin hormones and maturity onset diabetes of the young--pathophysiological implications and anti-diabetic treatment potential. ( 26324089 )
2015
40
Hepatocellular Carcinoma Arising in an HNF-1I+-Mutated Adenoma in a 23-Year-Old Woman with Maturity-Onset Diabetes of the Young: A Case Report. ( 26676820 )
2015
41
Molecular diagnosis of maturity-onset diabetes of the young (MODY) in Turkish children by using targeted next-generation sequencing. ( 26226118 )
2015
42
The clinical management of hyperglycemia in pregnancy complicated by maturity-onset diabetes of the young. ( 25935773 )
2015
43
The complexity of elucidating whether novel mutations in the hepatocyte nuclear factor one alpha gene are responsible for maturity onset diabetes of the young in ethnic minorities. ( 26393317 )
2015
44
A novel heterozygous mutation in the glucokinase gene is responsible for an early-onset mild form of maturity-onset diabetes of the young, type 2. ( 25921421 )
2015
45
A novel nonsense mutation of the HNF1I+ in maturity-onset diabetes of the young type 3 in Asian population. ( 26050565 )
2015
46
Comparability of high-sensitivity CRP methods to detect maturity-onset diabetes of the young due to HNF1A mutations. ( 24974685 )
2014
47
Screening of diabetes of youth for hepatocyte nuclear factor 1 mutations: clinical phenotype of HNF1I^-related maturity-onset diabetes of the young and HNF1I+-related maturity-onset diabetes of the young in Japanese. ( 24905847 )
2014
48
Molecular and clinical characterization of glucokinase maturity-onset diabetes of the young (GCK-MODY) in Japanese patients. ( 24804978 )
2014
49
Maturity-onset diabetes of the young type 5 in a family with diabetes and mild kidney disease diagnosed by whole exome sequencing. ( 25367728 )
2014
50
Current and Best Practices of Genetic Testing for Maturity Onset Diabetes of the Young: Views of Professional Experts. ( 25341961 )
2014

Variations for Maturity-Onset Diabetes of the Young

Expression for Maturity-Onset Diabetes of the Young

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

Pathways for Maturity-Onset Diabetes of the Young

Pathways related to Maturity-Onset Diabetes of the Young according to KEGG:

37
id Name Kegg Source Accession
1 Maturity onset diabetes of the young hsa04950

Pathways related to Maturity-Onset Diabetes of the Young according to GeneCards Suite gene sharing:

(show all 13)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
13.39 FOXA2 GCK HNF1A HNF1B HNF4A INS
2 12.41 FOXA2 GCKR HNF1A HNF4A INS NEUROD1
3
Show member pathways
12.31 ABCC8 GCK INS KCNJ11 PDX1 SLC2A2
4
Show member pathways
12.22 ABCC8 INS KCNJ11 PKLR SLC2A2
5 12.1 FOXA2 HNF1B INS NEUROD1 NEUROG3 NKX2-2
6
Show member pathways
12 FOXA2 GCK HNF1A HNF1B HNF4A INS
7
Show member pathways
11.97 FOXA2 GCK HNF1A HNF1B HNF4A INS
8
Show member pathways
11.69 GCK GCKR SLC2A2
9
Show member pathways
11.68 ABCC8 GCK HNF1A HNF1B HNF4A INS
10 11.13 FOXA2 NEUROD1 NKX2-2
11 10.97 ABCC8 FOXA2 GCK HNF1A HNF4A INS
12 10.73 INS SLC2A2
13 10.4 HNF1A HNF4A

GO Terms for Maturity-Onset Diabetes of the Young

Cellular components related to Maturity-Onset Diabetes of the Young according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 nucleoplasm GO:0005654 9.65 FOXA2 GCK GCKR HNF1B HNF4A KLF11
2 ATP-sensitive potassium channel complex GO:0008282 8.62 ABCC8 KCNJ11

Biological processes related to Maturity-Onset Diabetes of the Young according to GeneCards Suite gene sharing:

(show all 34)
id Name GO ID Score Top Affiliating Genes
1 carbohydrate metabolic process GO:0005975 9.93 GCK GCKR INS PKLR SLC2A2
2 response to drug GO:0042493 9.88 ABCC8 HNF1B KCNJ11 NEUROD1 PAX4 PDX1
3 positive regulation of sequence-specific DNA binding transcription factor activity GO:0051091 9.83 NEUROD1 NEUROG3 NKX2-2
4 positive regulation of neuron differentiation GO:0045666 9.81 NEUROD1 NEUROG3 NKX2-2
5 liver development GO:0001889 9.8 HNF1A HNF1B PDX1
6 positive regulation of insulin secretion GO:0032024 9.75 BLK GCK PDX1
7 glucose metabolic process GO:0006006 9.73 GCK INS KCNJ11 PDX1
8 glucose homeostasis GO:0042593 9.73 GCK HNF1A HNF4A INS NEUROD1 PDX1
9 positive regulation of cell differentiation GO:0045597 9.72 INS NEUROD1 NEUROG3 NKX2-2 PAX4
10 response to glucose GO:0009749 9.7 HNF1A HNF1B HNF4A NEUROD1 NKX2-2 PDX1
11 insulin secretion GO:0030073 9.67 HNF1A HNF1B NEUROD1 PDX1
12 signal transduction involved in regulation of gene expression GO:0023019 9.66 HNF4A NEUROD1
13 response to ATP GO:0033198 9.66 KCNJ11 PKLR
14 nitric oxide mediated signal transduction GO:0007263 9.65 NEUROD1 PDX1
15 hindbrain development GO:0030902 9.65 HNF1B NEUROD1 NEUROG3
16 positive regulation of glycogen biosynthetic process GO:0045725 9.64 GCK INS
17 negative regulation of gluconeogenesis GO:0045721 9.64 GCK INS
18 cellular glucose homeostasis GO:0001678 9.63 ABCC8 GCK GCKR
19 positive regulation of transcription initiation from RNA polymerase II promoter GO:0060261 9.62 HNF1A HNF1B
20 transdifferentiation GO:0060290 9.62 NEUROG3 PDX1
21 negative regulation of type B pancreatic cell apoptotic process GO:2000675 9.61 NEUROD1 PDX1
22 negative regulation of glucokinase activity GO:0033132 9.6 FOXA2 GCKR
23 detection of glucose GO:0051594 9.57 GCK PDX1
24 pancreatic PP cell fate commitment GO:0003329 9.52 NEUROD1 NKX2-2
25 pancreatic A cell fate commitment GO:0003326 9.51 NEUROD1 NKX2-2
26 regulation of insulin secretion GO:0050796 9.5 ABCC8 GCK HNF1A HNF4A KCNJ11 NEUROD1
27 regulation of pronephros size GO:0035565 9.49 HNF1A HNF1B
28 endocrine pancreas development GO:0031018 9.17 FOXA2 HNF1A NEUROD1 NEUROG3 NKX2-2 PAX4
29 transcription, DNA-templated GO:0006351 10.3 FOXA2 HNF1A HNF1B HNF4A KLF11 NEUROD1
30 regulation of transcription, DNA-templated GO:0006355 10.3 FOXA2 HNF1A HNF1B HNF4A INS KLF11
31 positive regulation of transcription from RNA polymerase II promoter GO:0045944 10.15 FOXA2 HNF1A HNF1B HNF4A NEUROD1 NEUROG3
32 cell differentiation GO:0030154 10.12 BLK NEUROD1 NEUROG3 NKX2-2 PAX4 PDX1
33 positive regulation of transcription, DNA-templated GO:0045893 10.07 FOXA2 HNF1A HNF1B HNF4A NEUROD1 PDX1
34 transcription from RNA polymerase II promoter GO:0006366 10.06 HNF1A HNF4A KLF11 NEUROD1 NEUROG3 PDX1

Molecular functions related to Maturity-Onset Diabetes of the Young according to GeneCards Suite gene sharing:

(show all 12)
id Name GO ID Score Top Affiliating Genes
1 RNA polymerase II core promoter proximal region sequence-specific DNA binding GO:0000978 9.85 FOXA2 HNF1A NEUROD1 NEUROG3 PDX1
2 transcriptional activator activity, RNA polymerase II core promoter proximal region sequence-specific binding GO:0001077 9.81 HNF1A HNF4A NEUROD1 NEUROG3
3 transcription factor binding GO:0008134 9.77 FOXA2 HNF1A NEUROD1 NKX2-2 PDX1
4 transcription factor activity, RNA polymerase II distal enhancer sequence-specific binding GO:0003705 9.65 HNF1B HNF4A PDX1
5 transcription factor activity, sequence-specific DNA binding GO:0003700 9.61 FOXA2 HNF1A HNF1B HNF4A KLF11 NEUROD1
6 double-stranded DNA binding GO:0003690 9.56 HNF1A NEUROD1 NEUROG3 PAX4
7 transcription regulatory region DNA binding GO:0044212 9.55 FOXA2 HNF1A HNF1B HNF4A KLF11
8 potassium ion binding GO:0030955 9.49 KCNJ11 PKLR
9 ATP-activated inward rectifier potassium channel activity GO:0015272 9.43 ABCC8 KCNJ11
10 core promoter proximal region DNA binding GO:0001159 9.4 HNF1B NKX2-2
11 sequence-specific DNA binding GO:0043565 9.23 FOXA2 HNF1A HNF1B HNF4A NEUROD1 NKX2-2
12 DNA binding GO:0003677 10.02 FOXA2 HNF1A HNF1B HNF4A KLF11 NEUROD1

Sources for Maturity-Onset Diabetes of the Young

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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