MODY
MCID: MTR004
MIFTS: 58

Maturity-Onset Diabetes of the Young (MODY) malady

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

Aliases & Classifications for Maturity-Onset Diabetes of the Young

Aliases & Descriptions for Maturity-Onset Diabetes of the Young:

Name: Maturity-Onset Diabetes of the Young 12 50 24 56 29 29 14
Mody 12 50 56
Maturity Onset Diabetes Mellitus in Young 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
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, type 13 and maturity-onset diabetes of the young 6. 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 Cilostazol have been mentioned in the context of this disorder. Affiliated tissues include testes, kidney and liver, 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 : 71 \"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 61)
id Related Disease Score Top Affiliating Genes
1 maturity-onset diabetes of the young, type 13 34.2 ABCC8 GCK HNF4A INS KCNJ11
2 maturity-onset diabetes of the young 6 33.9 ABCC8 GCK GCKR HNF1A HNF1B HNF4A
3 maturity-onset diabetes of the young, type viii 12.7
4 maturity-onset diabetes of the young, type vii 12.7
5 maturity-onset diabetes of the young, type ix 12.7
6 maturity-onset diabetes of the young, type 11 12.7
7 maturity-onset diabetes of the young, type 10 12.7
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 iii 12.1
13 mody, type iv 12.1
14 mody, type ii 12.1
15 fanconi renotubular syndrome 1 11.0
16 diabetes mellitus, transient neonatal, 1 11.0
17 hemochromatosis, type 4 10.3 HNF1A NEUROD1
18 biliary cirrhosis, primary, 5 10.3 HNF1B HNF4A
19 antidepressant type abuse 10.3 GCK HNF1A INS KCNJ11
20 pleuropulmonary blastoma 10.3 HNF1A INS
21 pancreatitis 10.3
22 progressive external ophthalmoplegia with mitochondrial dna deletions, autosomal dominant 6 10.2 NEUROD1 NEUROG3
23 hypoglycemia 10.2
24 nosophobia 10.2 ABCC8 GCK KCNJ11
25 diaphanospondylodysostosis 10.2 ABCC8 GCK
26 hepatitis 10.2
27 labyrinthine unilateral reactive loss 10.2 ABCC8 GCK INS KCNJ11
28 alopecia-mental retardation syndrome 2 10.2 ABCC8 INS SLC2A2
29 paroxysmal nonkinesigenic dyskinesia 2 10.2 INS NEUROD1
30 autoimmune disease 1 10.2 ABCC8 INS KCNJ11
31 deafness, autosomal recessive 66 10.2 ABCC8 INS KCNJ11
32 neurogenic arthropathy 10.2 ABCC8 GCK INS KCNJ11
33 non-syndromic intellectual disability 10.1 ABCC8 GCK INS KCNJ11 PDX1
34 diabetic cataract 10.1 ABCC8 GCK HNF4A INS KCNJ11
35 chondroma 10.1 ABCC8 INS KCNJ11
36 exostosis 10.1 ABCC8 GCK HNF4A INS KCNJ11
37 ischemic heart disease 10.1 ABCC8 GCK INS PDX1 SLC2A2
38 diabetes mellitus, insulin-dependent, 20 10.1 GCK HNF1A HNF1B HNF4A INS KCNJ11
39 peroneal neuropathy 10.1 ABCC8 GCK INS KCNJ11 SLC2A2
40 noonan syndrome 3 10.1 ABCC8 KCNJ11
41 body dysmorphic disorder 10.1 ABCC8 GCK INS KCNJ11 SLC2A2
42 focal segmental glomerulosclerosis 8 10.1 ABCC8 GCK INS KCNJ11 NEUROG3 PDX1
43 brain stem glioma 10.1 ABCC8 GCK HNF1A INS KCNJ11 PDX1
44 distal muscular dystrophy 10.0 ABCC8 GCK HNF1B INS KCNJ11 NEUROD1
45 duodenitis 10.0
46 hyperinsulinemic hypoglycemia 10.0
47 mosaic trisomy 13 10.0 ABCC8 CEL GCK HNF1A HNF1B HNF4A
48 obesity 9.9
49 hyperglycemia 9.9
50 kidney disease 9.8

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 GCKR PKLR GCK
2 Decreased viability GR00221-A-2 9.86 PKLR GCK
3 Decreased viability GR00221-A-3 9.86 APPL1 GCK
4 Decreased viability GR00221-A-4 9.86 GCKR PKLR APPL1 BLK GCK
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 PAX4 PDX1 PKLR SLC2A2 ABCC8 APPL1
2 growth/size/body region MP:0005378 10.31 NEUROG3 NKX2-2 PAX4 PDX1 SLC2A2 APPL1
3 endocrine/exocrine gland MP:0005379 10.3 ABCC8 BLK FOXA2 GCK HNF1A HNF1B
4 mortality/aging MP:0010768 10.2 SLC2A2 APPL1 FOXA2 GCK HNF1A HNF1B
5 adipose tissue MP:0005375 10.04 APPL1 CEL FOXA2 HNF1A INS KCNJ11
6 digestive/alimentary MP:0005381 10.02 CEL FOXA2 HNF1A INS NEUROD1 NEUROG3
7 liver/biliary system MP:0005370 10.02 CEL FOXA2 GCK HNF1A HNF1B HNF4A
8 muscle MP:0005369 9.8 APPL1 HNF1A HNF1B INS KCNJ11 PDX1
9 no phenotypic analysis MP:0003012 9.7 INS KCNJ11 NEUROG3 PDX1 ABCC8 APPL1
10 normal MP:0002873 9.65 BLK FOXA2 HNF4A INS KLF11 NEUROG3
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 237)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1 Moxonidine Approved Phase 4 75438-57-2 4810
2
Cilostazol Approved Phase 4 73963-72-1 2754
3
Metformin Approved Phase 4,Phase 3,Phase 2 657-24-9 14219 4091
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 3,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,Phase 2 141758-74-9 15991534
11
Ethanol Approved Phase 4 64-17-5 702
12
Menthol Approved Phase 4,Phase 3 2216-51-5 16666
13
Mirabegron Approved Phase 4 223673-61-8
14
Ledipasvir Approved Phase 4 1256388-51-8 67505836
15
Ribavirin Approved Phase 4 36791-04-5 37542
16
Ritonavir Approved, Investigational Phase 4 155213-67-5 392622
17
Sofosbuvir Approved Phase 4 1190307-88-0 45375808
18
Aspirin Approved, Vet_approved Phase 4 50-78-2 2244
19 Hormones Phase 4,Phase 2,Phase 3,Phase 1
20 insulin Phase 4,Phase 3,Phase 2,Phase 1
21 Insulin, Globin Zinc Phase 4,Phase 3,Phase 2,Phase 1
22 Antihypertensive Agents Phase 4,Phase 3
23 Anti-Asthmatic Agents Phase 4,Phase 2,Phase 1
24 Autonomic Agents Phase 4,Phase 2
25 Bronchodilator Agents Phase 4,Phase 2
26 Fibrinolytic Agents Phase 4
27 Neuroprotective Agents Phase 4
28 Peripheral Nervous System Agents Phase 4,Phase 2,Phase 1
29 Phosphodiesterase 3 Inhibitors Phase 4
30 Phosphodiesterase Inhibitors Phase 4,Phase 2
31 Platelet Aggregation Inhibitors Phase 4
32 Protective Agents Phase 4,Phase 1
33 Respiratory System Agents Phase 4,Phase 2,Phase 1
34 Vasodilator Agents Phase 4,Phase 2
35 Hypoglycemic Agents Phase 4,Phase 2,Phase 3,Phase 1
36 Sitagliptin Phosphate Phase 4,Phase 3
37 Hormone Antagonists Phase 4,Phase 2,Phase 3,Phase 1
38 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2,Phase 3,Phase 1
39 Incretins Phase 4,Phase 2,Phase 3,Phase 1
40 Liver Extracts Phase 4,Phase 3
41 Dipeptidyl-Peptidase IV Inhibitors Phase 4,Phase 3
42 Anticholesteremic Agents Phase 4
43 Antimetabolites Phase 4,Phase 2
44 Atorvastatin Calcium Phase 4 134523-03-8
45 Calcium, Dietary Phase 4,Phase 3
46 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 4
47 Hypolipidemic Agents Phase 4,Phase 2
48 Lipid Regulating Agents Phase 4,Phase 2
49 Anti-Arrhythmia Agents Phase 4,Phase 2,Phase 3
50 Immunosuppressive Agents Phase 4,Phase 2,Phase 3,Phase 1

Interventional clinical trials:

(show top 50) (show all 167)
id Name Status NCT ID Phase
1 Thyroid Study Type 2 Diabetes Mellitus (T2DM) Unknown status NCT01379170 Phase 4
2 Study of the Effect of Moxonidine and Diet on Sympathetic Functions in Young Adults With Obesity Unknown status NCT01180231 Phase 4
3 Effects of Cilostazol on Plasma Adipocytokine and Arterial Stiffness Unknown status NCT00573950 Phase 4
4 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
5 Efficacy and Safety Study of Pitavastatin Compared to atoRvastatin in Type 2 dIabeTes Mellitus With Hypercholesterolemia Completed NCT00889226 Phase 4
6 Management of Type-2 Diabetic Patients Treated With Insulin During the Ramadan Completed NCT01354925 Phase 4
7 Optimal Oral Hypoglycaemic Agents (OHA) for Combination With Insulin Glargine (Sulfonylurea vs. Metformin) Completed NCT00708578 Phase 4
8 Insulin Glargine (Lantus) vs Sulfonylurea (SU) for BETA Cell Function (BETA Study) Completed NCT00562172 Phase 4
9 Exenatide and Brown Adipose Tissue Recruiting NCT03002675 Phase 4
10 Mirabegron and Brown Adipose Tissue Recruiting NCT03012113 Phase 4
11 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
12 Effects of Dapagliflozin on Epicardial Fat in Subjects With Type 2 Diabetes Recruiting NCT02235298 Phase 4
13 Metformin for Ectopic Fat Deposition and Metabolic Markers in Polycystic Ovary Syndrome (PCOS) Recruiting NCT02500147 Phase 4
14 Exploratory Study to Compare the Effects of Tenelia® or Januvia® on Glucose Variability in add-on to Metformin (CGMS Study) Recruiting NCT02512523 Phase 4
15 Diabetes Interventional Assessment of Slimming or Training to Lessen Inconspicuous Cardiovascular Dysfunction Recruiting NCT02590822 Phase 4
16 Study of Oral Treatments for Hepatitis C Recruiting NCT02786537 Phase 4
17 Metformin in Longevity Study Active, not recruiting NCT02432287 Phase 4
18 Autoimmune Diabetes Accelerator Prevention Trial Enrolling by invitation NCT02881528 Phase 4
19 Effect of aSpirin Versus CilOstazol for Inhibition of Antiplatelet aggRegaTion in Type 2 DM Patients Not yet recruiting NCT02933788 Phase 4
20 The Effects of Exenatide on Post-Meal Sugar Peaks and Vascular Health in Obese/Pre-Diabetic Young Adults Withdrawn NCT00845559 Phase 4
21 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
22 The Effects of GLP-1 in Maturity-Onset Diabetes of The Young (MODY) Completed NCT01610934 Phase 2, Phase 3
23 Safety and Efficacy of OSTEOFORM (rhPTH [1-34]) in Increasing Bone Mineral Density in Osteoporosis Completed NCT00500409 Phase 3
24 Extension Study for 2993-112 Completed NCT01789957 Phase 3
25 Evaluation of the Effect on Glucose Control of AC2993 in Patients With Type 2 Diabetes Mellitus Treated With a Sulfonylurea Completed NCT00039026 Phase 3
26 Type 2 Diabetes Primary Prevention for At Risk Girls Completed NCT00063674 Phase 2, Phase 3
27 Phase III Study for Glimepiride + Metformin Hydrochloride (Amaryl M) Slow Release (SR) Completed NCT00437554 Phase 3
28 Glibentek in Patients With Neonatal Diabetes Secondary to Mutations in K+-ATP Channels Completed NCT02375828 Phase 3
29 Effects of Liraglutide in Young Adults With Type 2 DIAbetes (LYDIA) Recruiting NCT02043054 Phase 3
30 Safety and Efficacy of Bimatoprost Sustained-Release (SR) in Patients With Open-Angle Glaucoma or Ocular Hypertension Recruiting NCT02250651 Phase 3
31 An Open-Label, Long-Term Study of Oral Treprostinil in Subjects With Pulmonary Arterial Hypertension Recruiting NCT01560637 Phase 3
32 Trial of the Early Combination of Oral Treprostinil With Background Oral Monotherapy in Subjects With Pulmonary Arterial Hypertension Recruiting NCT01560624 Phase 3
33 Randomized Global Phase 3 Study to Evaluate the Impact on NASH With Fibrosis of Obeticholic Acid Treatment Recruiting NCT02548351 Phase 3
34 Treating Insulin Resistance as a Strategy to Improve Outcome in Refractory Bipolar Disorder Recruiting NCT02519543 Phase 3
35 Effect Of Mesenchymal Stem Cells Transfusion on the Diabetic Peripheral Neuropathy Patients . Active, not recruiting NCT02387749 Phase 2, Phase 3
36 Effect of a Continuous Glucose Monitoring on Maternal and Neonatal Outcomes in Gestational Diabetes Mellitus Not yet recruiting NCT02838147 Phase 3
37 Efficacy of Foot Orthotics in Veterans With Chronic Lower Back Pain Terminated NCT01865539 Phase 2, Phase 3
38 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
39 The Effects of California Strawberries on Parameters of Cardiovascular Health Unknown status NCT01705093 Phase 2
40 Clinical Trial of YH14618 in Patients With Degenerative Disc Disease Completed NCT02320019 Phase 2
41 Open-label Study of STA-9090 for Patients With Metastatic Breast Cancer Completed NCT01273896 Phase 2
42 RTA 408 Ophthalmic Suspension for the Prevention of Corneal Endothelial Cell Loss Following Cataract Surgery - GUARD Completed NCT02128113 Phase 2
43 A Clinical Trial to Evaluate the Safety and Efficacy of ZYH7 Compared to Fenofibrate in Patients With Dyslipidemia Completed NCT01539616 Phase 2
44 Partners for Better Health in Adolescent Type 2 Diabetes: The Buddy Study Completed NCT01007266 Phase 2
45 Stem Cell Educator Therapy in Type 2 Diabetes Completed NCT01415726 Phase 1, Phase 2
46 Translating the DPP Into the Community Completed NCT00302042 Phase 2
47 Mobile Device Outcomes-based Rehabilitation Program Recruiting NCT02891707 Phase 1, Phase 2
48 Bone Marrow Transplantation vs Standard of Care in Patients With Severe Sickle Cell Disease (BMT CTN 1503) Recruiting NCT02766465 Phase 2
49 Double-blinded, 6 Months Study With Bydureon® or Placebo in Adolescents With Obesity to Explore Changes in BMI Active, not recruiting NCT02794402 Phase 2
50 Theophylline Treatment for Pseudohypoparathyroidism Not yet recruiting NCT03029429 Phase 2

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
Testes, Kidney, Liver, Pancreas, Thyroid, Uterus

Publications for Maturity-Onset Diabetes of the Young

Articles related to Maturity-Onset Diabetes of the Young:

(show top 50) (show all 308)
id Title Authors Year
1
An analysis of the sequence of the BAD gene among patients with maturity-onset diabetes of the young (MODY). ( 27935851 )
2017
2
Maturity-onset diabetes of the young (MODY) in Brazil: Establishment of a national registry and appraisal of available genetic and clinical data. ( 28012402 )
2017
3
Maturity onset diabetes of the young 5 accompanied by duodenal cysts. ( 26676964 )
2016
4
A review of maturity onset diabetes of the young (MODY) and challenges in the management of glucokinase-MODY. ( 27852188 )
2016
5
ABCC8-Related Maturity-Onset Diabetes of the Young (MODY12): Clinical Features and Treatment Perspective. ( 27538677 )
2016
6
Studies of genetic variability of the hepatocyte nuclear factor-1I+ gene in an Indian maturity-onset diabetes of the young family. ( 27148439 )
2016
7
A Single Nucleotide Variant in HNF-1I^ is Associated with Maturity-Onset Diabetes of the Young in a Large Chinese Family. ( 27114981 )
2016
8
Maturity-Onset Diabetes of the Young (MODY): Making the Right Diagnosis to Optimize Treatment. ( 27130141 )
2016
9
Genetic Confirmation Rate in Clinically Suspected Maturity-Onset Diabetes of the Young. ( 27634015 )
2016
10
Maturity onset diabetes of the young: Seek and you will find. ( 27323672 )
2016
11
GCK gene mutations are a common cause of childhood-onset MODY (maturity-onset diabetes of the young) in Turkey. ( 27256595 )
2016
12
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
13
Searching for Maturity-Onset Diabetes of the Young (MODY): When and What for? ( 27103109 )
2016
14
A three-step programmed method for the identification of causative gene mutations of maturity onset diabetes of the young (MODY). ( 27185633 )
2016
15
Maturity-Onset Diabetes of the Young: A Genetic Form of Diabetes in Children. ( 27955956 )
2016
16
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
17
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
18
Maturity-Onset Diabetes of the Young: What Do Clinicians Need to Know? ( 26706916 )
2015
19
Molecular diagnosis of maturity-onset diabetes of the young (MODY) in Turkish children by using targeted next-generation sequencing. ( 26226118 )
2015
20
Hepatocyte nuclear factor-I+ genetic mutation in a Chinese pedigree with maturity-onset diabetes of the young (MODY3). ( 26436572 )
2015
21
Molecular and phenotypic characteristics of maturity-onset diabetes of the young compared with early onset type 2 diabetes in China. ( 25588466 )
2015
22
The clinical management of hyperglycemia in pregnancy complicated by maturity-onset diabetes of the young. ( 25935773 )
2015
23
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
24
A genetic diagnosis of maturity-onset diabetes of the young (MODY): experiences of patients and family members. ( 25763774 )
2015
25
Characteristics of maturity onset diabetes of the young in a large diabetes center. ( 26059258 )
2015
26
Maturity-onset diabetes of the young (MODY): an update. ( 25581748 )
2015
27
Frameshift mutations in the insulin gene leading to prolonged molecule of insulin in two families with Maturity-Onset Diabetes of the Young. ( 25721872 )
2015
28
Establishment of maturity-onset diabetes of the young-induced pluripotent stem cells from a Japanese patient. ( 26417411 )
2015
29
Postprandial incretin and islet hormone responses and dipeptidyl-peptidase 4 enzymatic activity in patients with maturity onset diabetes of the young. ( 25953829 )
2015
30
A novel nonsense mutation of the HNF1I+ in maturity-onset diabetes of the young type 3 in Asian population. ( 26050565 )
2015
31
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
32
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
33
Aberrant mRNA splicing of paired box 4 (PAX4) IVS7-1G>A mutation causing maturity-onset diabetes of the young, type 9. ( 25951767 )
2015
34
Incretin hormones and maturity onset diabetes of the young--pathophysiological implications and anti-diabetic treatment potential. ( 26324089 )
2015
35
"MODY2" (maturity onset diabetes of the young type 2). ( 25622392 )
2014
36
Maturity onset diabetes of the young in India - a distinctive mutation pattern identified through targeted next-generation sequencing. ( 25041077 )
2014
37
Three years of liraglutide treatment offers continuously optimal glycemic control in a pediatric patient with maturity-onset diabetes of the young type 3. ( 25332292 )
2014
38
Maturity onset diabetes of the young (MODY)-History, first case reports and recent advances. ( 25281821 )
2014
39
Identification and Functional Characterization of P159L Mutation in HNF1B in a Family with Maturity-Onset Diabetes of the Young 5 (MODY5). ( 25705165 )
2014
40
Carboxyl-ester lipase maturity-onset diabetes of the young is associated with development of pancreatic cysts and upregulated MAPK signaling in secretin-stimulated duodenal fluid. ( 24062244 )
2014
41
Genetic causes of maturity onset diabetes of the young may be less prevalent in American pregnant women recently diagnosed with diabetes mellitus than in previously studied European populations. ( 25012807 )
2014
42
Current and Best Practices of Genetic Testing for Maturity Onset Diabetes of the Young: Views of Professional Experts. ( 25341961 )
2014
43
Clinical utility gene card for: Maturity-onset diabetes of the young. ( 24518839 )
2014
44
A family with a novel termination mutation in hepatic nuclear factor 1I+ in maturity-onset diabetes of the young type 3 which is unresponsive to sulphonylurea therapy. ( 24642958 )
2014
45
Molecular and clinical characterization of glucokinase maturity-onset diabetes of the young (GCK-MODY) in Japanese patients. ( 24804978 )
2014
46
Structural and functional study of the GlnB22-insulin mutant responsible for maturity-onset diabetes of the young. ( 25423173 )
2014
47
Comparability of high-sensitivity CRP methods to detect maturity-onset diabetes of the young due to HNF1A mutations. ( 24974685 )
2014
48
Carboxyl-Ester Lipase Maturity-Onset Diabetes of the Young Disease Protein Biomarkers in Secretin-Stimulated Duodenal Juice. ( 25369532 )
2014
49
Low serum level of high-sensitivity C-reactive protein in a Japanese patient with maturity-onset diabetes of the young type 3 (MODY3). ( 25411618 )
2014
50
Maturity-onset diabetes of the young type 5 in a family with diabetes and mild kidney disease diagnosed by whole exome sequencing. ( 25367728 )
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 35)
id Name GO ID Score Top Affiliating Genes
1 carbohydrate metabolic process GO:0005975 9.95 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 9.81 HNF1A HNF1B PDX1
6 positive regulation of insulin secretion GO:0032024 9.77 BLK GCK PDX1
7 glucose transport GO:0015758 9.75 GCK INS SLC2A2
8 glucose metabolic process GO:0006006 9.73 GCK INS KCNJ11 PDX1
9 glucose homeostasis GO:0042593 9.73 GCK HNF1A HNF4A INS NEUROD1 PDX1
10 positive regulation of cell differentiation GO:0045597 9.72 INS NEUROD1 NEUROG3 NKX2-2 PAX4
11 response to glucose GO:0009749 9.7 HNF1A HNF1B HNF4A NEUROD1 NKX2-2 PDX1
12 signal transduction involved in regulation of gene expression GO:0023019 9.67 HNF4A NEUROD1
13 insulin secretion GO:0030073 9.67 HNF1A HNF1B NEUROD1 PDX1
14 response to ATP GO:0033198 9.66 KCNJ11 PKLR
15 nitric oxide mediated signal transduction GO:0007263 9.66 NEUROD1 PDX1
16 positive regulation of glycogen biosynthetic process GO:0045725 9.65 GCK INS
17 negative regulation of gluconeogenesis GO:0045721 9.65 GCK INS
18 hindbrain development GO:0030902 9.65 HNF1B NEUROD1 NEUROG3
19 positive regulation of transcription initiation from RNA polymerase II promoter GO:0060261 9.63 HNF1A HNF1B
20 cellular glucose homeostasis GO:0001678 9.63 ABCC8 GCK GCKR
21 transdifferentiation GO:0060290 9.62 NEUROG3 PDX1
22 negative regulation of type B pancreatic cell apoptotic process GO:2000675 9.62 NEUROD1 PDX1
23 negative regulation of glucokinase activity GO:0033132 9.61 FOXA2 GCKR
24 detection of glucose GO:0051594 9.57 GCK PDX1
25 pancreatic A cell fate commitment GO:0003326 9.54 NEUROD1 NKX2-2
26 pancreatic PP cell fate commitment GO:0003329 9.52 NEUROD1 NKX2-2
27 regulation of pronephros size GO:0035565 9.51 HNF1A HNF1B
28 regulation of insulin secretion GO:0050796 9.5 ABCC8 GCK HNF1A HNF4A KCNJ11 NEUROD1
29 endocrine pancreas development GO:0031018 9.17 FOXA2 HNF1A NEUROD1 NEUROG3 NKX2-2 PAX4
30 transcription, DNA-templated GO:0006351 10.31 FOXA2 HNF1A HNF1B HNF4A KLF11 NEUROD1
31 regulation of transcription, DNA-templated GO:0006355 10.3 FOXA2 HNF1A HNF1B HNF4A INS KLF11
32 cell differentiation GO:0030154 10.12 BLK NEUROD1 NEUROG3 NKX2-2 PAX4 PDX1
33 positive regulation of transcription from RNA polymerase II promoter GO:0045944 10.1 FOXA2 HNF1A HNF1B HNF4A NEUROD1 NEUROG3
34 positive regulation of transcription, DNA-templated GO:0045893 10.08 FOXA2 HNF1A HNF1B HNF4A NEUROD1 PDX1
35 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 9.83 FOXA2 HNF1A NEUROD1 NEUROG3 PDX1
2 transcriptional activator activity, RNA polymerase II core promoter proximal region sequence-specific binding GO:0001077 9.8 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 9.51 KCNJ11 PKLR
9 ATP-activated inward rectifier potassium channel activity GO:0015272 9.46 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
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44 MGI
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48 NDF-RT
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59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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