MODY1
MCID: MDY005
MIFTS: 30

Mody, Type I (MODY1) malady

Categories: Genetic diseases, Rare diseases

Aliases & Classifications for Mody, Type I

Aliases & Descriptions for Mody, Type I:

Name: Mody, Type I 54 13 38
Mody Type 1 12 50 66
Mody1 12 50 66
Maturity-Onset Diabetes of the Young, Type 1 50 69
Maturity-Onset Diabetes of the Young Type 1 12 24
Maturity-Onset Diabetes of the Young 1 66 29
Mild Juvenile Diabetes Mellitus 12 66
Hnf4a-Related Maturity-Onset Diabetes of the Young Type 1 24
Hnf4a-Related Maturity-Onset Diabetes of the Young Type I 24
Hnf4a-Related Diabetes Mellitus, Mody Type 1 24
Type 1 Maturity-Onset Diabetes of the Young 50
Diabetes Mellitus Mody Type 1 50
Hnf4a-Related Mody1 24
Mody Hnf4a Related 50
Mody-1 66

Characteristics:

HPO:

32
mody, type i:
Inheritance autosomal dominant inheritance


Classifications:



External Ids:

OMIM 54 125850
Disease Ontology 12 DOID:0111099
MedGen 40 C1852093
MeSH 42 D003924

Summaries for Mody, Type I

UniProtKB/Swiss-Prot : 66 Maturity-onset diabetes of the young 1: A form of diabetes that is characterized by an autosomal dominant mode of inheritance, onset in childhood or early adulthood (usually before 25 years of age), a primary defect in insulin secretion and frequent insulin-independence at the beginning of the disease.

MalaCards based summary : Mody, Type I, also known as mody type 1, is related to maturity-onset diabetes of the young, and has symptoms including maturity-onset diabetes of the young An important gene associated with Mody, Type I is HNF4A (Hepatocyte Nuclear Factor 4 Alpha). The drugs Ethanol and Clonidine have been mentioned in the context of this disorder.

Disease Ontology : 12 A maturity-onset diabetes of the young that has material basis in mutation in the HNF4A gene on chromosome 20.

Description from OMIM: 125850

Related Diseases for Mody, Type I

Diseases related to Mody, Type I via text searches within MalaCards or GeneCards Suite gene sharing:

id Related Disease Score Top Affiliating Genes
1 maturity-onset diabetes of the young 10.1

Symptoms & Phenotypes for Mody, Type I

Symptoms by clinical synopsis from OMIM:

125850

Clinical features from OMIM:

125850

Human phenotypes related to Mody, Type I:

32
id Description HPO Frequency HPO Source Accession
1 maturity-onset diabetes of the young 32 HP:0004904

Drugs & Therapeutics for Mody, Type I

Drugs for Mody, Type I (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 559)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ethanol Approved Phase 4 64-17-5 702
2
Clonidine Approved Phase 4 4205-90-7 2803
3
Glyburide Approved Phase 4,Phase 3,Phase 2,Phase 1 10238-21-8 3488
4
Rosiglitazone Approved, Investigational Phase 4,Phase 3,Phase 2 122320-73-4 77999
5
Simvastatin Approved Phase 4,Phase 1 79902-63-9 54454
6
Zinc Approved Phase 4,Phase 3,Phase 2,Phase 1 7440-66-6 32051 23994
7
Aspirin Approved, Vet_approved Phase 4,Phase 3 50-78-2 2244
8
Fluvastatin Approved Phase 4,Phase 3 93957-54-1 1548972
9
Furosemide Approved, Vet_approved Phase 4 54-31-9 3440
10
Metformin Approved Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1 657-24-9 14219 4091
11
Ramipril Approved Phase 4,Phase 3 87333-19-5 5362129
12
Losartan Approved Phase 4,Phase 3,Phase 2 114798-26-4 3961
13
Valsartan Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1 137862-53-4 60846
14
Hydrochlorothiazide Approved, Vet_approved Phase 4,Phase 3 58-93-5 3639
15
Candesartan Approved Phase 4,Phase 3,Phase 2 139481-59-7 2541
16
Amlodipine Approved Phase 4,Phase 3 88150-42-9 2162
17
Irbesartan Approved, Investigational Phase 4,Phase 3,Phase 1,Phase 2,Early Phase 1 138402-11-6 3749
18
Atenolol Approved Phase 4 29122-68-7 2249
19
Doxazosin Approved Phase 4 74191-85-8 3157
20
Acarbose Approved, Investigational Phase 4,Phase 3 56180-94-0 441184
21
Liraglutide Approved Phase 4,Phase 3,Phase 2,Phase 1 204656-20-2
22
Menthol Approved Phase 4,Phase 3 2216-51-5 16666
23
Empagliflozin Approved Phase 4,Phase 3,Phase 2,Phase 1 864070-44-0
24
Insulin Aspart Approved Phase 4,Phase 3,Phase 2,Phase 1 116094-23-6 16132418
25
Insulin Lispro Approved Phase 4,Phase 3,Phase 2,Phase 1 133107-64-9
26
Heparin Approved, Investigational Phase 4,Phase 3 9005-49-6 772 46507594
27
Norepinephrine Approved Phase 4 51-41-2 439260
28
Dopamine Approved Phase 4,Phase 2,Phase 3 51-61-6, 62-31-7 681
29
Ezetimibe Approved Phase 4 163222-33-1 150311
30
Pioglitazone Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1 111025-46-8 4829
31
Midazolam Approved, Illicit Phase 4 59467-70-8 4192
32
Repaglinide Approved, Investigational Phase 4 135062-02-1 65981
33
Gliclazide Approved Phase 4 21187-98-4 3475
34
Nateglinide Approved, Investigational Phase 4,Phase 3 105816-04-4 60026
35
Glimepiride Approved Phase 4,Phase 3,Phase 2,Phase 1 93479-97-1 3476
36
Nifedipine Approved Phase 4 21829-25-4 4485
37
Enalapril Approved, Vet_approved Phase 4,Phase 3,Phase 2 75847-73-3 5362032 40466924
38
Captopril Approved Phase 4 62571-86-2 44093
39
Lisinopril Approved, Investigational Phase 4,Phase 3 83915-83-7 5362119
40
Enalaprilat Approved Phase 4,Phase 3,Phase 2 76420-72-9 6917719
41
Benazepril Approved, Investigational Phase 4,Phase 3 86541-75-5 5362124
42
Telmisartan Approved, Investigational Phase 4,Phase 3 144701-48-4 65999
43
Indapamide Approved Phase 4 26807-65-8 3702
44
Metoprolol Approved, Investigational Phase 4 37350-58-6, 51384-51-1 4171
45
Olmesartan Approved, Investigational Phase 4,Phase 3 144689-24-7, 144689-63-4 158781 130881
46
Nitroglycerin Approved, Investigational Phase 4 55-63-0 4510
47
Ticlopidine Approved Phase 4 55142-85-3 5472
48 sodium fluoride Approved Phase 4 7681-49-4
49
Adenosine Approved, Investigational Phase 4 58-61-7 60961
50
Regadenoson Approved Phase 4 313348-27-5 219024

Interventional clinical trials:

(show top 50) (show all 1136)
id Name Status NCT ID Phase
1 Dapagliflozin in Type 1 Diabetes Unknown status NCT02211742 Phase 4
2 Liraglutide Actions on the Liver: Effects on Glucose Phosphorylation Unknown status NCT02198209 Phase 4
3 A Study on Ca Blocker Versus AII Antagonists in Hypertension With Type 2 Diabetes Unknown status NCT00144144 Phase 4
4 ACEIs and ARBs Treatment in Diabetic Patients -Drug Interactions and Adverse Drug Effects Unknown status NCT00437775 Phase 4
5 Non-Insulin Dependent Diabetes Mellitus (NIDDM) and Angiotensin Converting Enzyme 2 (ACE2): Diabetic Patients Treated With Antihypertensive Drugs Unknown status NCT00192803 Phase 4
6 Growth Hormone Feedback to Insulin-like Growth Factor-I (IGF-1) and Oral Glucose Tolerance Test (OGTT) Unknown status NCT00915954 Phase 4
7 Safety of Add on Aliskiren to Angiotensin Converting Enzyme Inhibitor (ACEI) and Angiotensin I Receptor Blocker (ARB) Treatment in Type 2 Diabetes With Nephropathy Unknown status NCT00949351 Phase 4
8 Vitamin D and Glucose Metabolism in Pediatrics Unknown status NCT01386736 Phase 4
9 Effect of Doxium on High Sensitivity CRP and Endothelin-1 Serum Levels in Patients With Diabetic Retinopathy Unknown status NCT01382498 Phase 4
10 Multifactorial Intervention in Type 2 Diabetes - Italy Unknown status NCT01240070 Phase 4
11 Effects of Valsartan and Aliskiren on Hemostatic Indices in Hypertensive Diabetics Unknown status NCT01095822 Phase 4
12 The INDORSE Study: Inhibition of Dipeptidyl Peptidase IV: Outcomes on Renal Sodium Excretion Unknown status NCT02406443 Phase 4
13 Intensive Medical Treatment for Nephropathy Caused by Type 2 Diabetes With Hypertension Unknown status NCT00407680 Phase 4
14 Nephropathy In Type 2 Diabetes and Cardio-renal Events Unknown status NCT00535925 Phase 4
15 Rationale and Design for Shiga Microalbuminuria Reduction Trial Unknown status NCT00202618 Phase 4
16 Regression of Fatty Heart by Valsartan Therapy Unknown status NCT00745953 Phase 4
17 German Diabetes-Diet-Intervention and Energy Restriction-Trial (DDIET) Unknown status NCT01409330 Phase 4
18 Observational Study of Patients Using NovoMix® 30 or Levemir® for Treatment of Type 1 or Type 2 Diabetes Completed NCT00665093 Phase 4
19 NovoLog Observation Trial in Subjects With Type 1 and Type 2 Diabetes Completed NCT00095446 Phase 4
20 Clinical Trial of Efficiency and Safety of Subetta in the Combined Treatment of Patients With Type II Diabetes Mellitus Completed NCT01868646 Phase 4
21 Efficacy and Safety of Insulin Aspart in Subjects With Type 1 or Type 2 Diabetes Completed NCT00593255 Phase 4
22 Influence of Diabetes on Tramadol Pharmacokinetics Completed NCT02246712 Phase 4
23 Changes in Bone Turnover With Exposure to a GLP-1 Receptor Agonist Completed NCT01381926 Phase 4
24 Assessment of SMS and Smartphone Interventions for OneTouch Reveal® Experiences Completed NCT02429024 Phase 4
25 Regadenoson Blood Flow in Type 1 Diabetes (RABIT1D) Completed NCT01019486 Phase 4
26 Effect of Metformin in Patients With Type-1 Diabetes With Inadequate Glycaemic Control by Insulin and Diet Completed NCT00118937 Phase 4
27 A Study for Patients With Diabetes Mellitus (IOPA) Completed NCT00420095 Phase 4
28 Liraglutide in Type 1 Diabetes Completed NCT01612468 Phase 4
29 The Effect of Metformin in Overweight Patients With Dysregulated Type 1 Diabetes Mellitus Completed NCT00145379 Phase 4
30 Sitagliptin Dose Determination Study Completed NCT01530178 Phase 4
31 Rosiglitazone and Insulin in T1DM Adolescents Completed NCT00372086 Phase 4
32 The Effect of Prostaglandin I2 on the Endothelial Cell Function Disorder in Type 2 Diabetes Mellitus Patients Completed NCT01061060 Phase 4
33 Effect of Vildagliptin on Left Ventricular Function in Patients With Type 2 Diabetes and Congestive Heart Failure Completed NCT00894868 Phase 4
34 Vildagliptin vs Sitagliptin add-on to Insulin - Impact on Glycemic Profile and Correlation of Hypoglycemic Episodes and Heart Function Completed NCT01686932 Phase 4
35 Chronic Reduction of Fasting Glycaemia With Insulin Glargine Improves First and Second Phase Insulin Secretion in Patients With Type 2 Diabetes Completed NCT01249677 Phase 4
36 SAFEGUARD: Pleiotropic Effects of Incretin Based Therapies Completed NCT01744236 Phase 4
37 Study In Postmenopausal Women With Type 2 Diabetes Looking At Approved Diabetes Drugs And How They Affect Bone Health Completed NCT00679939 Phase 4
38 Basal Insulin in the Management of Patients With Diabetic Ketoacidosis (DKA) Completed NCT00590044 Phase 4
39 A Study to Explore the Effects of Azilsartan Compared to Telmisartan on Insulin Resistance of Patients With Essential Hypertension on Type 2 Diabetes Mellitus by HOMA-R Completed NCT02079805 Phase 4
40 Renal Effects of DPP-4 Inhibitor Linagliptin in Type 2 Diabetes Completed NCT02106104 Phase 4
41 Anti-Inflammatory Actions of Valsartan in Patients With Type 2 Diabetes Mellitus Completed NCT00982358 Phase 4
42 To Assess the Effects of Valsartan on Albuminuria/Proteinuria in Hypertensive Patients With Type 2 Diabetes Mellitus Completed NCT00550095 Phase 4
43 Effect of Valsartan on Proteinuria in Patients With Hypertension and Diabetes Mellitus Completed NCT00241085 Phase 4
44 Aliskiren and Valsartan vs Valsartan Alone in Patients With Stage II Systolic Hypertension and Type II Diabetes Mellitus Completed NCT00927394 Phase 4
45 Lovaza® and Microvascular Function in Type 2 Diabetes Completed NCT00931879 Phase 4
46 Stem Cell Mobilization With Plerixafor in Diabetic vs Control Subjects Completed NCT02056210 Phase 4
47 DPP IV Inhibition Facilitates Healing of Chronic Foot Ulcers in Type 2 Diabetes Completed NCT01472432 Phase 4
48 Efficacy and Safety of DLBS1033 in Subjects With Type 2 Diabetes Mellitus Completed NCT01865474 Phase 4
49 A Clinical Study to Evaluate Renal Hemodynamic Responses to Aliskiren in Patients With Type 2 Diabetes Mellitus Completed NCT00660309 Phase 4
50 A Type 2 Diabetes Study of the Longer-Term Glycemic Effect of AVANDAMET vs. Metformin Completed NCT00386100 Phase 4

Search NIH Clinical Center for Mody, Type I

Genetic Tests for Mody, Type I

Genetic tests related to Mody, Type I:

id Genetic test Affiliating Genes
1 Maturity-Onset Diabetes of the Young, Type 1 29
2 Maturity-Onset Diabetes of the Young Type 1 24 HNF4A

Anatomical Context for Mody, Type I

Publications for Mody, Type I

Variations for Mody, Type I

UniProtKB/Swiss-Prot genetic disease variations for Mody, Type I:

66
id Symbol AA change Variation ID SNP ID
1 HNF4A p.Arg136Trp VAR_004668 rs137853336
2 HNF4A p.Glu285Gln VAR_010601
3 HNF4A p.Met373Arg VAR_071952 rs137853338

ClinVar genetic disease variations for Mody, Type I:

6 (show all 17)
id Gene Variation Type Significance SNP ID Assembly Location
1 HNF4A NM_000457.4(HNF4A): c.829C> T (p.Gln277Ter) single nucleotide variant Pathogenic rs137853334 GRCh37 Chromosome 20, 43048453: 43048453
2 HNF4A NM_000457.4(HNF4A): c.487C> T (p.Arg163Ter) single nucleotide variant Pathogenic rs137853335 GRCh37 Chromosome 20, 43042435: 43042435
3 HNF4A NM_000457.4(HNF4A): c.406C> T (p.Arg136Trp) single nucleotide variant Pathogenic rs137853336 GRCh37 Chromosome 20, 43042354: 43042354
4 HNF4A HNF4A, 1-BP DEL, PHE75T deletion Pathogenic
5 HNF4A HNF4A, IVS5, DEL A, -2 deletion Pathogenic
6 HNF4A NM_000457.4(HNF4A): c.1118T> G (p.Met373Arg) single nucleotide variant Pathogenic rs137853338 GRCh37 Chromosome 20, 43052883: 43052883
7 HNF4A NM_000457.4(HNF4A): c.1163C> G (p.Pro388Arg) single nucleotide variant Likely pathogenic rs193922469 GRCh37 Chromosome 20, 43057008: 43057008
8 HNF4A NM_000457.4(HNF4A): c.1253G> C (p.Cys418Ser) single nucleotide variant Likely pathogenic rs193922470 GRCh37 Chromosome 20, 43057098: 43057098
9 HNF4A NM_000457.4(HNF4A): c.347_348delGGinsC (p.Arg116Thrfs) indel Likely pathogenic rs193922471 GRCh37 Chromosome 20, 43036077: 43036078
10 HNF4A NM_000457.4(HNF4A): c.619G> C (p.Ala207Pro) single nucleotide variant Likely pathogenic rs193922474 GRCh37 Chromosome 20, 43043273: 43043273
11 HNF4A NM_000457.4(HNF4A): c.641_648+10del18 deletion Likely pathogenic rs193922475 GRCh37 Chromosome 20, 43043295: 43043312
12 HNF4A NM_000457.4(HNF4A): c.726_728delGCTinsTCAA (p.Leu243Glnfs) indel Likely pathogenic rs193922476 GRCh37 Chromosome 20, 43047142: 43047144
13 HNF4A NM_000457.4(HNF4A): c.834G> C (p.Glu278Asp) single nucleotide variant Likely pathogenic rs193922477 GRCh37 Chromosome 20, 43048458: 43048458
14 HNF4A NM_000457.4(HNF4A): c.991C> T (p.Arg331Cys) single nucleotide variant Likely pathogenic rs193922479 GRCh37 Chromosome 20, 43052756: 43052756
15 HNF4A NM_000457.4(HNF4A): c.997C> T (p.Arg333Cys) single nucleotide variant Likely pathogenic rs193922480 GRCh37 Chromosome 20, 43052762: 43052762
16 HNF4A deletion Likely pathogenic
17 HNF4A NM_175914.4(HNF4A): c.640T> A (p.Ser214Thr) single nucleotide variant Likely pathogenic rs1060499693 GRCh37 Chromosome 20, 43047122: 43047122

Expression for Mody, Type I

Search GEO for disease gene expression data for Mody, Type I.

Pathways for Mody, Type I

GO Terms for Mody, Type I

Sources for Mody, Type I

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 Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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