MCID: HYP060
MIFTS: 55

Hyperinsulinism

Categories: Endocrine diseases, Metabolic diseases

Aliases & Classifications for Hyperinsulinism

MalaCards integrated aliases for Hyperinsulinism:

Name: Hyperinsulinism 12 55 44 15 72
Hyperinsulinemia 12 29 55 6 15

Classifications:



External Ids:

Disease Ontology 12 DOID:2018
MeSH 44 D006946
SNOMED-CT 68 83469008
ICD10 33 E16.1
UMLS 72 C0020459

Summaries for Hyperinsulinism

MalaCards based summary : Hyperinsulinism, also known as hyperinsulinemia, is related to hyperinsulinemic hypoglycemia, familial, 6 and hyperinsulinemic hypoglycemia, familial, 7. An important gene associated with Hyperinsulinism is HNF4A (Hepatocyte Nuclear Factor 4 Alpha), and among its related pathways/superpathways are Regulation of lipid metabolism Insulin signaling-generic cascades and Aldosterone synthesis and secretion. The drugs Curcumin and Ribavirin have been mentioned in the context of this disorder. Affiliated tissues include ovary, liver and skeletal muscle, and related phenotypes are Reduced mammosphere formation and homeostasis/metabolism

Wikipedia : 75 Hyperinsulinism refers to an above normal level of insulin in the blood of a person or animal. Normal... more...

Related Diseases for Hyperinsulinism

Diseases related to Hyperinsulinism via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 545)
# Related Disease Score Top Affiliating Genes
1 hyperinsulinemic hypoglycemia, familial, 6 34.4 INS HADH GLUD1
2 hyperinsulinemic hypoglycemia, familial, 7 34.1 SLC16A1 INS
3 alstrom syndrome 33.1 LEP KCNJ11 INS
4 3-hydroxyacyl-coa dehydrogenase deficiency 31.8 LEP INS HADH GLUD1 GHRL GCK
5 pineal hyperplasia, insulin-resistant diabetes mellitus, and somatic abnormalities 31.8 INSR INS IGFBP3 IGF1
6 insulinoma 31.4 SST INS GCK ABCC8
7 fasting hypoglycemia 31.1 INSR IGF1 GLUD1
8 munchausen by proxy 31.0 KCNJ11 GCK ABCC8
9 hypoglycemia 31.0 SLC16A1 KCNJ11 INSR INS HADH GLUD1
10 insulinomatosis and diabetes mellitus 30.7 SST INS ABCC8
11 maturity-onset diabetes of the young 30.7 KCNJ11 INS HNF4A GCK ABCC8
12 monogenic diabetes 30.6 KCNJ11 INS HNF4A GCK ABCC8
13 diabetes mellitus, permanent neonatal 30.6 KCNJ11 INS GCK ABCC8
14 donohue syndrome 30.5 INSR INS IGF1
15 maturity-onset diabetes of the young, type 2 30.5 INS HNF4A GCK
16 maturity-onset diabetes of the young, type 3 30.4 INS HNF4A GCK
17 acute insulin response 30.4 UCP2 KCNJ5 KCNJ11 INS ABCC8
18 hyperproinsulinemia 30.2 SHBG INS IGFBP1
19 neonatal diabetes mellitus 30.2 KCNJ5 KCNJ11 INS GCK ABCC8
20 pancreatic agenesis 30.1 KCNJ11 INS GCK ABCC8
21 maturity-onset diabetes of the young, type 4 30.1 HNF4A GCK
22 hypopituitarism 30.0 INS IGFBP3 IGF1
23 hyperandrogenism 30.0 SHBG INSR INS IGFBP1 IGF1
24 abdominal obesity-metabolic syndrome 1 29.9 SERPINE1 LEP INS
25 prediabetes syndrome 29.9 LEP INS APOB
26 ovarian disease 29.8 SHBG INS IGFBP1 IGF1
27 hyperinsulinemic hypoglycemia 29.8 SST SLC16A1 KCNJ11 INSR INS HNF4A
28 anovulation 29.7 SHBG LEP INS IGF1
29 acanthosis nigricans 29.7 SHBG LEP INSR INS IGF1
30 gestational diabetes 29.7 SHBG LEP KCNJ11 INSR INS HNF4A
31 diffuse idiopathic skeletal hyperostosis 29.6 IGFBP3 IGF1
32 craniopharyngioma 29.6 INS IGF1 GHRL
33 polycystic ovary syndrome 29.6 SHBG LEP INSR INS IGFBP1 IGF1
34 familial hyperlipidemia 29.5 SERPINE1 INS APOB
35 growth hormone deficiency 29.4 IGFBP3 IGF1 GHRL
36 atherosclerosis susceptibility 29.3 SERPINE1 INS APOB
37 hyperglycemia 29.3 SST LEP KCNJ11 INSR INS IGF1
38 lipid metabolism disorder 29.2 SERPINE1 LEP INS APOB
39 sleep apnea 29.2 LEP INS IGF1 GHRL
40 turner syndrome 29.2 SHBG IGFBP3 IGFBP1 IGF1
41 coronary heart disease 1 29.2 SERPINE1 INS APOB
42 microvascular complications of diabetes 5 29.1 SERPINE1 INS IGF1
43 large intestine cancer 29.0 INS IGFBP3 IGF1 HNF4A
44 arteries, anomalies of 28.9 SERPINE1 LEP INS APOB
45 hyperthyroidism 28.8 SST SHBG LEP INS GHRL
46 hypertension, essential 28.8 SERPINE1 LEP INSR INS APOB
47 hypothyroidism 28.6 SHBG LEP IGFBP3 IGF1 APOB
48 vascular disease 28.6 SERPINE1 INS IGFBP1 APOB
49 arteriosclerosis 28.5 SERPINE1 INS APOB
50 fetal macrosomia 28.4 LEP INSR INS IGFBP3 IGF1 APOB

Graphical network of the top 20 diseases related to Hyperinsulinism:



Diseases related to Hyperinsulinism

Symptoms & Phenotypes for Hyperinsulinism

GenomeRNAi Phenotypes related to Hyperinsulinism according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Reduced mammosphere formation GR00396-S 9.23 ABCC8 GHRL HNF4A IGF1 IGFBP3 KCNJ11

MGI Mouse Phenotypes related to Hyperinsulinism:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.25 ABCC8 APOB GCK GHRL GLUD1 HADH
2 growth/size/body region MP:0005378 10.24 APOB GCK GLUD1 HADH HNF4A IGF1
3 adipose tissue MP:0005375 10.16 GHRL HADH IGF1 IGFBP3 INS INSR
4 endocrine/exocrine gland MP:0005379 10.14 ABCC8 GCK GHRL GLUD1 HADH IGF1
5 cardiovascular system MP:0005385 10.03 APOB IGF1 INS INSR KCNJ11 KCNJ5
6 liver/biliary system MP:0005370 9.96 APOB GCK HNF4A IGFBP1 IGFBP3 INS
7 mortality/aging MP:0010768 9.93 APOB GCK HNF4A IGF1 INS INSR
8 muscle MP:0005369 9.5 APOB IGF1 IGFBP3 INS INSR KCNJ11
9 renal/urinary system MP:0005367 9.17 GCK HADH IGF1 INS INSR LEP

Drugs & Therapeutics for Hyperinsulinism

Drugs for Hyperinsulinism (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 365)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Curcumin Approved, Experimental, Investigational Phase 4 458-37-7 969516
2
Ribavirin Approved Phase 4 36791-04-5 37542
3
Parecoxib Approved Phase 4 198470-84-7
4
Tramadol Approved, Investigational Phase 4 27203-92-5 33741
5
Celecoxib Approved, Investigational Phase 4 169590-42-5 2662
6
Dopamine Approved Phase 4 51-61-6, 62-31-7 681
7
Tocopherol Approved, Investigational Phase 4 1406-66-2, 54-28-4 14986
8
Methylcobalamin Approved, Experimental, Investigational Phase 4 13422-55-4
9
Hydroxocobalamin Approved Phase 4 13422-51-0 11953898 15589840
10
Ethinyl Estradiol Approved Phase 4 57-63-6 5991
11
Drospirenone Approved Phase 4 67392-87-4 68873
12
Losartan Approved Phase 4 114798-26-4 3961
13
Nateglinide Approved, Investigational Phase 4 105816-04-4 60026
14
Caffeine Approved Phase 4 58-08-2 2519
15
Guaifenesin Approved, Investigational, Vet_approved Phase 4 93-14-1 3516
16
Omeprazole Approved, Investigational, Vet_approved Phase 4 73590-58-6 4594
17
Dextromethorphan Approved Phase 4 125-71-3 5360696 5362449
18
Midazolam Approved, Illicit Phase 4 59467-70-8 4192
19
Chlorzoxazone Approved Phase 4 95-25-0 2733
20
Tolbutamide Approved, Investigational Phase 4 64-77-7 5505
21
Bupropion Approved Phase 4 34841-39-9, 34911-55-2 444
22
Aripiprazole Approved, Investigational Phase 4 129722-12-9 60795
23
Icodextrin Approved, Investigational Phase 4 337376-15-5
24
Atorvastatin Approved Phase 4 134523-00-5 60823
25
Pasireotide Approved Phase 4 396091-73-9 9941444
26
Zinc Approved, Investigational Phase 4 7440-66-6 32051
27
tannic acid Approved Phase 4 1401-55-4
28
Benzocaine Approved, Investigational Phase 4 94-09-7, 1994-09-7 2337
29
Testosterone enanthate Approved Phase 4 315-37-7 9416
30
Methyltestosterone Approved Phase 4 58-18-4 6010
31
Testosterone undecanoate Approved, Investigational Phase 4 5949-44-0
32
Metformin Approved Phase 4 657-24-9 4091 14219
33
Rosiglitazone Approved, Investigational Phase 4 122320-73-4 77999
34
Pioglitazone Approved, Investigational Phase 4 111025-46-8 4829
35
Angiotensin II Approved, Investigational Phase 4 11128-99-7, 68521-88-0, 4474-91-3 172198
36
Candesartan cilexetil Approved Phase 4 145040-37-5 2540
37
Progesterone Approved, Vet_approved Phase 4 57-83-0 5994
38
Testosterone Approved, Experimental, Investigational Phase 4 481-30-1, 58-22-0 6013 10204
39
Fenofibrate Approved Phase 4 49562-28-9 3339
40
Leuprolide Approved, Investigational Phase 4 53714-56-0 3911 657181
41 Garlic Approved, Nutraceutical Phase 4
42
Vitamin E Approved, Nutraceutical, Vet_approved Phase 4 59-02-9 14985
43
Cyanocobalamin Approved, Nutraceutical Phase 4 68-19-9 44176380
44
Vitamin D3 Approved, Nutraceutical Phase 4 67-97-0 6221 5280795
45
Ergocalciferol Approved, Nutraceutical Phase 4 50-14-6 5280793
46
Manganese Approved, Nutraceutical Phase 4 7439-96-5 27854
47
Inulin Approved, Investigational, Nutraceutical Phase 4 9005-80-5 24763
48 Tocotrienol Investigational Phase 4 6829-55-6
49
Cobalamin Experimental Phase 4 13408-78-1 6857388
50
Candesartan Experimental Phase 4 139481-59-7 2541

Interventional clinical trials:

(show top 50) (show all 331)
# Name Status NCT ID Phase Drugs
1 Treatment With Lanreotide Autogel (Somatostatin Analogue) in Patients With Congenital Hyperinsulinism of Infancy Already Treated With Somatostatin Analog by Pump Unknown status NCT01070758 Phase 4 Lanreotide autogel
2 Double-blind Randomized Trial Using Oral Metformin Versus Placebo in the Treatment of Acanthosis Nigricans in Children With Obesity Unknown status NCT02438020 Phase 4 Metformin
3 To Compare the Efficacy and Safety Between Pioglitazone Added to Combination Therapy of Sulfonylurea Plus Metformin and Placebo Control Group in Patients With Type 2 Diabetes Unknown status NCT00991055 Phase 4 Pioglitazone
4 Antiatherogenic and Antimetabolic Effect of Curcumin in Type 2 Diabetic Patients Unknown status NCT01052597 Phase 4 curcumin
5 An Efficacy and Safety Study Comparing Pegylated-Interferon and Ribavirin Plus Metformin to Pegylated-Interferon and Ribavirin in the Treatment of naïve Patients With Genotype 1 Chronic HCV Infection and Insulin Resistance Unknown status NCT00370617 Phase 4 metformin
6 Effect of COX-2 Selective Inhibitors on Postoperative Insulin Resistance, Tramadol PCA and Early Postoperative Enteral Nutrition in Patients After Gastrointestinal Laparoscopic Surgery Unknown status NCT01930318 Phase 4 parecoxib
7 A PROSPECTIVE, RANDOMIZED, DOUBLE-BLIND, PLACEBO-CONTROLLED TRIAL OF THE SUPPLEMENTATION WITH KIOLIC® IN PATIENTS WITH DIAGNOSIS OF METABOLIC SYNDROME TO IMPROVE ENDOTHELIAL FUNCTION AND INFLAMMATORY STATE. "KYMES" Unknown status NCT01168700 Phase 4 aged garlic extract;Placebo
8 Insulin Resistance and Mild Cognitive Impairment (MCI) in Older Chinese Adults With Pre-Diabetes and Diabetes: Cognitive Effects of Lifestyle Intervention and Metformin Treatment in a Randomized Controlled Trial Unknown status NCT02409238 Phase 4 Metformin
9 The ACT-OUT Trial: ACTivity OUTcomes Based on the Consumption of a High Carbohydrate or High Fat Diet in Patients With Metabolic Syndrome Unknown status NCT01357382 Phase 4
10 Clinical Metabolic and Endocrine Parameters in Response to Metformin and Lifestyle Intervention in Women With Polycystic Ovary Syndrome: A Phase 4 Randomized, Double- Blind and Placebo Control Trial Completed NCT00679679 Phase 4 Metformin;Placebo
11 Metabolic Effect of Metformin in Obese Insulin Resistant Adolescents With Normal Glucose Tolerance Completed NCT00667498 Phase 4 Metformin;Placebo
12 Obesity, Insulin Resistance, and Bone Metabolism in Adolescents With PCOS: Effects of Insulin Sensitizers Versus Oral Contraceptives Completed NCT00640224 Phase 4 rosiglitazone;drospirenone/ethinyl estradiol
13 Role of Myo-inositol and D-chiro Inositol on the Ovaric and Metabolic Functions Completed NCT01626443 Phase 4
14 Metformin vs. Acarbose Treatment in Infertile Overweight Women With PCOS: A Prospective Randomized Clinical Trial Completed NCT01279512 Phase 4 Metformin;Acarbose
15 Myo-inositol Versus D-chiro-inositol in the Treatment of Polycystic Ovary Syndrome and Insulin Resistance: Evaluation of Clinical, Metabolic, Endocrine and Ultrasound Parameters Completed NCT01514942 Phase 4 Folic acid, vit B12;Folic acid, vit B12
16 Losartan Improved Insulin Resistance and Decreased Inflammatory Cytokines in Patients With Chronic Heart Failure Treated With Angiotensin Converting Enzyme Inhibitors Completed NCT00663377 Phase 4 losartan
17 Insulin Resistance and Central Nervous System (CNS) Function in Type 2 Diabetes Completed NCT00212290 Phase 4 pioglitazone;nateglinide;placebo
18 Effect of Pioglitazone on Endothelial Function in Premenopausal Women With Uncomplicated Systemic Lupus Erythematosus, a Randomized, Double-blind, Placebo-controlled Clinical Trial Completed NCT01322308 Phase 4 pioglitazone;placebo
19 Targeting Peroxisome Proliferator-Activated Receptor-Gamma in Peritoneal Dialysis Patients - Will it Reduce Inflammation, Atherosclerosis, Calcification and Improve Survival of Peritoneal Dialysis Patients? Completed NCT00745225 Phase 4 Pioglitazone;placebo comparator
20 Effect of Magnesium Administration in Subjects With Family History of Diabetes or Metabolic Syndrome Completed NCT01181830 Phase 4 magnesium pidolate;placebo
21 Multicenter, Prospective, Randomised, Open-label Study, Evaluating the Effect of Two Levels of Haemoglobin on Quality of Life and Speed of Progression of Renal Insufficiency on Renal Transplanted Patients With Chronic Graft Dysfunction (CGD) Completed NCT00396435 Phase 4 Neorecormon
22 Cardiovascular Effects of Chronic Sildenafil (Viagra) Treatment in Diabetic Subjects With Endothelial Dysfunction. Completed NCT00692237 Phase 4 Sildenafil;Placebo
23 Effect of a Basal/Pre-Meal Insulin Strategy (Detemir/Aspart) to Improve Insulin Secretion and Action in Subjects With Type 2 Diabetes Completed NCT00998335 Phase 4 Long-acting bedtime insulin detemir (Levemir);Insulin detemir and pre-meal insulin aspart.
24 Long-term Role of Pioglitazone in Non-Alcoholic Fatty Liver Disease (NAFLD) in Type 2 Diabetes Mellitus (T2DM). Completed NCT00994682 Phase 4 Pioglitazone study drug;Placebo;Pioglitazone Open Label
25 A New Treatment Strategy of Adding Exenatide to Insulin Therapy for Patients With Type 2 Diabetes and Non-Alcoholic Fatty Liver Disease (NAFLD) Completed NCT01006889 Phase 4 Exenatide
26 Effects of Type 2 Diabetes on CYP450s Activities; Intersubject Variability in Drug Metabolism. Completed NCT02291666 Phase 4 CRCHUM-MT cocktail
27 Changes in Adiposity and Metabolic Measures During Medication Switches to Aripiprazole From Other Atypical Antipsychotics Completed NCT00205660 Phase 4
28 NAFLD in T2DM: Prevalence in Hispanics and Role of Treatment Completed NCT01002547 Phase 4 pioglitazone-placebo;pioglitazone;pioglitazone-placebo;Vitamin E-placebo
29 The Effects of Vitamin D Supplementation on Transforming Growth Factor-beta1 and Vascular Endothelial Growth Factor in Vitamin D-Deficient Women With Polycystic Ovary Syndrome: A Randomized Placebo-Controlled Trial Completed NCT02460380 Phase 4 Vitamin D3
30 Prospective, Randomized, Double - Blind, Placebo Controlled Trial of Dietary Modification in Conjunction With Probiotic Therapy on Clinical and Endocrinological Parameters as Well as Body Composition in Women With Polycystic Ovary Syndrome Recruiting NCT03325023 Phase 4
31 Effect of Myo-Inositol- Based Co-treatment on Oocyte Quality in Women With Polycystic Ovarian Syndrome Undergoing Assisted Reproductive Technology Recruiting NCT03177122 Phase 4 Myo-inositol
32 Effects of Non-Glucose-Based Peritoneal Dialysis Solution "EXTRANEAL" on Changes in Leptin Levels and Sympathetic Activity Induced by Conventional Glucose-Based Dialysate "DIANEAL" in Patients on Peritoneal Dialysis Recruiting NCT01228279 Phase 4
33 Relationship Between Insulin Resistance and Statin Induced Type 2 Diabetes, and Integrative Personal Omics Profiling Recruiting NCT02437084 Phase 4 Atorvastatin
34 Enhancing the Anabolic Effect of Perioperative Nutrition With Insulin While Maintaining Normoglycemia Active, not recruiting NCT02032953 Phase 4 Insulin
35 Pasireotide in the Treatment of Hypoglycemia Following Gastric Bypass Surgery Enrolling by invitation NCT03514576 Phase 4 Pasireotide 0.3 MG/ML
36 52 Week RCT to Investigate the Effect of Testosterone Undecanoate vs Placebo on Intrahepatic Fat Content in Obese Men With T2DM and Hypogonadism and Subsequent 108 Week Open Label Phase to Investigate Effects on Cardiometabolic Parameters Not yet recruiting NCT03851627 Phase 4 Testosterone Undecanoate;Placebo
37 Can Addition of Pioglitazone to SGLT2 Inhibitor in Type 1 Diabetic Patients Amplify the Decrease in HbA1c and Prevent the Increase in Plasma Ketone Concentration? Not yet recruiting NCT03878459 Phase 4 Pioglitazone 45 mg;Placebo
38 Insulin Therapy Reduce Post-Operative Inflammatory Response After Curative Colorectal Cancer Resection: Randomization Controlled Trial Not yet recruiting NCT02746432 Phase 4 Hyper insulinemic euglycemic clamp
39 A Randomized, Double Blind, Cross-Over, Placebo-Controlled Clinical Trial to Assess the Effects of Candesartan on the Carbohydrate Metabolism, of Non Diabetic, Non Hypertensive Subjects With Dysglycemia and Abdominal Obesity."ARAMIA" Terminated NCT00319202 Phase 4 Candesartan;Placebo
40 Hyperinsulinemia and Insulin Resistance in Nonalcoholic Fatty Liver Disease. Metformin for the Treatment of Nonalcoholic Fatty Liver Disease: A Randomized, Double-Blinded, Placebo-Controlled Trial Terminated NCT00736385 Phase 4 Glucophage (Metformin);Placebo
41 Rosiglitazone And Fenofibrate Additive Effects on Lipids (RAFAEL) Terminated NCT00819910 Phase 4 Rosiglitazone;Placebo (Rosiglitazone);Placebo (Fenofibrate);Fenofibrate
42 Sleep, Metabolic, and Cardiovascular Dysfunction in Polycystic Ovary Syndrome Terminated NCT00203996 Phase 4 depot leuprolide plus estrogen/progestin replacement;pioglitazone
43 A Phase III Randomized, Double-blind, Parallel-group Study to Evaluate the Efficacy and Safety of Acarmet (Metformin HCl 500 mg Plus Acarbose 50 mg Tablets) Versus Acarbose Alone in Subjects With Type 2 Diabetes Mellitus Unknown status NCT01245166 Phase 3 Acarbose;Metformin/Acarbose
44 Clinical Assessment of the Treatment With Diazoxide in Children Suffering From Obesity and Hyperinsulinemia Secondary to Surgery of Hypothalamic-pituitary Lesions Completed NCT00306683 Phase 3 DIAZOXIDE
45 A Multicenter, Randomized, Double-Blind, Active-Controlled Study to Evaluate the Durability of the Efficacy and Safety of Alogliptin Compared to Glipizide When Used in Combination With Metformin in Subjects With Type 2 Diabetes Completed NCT00856284 Phase 3 Alogliptin;Metformin;Glipizide
46 TART - Troglitazone Atherosclerosis Regression Trial Completed NCT00116545 Phase 2, Phase 3 troglitazone
47 A Multicenter, Prospective, Randomized Study to Assess the Effect of Metformin Supplementation on IVF Outcome and Intrafollicular Environment in Patients With Polycystic Ovarian Syndrome Undergoing In Vitro Fertilization/Embryo Transfer Completed NCT03086005 Phase 3 Metformin
48 Effects of Metformin on Fertility and Pregnancy in Women With Polycystic Ovary Syndrome: a Randomized, Prospective, Placebo-controlled Multicenter Study Completed NCT00994812 Phase 3 metformin
49 Effect of Administration of Combination Metformine/Inulin Versus Metformine on Patients With Prostate Benign Hyperplasia and Metabolic Syndrome Syndrome Completed NCT02778776 Phase 3 Metformin
50 Effect of GO2KA1(Chitosan Oligosaccharide) on Postprandial Blood Glucose in Korean Adults: a Clinical Randomized Trial Completed NCT01496820 Phase 2, Phase 3

Search NIH Clinical Center for Hyperinsulinism

Cochrane evidence based reviews: hyperinsulinism

Genetic Tests for Hyperinsulinism

Genetic tests related to Hyperinsulinism:

# Genetic test Affiliating Genes
1 Hyperinsulinemia 29

Anatomical Context for Hyperinsulinism

MalaCards organs/tissues related to Hyperinsulinism:

41
Ovary, Liver, Skeletal Muscle, Heart, Pancreas, Endothelial, Breast

Publications for Hyperinsulinism

Articles related to Hyperinsulinism:

(show top 50) (show all 14156)
# Title Authors PMID Year
1
Plasma apelin levels in subjects with nonalcoholic fatty liver disease. 9 38
20045153 2010
2
Apelin and APJ regulation in adipose tissue and skeletal muscle of type 2 diabetic mice and humans. 9 38
20233941 2010
3
Adaptations in mitochondrial function parallel, but fail to rescue, the transition to severe hyperglycemia and hyperinsulinemia: a study in Zucker diabetic fatty rats. 9 38
19875988 2010
4
Changes in cytokine levels during acute hyperinsulinemia in offspring of type 2 diabetic subjects. 9 38
20031127 2010
5
The effect of insulin signaling on female reproductive function independent of adiposity and hyperglycemia. 9 38
20176725 2010
6
Hypoadiponectinemia--cause or consequence of human "insulin resistance"? 9 38
20164291 2010
7
A common polymorphism in the promoter of UCP2 is associated with obesity and hyperinsulenemia in northern Indians. 9 38
19908126 2010
8
Changes in the expression of insulin signaling pathway molecules in endometria from polycystic ovary syndrome women with or without hyperinsulinemia. 9 38
20011249 2010
9
NO-1886 suppresses diet-induced insulin resistance and cholesterol accumulation through STAT5-dependent upregulation of IGF1 and CYP7A1. 9 38
19815588 2010
10
Impact of disease-causing SUR1 mutations on the KATP channel subunit interface probed with a rhodamine protection assay. 9 38
19933268 2010
11
On the reversibility of glutamate dehydrogenase and the source of hyperammonemia in the hyperinsulinism/hyperammonemia syndrome. 9 38
19895831 2010
12
Congenital hyperinsulinism due to a compound heterozygous ABCC8 mutation with spontaneous resolution at eight weeks. 9 38
20215776 2010
13
The increase in plasma PAI-1 associated with insulin resistance may be mediated by the presence of hepatic steatosis. 9 38
19656511 2010
14
Impact of type 1 diabetes and insulin treatment on plasma levels and fractional synthesis rate of retinol-binding protein 4. 9 38
19850685 2009
15
Opposite clinical phenotypes of glucokinase disease: Description of a novel activating mutation and contiguous inactivating mutations in human glucokinase (GCK) gene. 9 38
19884385 2009
16
Acute hyperinsulinemia differentially regulates interstitial and circulating adiponectin oligomeric pattern in lean and insulin-resistant, obese individuals. 9 38
19820029 2009
17
Metabolic syndrome and risk of subsequent colorectal cancer. 9 38
19891012 2009
18
Metabolic syndrome racial differences in adolescents. 9 38
19925390 2009
19
Fasting hyperinsulinemia associates with increased sub-clinical inflammation in first-degree relatives normal glucose tolerant women independently of the metabolic syndrome. 9 38
19685554 2009
20
The change in sex hormone binding globulin and the influence by gestational diabetes mellitus in fetal period. 9 38
19557594 2009
21
Genetic variant near IRS1 is associated with type 2 diabetes, insulin resistance and hyperinsulinemia. 9 38
19734900 2009
22
Molecular mechanisms involved in obesity-associated insulin resistance: therapeutical approach. 9 38
19673658 2009
23
Molecular biology of K(ATP) channels and implications for health and disease. 9 38
19787700 2009
24
Insulin receptor isoforms and insulin receptor/insulin-like growth factor receptor hybrids in physiology and disease. 9 38
19752219 2009
25
Central leptin gene therapy ameliorates diabetes type 1 and 2 through two independent hypothalamic relays; a benefit beyond weight and appetite regulation. 9 38
19647774 2009
26
Low-birth weight children develop lower sex hormone binding globulin and higher dehydroepiandrosterone sulfate levels and aggravate their visceral adiposity and hypoadiponectinemia between six and eight years of age. 9 38
19737922 2009
27
[Obesity and cancer]. 9 38
19524333 2009
28
Alterations in cochlear function during induced acute hyperinsulinemia in an animal model. 9 38
19893948 2009
29
Forkhead transcription factor FoxO1 inhibits insulin- and transforming growth factor-beta-stimulated plasminogen activator inhibitor-1 expression. 9 38
19563779 2009
30
The hypertriglyceridemic waist phenotype versus the National Cholesterol Education Program-Adult Treatment Panel III and International Diabetes Federation clinical criteria to identify high-risk men with an altered cardiometabolic risk profile. 9 38
19481769 2009
31
Circumventing glucocorticoid-mediated hyperinsulinemia via the activation of PPARalpha. 9 38
19625768 2009
32
American Diabetes Association--69th Scientific sessions. Hyperinsulinism, insulin resistance and the treatment of diabetes: Part 2. 9 38
19629874 2009
33
Novel inhibitors complexed with glutamate dehydrogenase: allosteric regulation by control of protein dynamics. 9 38
19531491 2009
34
Effects of a PPAR-gamma agonist, on growth factor and insulin stimulated endothelial cells. 9 38
19520186 2009
35
A novel melanocortin-4 receptor gene mutation in a female patient with severe childhood obesity. 9 38
19214805 2009
36
Insulin resistance and carotid atherosclerosis in patients with type 2 diabetes. 9 38
19147142 2009
37
Identification of a diffuse form of hyperinsulinemic hypoglycemia by 18-fluoro-L-3,4 dihydroxyphenylalanine positron emission tomography/CT in a patient carrying a novel mutation of the HADH gene. 9 38
19318379 2009
38
Extremes of clinical and enzymatic phenotypes in children with hyperinsulinism caused by glucokinase activating mutations. 9 38
19336674 2009
39
Acute hyperinsulinemia raises plasma interleukin-6 in both nondiabetic and type 2 diabetes mellitus subjects, and this effect is inversely associated with body mass index. 9 38
19375766 2009
40
Acylated ghrelin concentrations are markedly decreased during pregnancy in mothers with and without gestational diabetes: relationship with cholinesterase. 9 38
19240252 2009
41
The spectrum of ABCC8 mutations in Norwegian patients with congenital hyperinsulinism of infancy. 9 38
19475716 2009
42
The genetic basis of congenital hyperinsulinism. 9 38
19254908 2009
43
States of serum leptin and insulin in children with epilepsy: risk predictors of weight gain. 9 38
18586538 2009
44
[Hyperinsulinism in infancy and childhood: when an insulin level is not always enough]. 9 38
19411227 2009
45
(Pre)diabetes, brain aging, and cognition. 9 38
19135149 2009
46
Down-regulation of hepatic HNF4alpha gene expression during hyperinsulinemia via SREBPs. 9 38
19179483 2009
47
[Adipokines and highly active antiretroviral therapy related lipodystrophy: clinical study of 52 cases]. 9 38
19671283 2009
48
Insulin-like growth factor-binding protein-1: serum levels, promoter polymorphism, and associations with components of the metabolic syndrome in short subjects born small for gestational age. 9 38
19158202 2009
49
Diagnostic difficulties in glucokinase hyperinsulinism. 9 38
19053014 2009
50
Long-term resveratrol administration reduces metabolic disturbances and lowers blood pressure in obese Zucker rats. 9 38
19100718 2009

Variations for Hyperinsulinism

ClinVar genetic disease variations for Hyperinsulinism:

6
# Gene Variation Type Significance SNP ID GRCh37 Pos GRCh38 Pos
1 HNF4A NM_000457.4(HNF4A): c.253C> T (p.Arg85Trp) single nucleotide variant Pathogenic rs587777732 20:43034835-43034835 20:44406195-44406195
2 KCNJ11 NM_000525.3(KCNJ11): c.185C> G (p.Thr62Arg) single nucleotide variant Likely pathogenic rs1057518775 11:17409454-17409454 11:17387907-17387907
3 HNF4A NM_000457.4(HNF4A): c.991C> T (p.Arg331Cys) single nucleotide variant Conflicting interpretations of pathogenicity rs193922479 20:43052756-43052756 20:44424116-44424116

Expression for Hyperinsulinism

Search GEO for disease gene expression data for Hyperinsulinism.

Pathways for Hyperinsulinism

Pathways related to Hyperinsulinism according to GeneCards Suite gene sharing:

(show all 22)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.77 SERPINE1 INSR INS IGFBP3 IGF1 GCK
2
Show member pathways
12.76 KCNJ5 KCNJ11 INS GCK ABCC8
3 12.29 UCP2 INSR INS IGFBP3 IGFBP1 HNF4A
4
Show member pathways
12.27 LEP INSR INS IGF1 HNF4A
5
Show member pathways
12.22 SERPINE1 INSR INS APOB
6
Show member pathways
12.14 LEP INS IGF1 GHRL
7 11.92 SERPINE1 LEP INS IGF1
8
Show member pathways
11.87 INSR INS HNF4A GCK
9
Show member pathways
11.84 KCNJ5 KCNJ11 ABCC8
10
Show member pathways
11.77 INS HNF4A GCK
11 11.76 SERPINE1 INS IGFBP3 IGF1
12 11.74 SERPINE1 INSR INS IGF1
13 11.49 KCNJ5 KCNJ11 ABCC8
14 11.45 LEP KCNJ11 ABCC8
15 11.42 LEP INSR INS
16
Show member pathways
11.42 KCNJ11 INSR INS HNF4A GCK ABCC8
17 11.4 INSR INS IGF1
18 11.37 SERPINE1 LEP IGFBP1 HNF4A GCK
19 11.25 INSR INS IGF1
20 11.19 SLC16A1 SERPINE1 IGFBP3 IGFBP1
21 10.87 IGFBP3 IGFBP1 IGF1
22 10.87 UCP2 KCNJ11 INS IGFBP1 HNF4A HADH

GO Terms for Hyperinsulinism

Cellular components related to Hyperinsulinism according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.91 SST SERPINE1 LEP INS IGFBP3 IGFBP1
2 extracellular region GO:0005576 9.85 SST SHBG SERPINE1 LEP INS IGFBP3
3 endosome lumen GO:0031904 9.4 INS APOB
4 inward rectifying potassium channel GO:0008282 9.37 KCNJ11 ABCC8
5 insulin-like growth factor ternary complex GO:0042567 9.32 IGFBP3 IGF1
6 endoplasmic reticulum lumen GO:0005788 9.02 INS IGFBP3 IGFBP1 GHRL APOB
7 insulin-like growth factor binding protein complex GO:0016942 8.96 IGFBP3 IGF1

Biological processes related to Hyperinsulinism according to GeneCards Suite gene sharing:

(show all 35)
# Name GO ID Score Top Affiliating Genes
1 response to drug GO:0042493 9.97 SST KCNJ11 HADH ABCC8
2 negative regulation of apoptotic process GO:0043066 9.95 UCP2 LEP IGF1 GHRL
3 response to estradiol GO:0032355 9.83 LEP KCNJ11 APOB
4 cellular protein metabolic process GO:0044267 9.83 INS IGFBP3 IGFBP1 IGF1 APOB
5 female pregnancy GO:0007565 9.82 UCP2 LEP ABCC8
6 cellular response to insulin stimulus GO:0032869 9.81 UCP2 INSR GCK
7 insulin receptor signaling pathway GO:0008286 9.79 INSR INS IGFBP1
8 positive regulation of MAPK cascade GO:0043410 9.77 LEP INSR INS IGFBP3 IGF1
9 response to insulin GO:0032868 9.76 LEP HADH ABCC8
10 positive regulation of cold-induced thermogenesis GO:0120162 9.76 UCP2 LEP HADH GHRL
11 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.75 LEP INS IGF1
12 positive regulation of insulin secretion GO:0032024 9.71 GLUD1 GHRL GCK
13 positive regulation of glucose import GO:0046326 9.7 INSR INS IGF1
14 activation of protein kinase B activity GO:0032148 9.69 INSR INS IGF1
15 positive regulation of mitotic nuclear division GO:0045840 9.67 INSR INS IGF1
16 cellular glucose homeostasis GO:0001678 9.66 GCK ABCC8
17 positive regulation of insulin receptor signaling pathway GO:0046628 9.66 LEP INS
18 negative regulation of gluconeogenesis GO:0045721 9.65 INS GCK
19 glucose metabolic process GO:0006006 9.65 LEP KCNJ11 INS GHRL GCK
20 negative regulation of wound healing GO:0061045 9.64 SERPINE1 ABCC8
21 positive regulation of insulin-like growth factor receptor signaling pathway GO:0043568 9.63 IGFBP3 IGF1
22 negative regulation of smooth muscle cell migration GO:0014912 9.62 SERPINE1 IGFBP3
23 negative regulation of insulin secretion GO:0046676 9.62 KCNJ11 HADH GHRL ABCC8
24 neuron projection maintenance GO:1990535 9.61 INSR INS
25 cellular response to leptin stimulus GO:0044320 9.6 LEP GCK
26 adult feeding behavior GO:0008343 9.59 LEP GHRL
27 positive regulation of glycolytic process GO:0045821 9.58 INSR INS IGF1
28 positive regulation of respiratory burst GO:0060267 9.57 INSR INS
29 positive regulation of developmental growth GO:0048639 9.56 LEP INSR
30 bone mineralization involved in bone maturation GO:0035630 9.55 LEP IGF1
31 positive regulation of glycogen biosynthetic process GO:0045725 9.46 INSR INS IGF1 GCK
32 glucose homeostasis GO:0042593 9.43 SLC16A1 LEP INSR INS HNF4A GCK
33 regulation of signaling receptor activity GO:0010469 9.18 SERPINE1
34 regulation of insulin secretion GO:0050796 9.1 SLC16A1 LEP KCNJ11 HNF4A GCK ABCC8
35 lipid metabolic process GO:0006629 10.02 SLC16A1 LEP HNF4A HADH APOB

Molecular functions related to Hyperinsulinism according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 insulin-like growth factor receptor binding GO:0005159 9.43 INSR INS IGF1
2 NAD+ binding GO:0070403 9.37 HADH GLUD1
3 insulin-like growth factor I binding GO:0031994 9.33 INSR IGFBP3 IGFBP1
4 insulin-like growth factor II binding GO:0031995 9.13 INSR IGFBP3 IGFBP1
5 ATP-activated inward rectifier potassium channel activity GO:0015272 9.02 KCNJ11
6 hormone activity GO:0005179 9.02 SST LEP INS IGF1 GHRL

Sources for Hyperinsulinism

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA