MCID: INS001
MIFTS: 59

Insulinoma malady

Rare diseases, Gastrointestinal diseases, Endocrine diseases, Cancer diseases categories

Summaries for Insulinoma

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9Disease Ontology, 66Wikipedia, 34MalaCards
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Wikipedia:66 An insulinoma is a tumor of the pancreas that is derived from beta cells and secretes insulin. more...

MalaCards: Insulinoma, also known as islet cell adenoma, is related to hypoglycemia and glucagonoma. An important gene associated with Insulinoma is ITS (Insulinoma tumor suppressor gene locus), and among its related pathways are Selected targets of HNF1 and G alpha (s) signalling events. The compounds repaglinide and secretoneurin have been mentioned in the context of this disorder. Affiliated tissues include pancreas, testes and pancreatic islet, and related mouse phenotypes are growth/size/body and endocrine/exocrine gland.

Disease Ontology:9 An adenoma that is a tumor in the pancreas that produces too much insulin.

Aliases & Classifications for Insulinoma

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9Disease Ontology, 44NIH Rare Diseases, 48OMIM, 11DISEASES, 46Novoseek, 50Orphanet, 63UMLS, 59SNOMED-CT, 41NCIt, 36MeSH, 27ICD10 via Orphanet, 37MESH via Orphanet, 60SNOMED-CT via Orphanet, 64UMLS via Orphanet
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Classifications:



Aliases & Descriptions:

insulinoma 9 44 48 11 46 50 63
islet cell adenoma 9 63
insulin-producing tumor of islet cells 9
adenoma islet cell 46
insulinoma nos 9
insuloma 50


External Ids:

Disease Ontology9 DOID:3892
NCIt41 C3140
MeSH36 D007340
ICD10 via Orphanet27 E16.8
MESH via Orphanet37 D007340
SNOMED-CT via Orphanet60 25324008, 302822000
UMLS via Orphanet64 C0021670

Related Diseases for Insulinoma

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18GeneCards, 19GeneDecks
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Diseases related to Insulinoma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 87)
idRelated DiseaseScoreTop Affiliating Genes
1hypoglycemia30.9SCT, IAPP, GCK, SLC2A2
2glucagonoma30.7SSTR5, SCT
3gastrinoma30.5SCT, IAPP, SSTR5, CASR
4hyperinsulinism30.3GCK, IAPP, PAX4, SCT
5insulin resistance30.3GCK, IAPP, PAX4
6diabetes mellitus30.3SCT, PAX4, GIPR, IAPP, WFS1, CASR
7type 2 diabetes mellitus30.3SCT, PAX4, IAPP, GCK, SLC2A2
8hyperparathyroidism30.2SCT, CASR
9neuroendocrine tumor30.2INSM1, SCT, SCG2
10pheochromocytoma30.2SCT, SCG2, IAPP, SSTR5
11primary hyperparathyroidism30.2CASR, SCT
12hypercalcemia30.1CASR, IAPP, SCT
13pituitary adenoma29.8CASR, SSTR5, SCG2
14glucose intolerance29.8SLC2A2, GCK, IAPP
15adenocarcinoma29.8CASR, IAPP, SCG2, SCT
16adenoma29.7SCT, SCG2, GIPR, SSTR5, CASR
17hyperglycemia29.7SLC2A2, GCK, IAPP, PAX4, SCT
18pancreatitis10.8
19metastatic insulinoma10.6
20multiple endocrine neoplasia10.6
21hepatitis10.4
22duodenitis10.3
23hyperinsulinemic hypoglycemia10.3
24obesity10.3
25seizure disorder10.3
26fasting hypoglycemia10.2
27gastroduodenitis10.2
28hypokalemia10.2
29diarrhea10.2
30pancreatic adenoma10.2
31wdha syndrome10.2
32sdha-related hereditary paraganglioma-pheochromocytoma syndrome10.2
33islet cell tumor10.1
34osteomalacia10.1CASR
35duodenal ulcer10.1
36zollinger-ellison syndrome10.1
37hypertension10.1
38neuropathy10.1
39pancreatic cancer10.1
40familial hypocalciuric hypercalcemia10.1
41somatostatin analog10.1
42adult-onset non-insulinoma persistent hyperinsulinemic hypoglycemia10.1
43wermer syndrome10.0SCT, CASR
44primary hyperoxaluria10.0SCT, IAPP, CASR
45gestational diabetes10.0GCK, IAPP
46maturity-onset diabetes of the young10.0GCK, PAX4
47thyroid medullary carcinoma10.0IAPP, SSTR5, CASR
48neonatal diabetes mellitus10.0SLC2A2, GCK
49lung small cell carcinoma10.0INSM1, SCT, SCG2
50metabolic syndrome x10.0SLC2A2, GCK, IAPP

Graphical network of the top 20 diseases related to Insulinoma:



Diseases related to insulinoma

Symptoms for Insulinoma

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Drugs & Therapeutics for Insulinoma

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
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Approved drugs:

Search CenterWatch for Insulinoma

Drug clinical trials:

Search ClinicalTrials for Insulinoma

Search NIH Clinical Center for Insulinoma

Search CenterWatch for Insulinoma

Genetic Tests for Insulinoma

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Anatomical Context for Insulinoma

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Sources:
32LifeMap Discoveryā„¢, 34MalaCards
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MalaCards organs/tissues related to Insulinoma:

34
Pancreas, Testes, Pancreatic islet, Liver, T cells, Pituitary, Spleen, Brain, B cells, Lung, Lymph node, Appendix, Hypothalamus, Bone, Retina, Temporal lobe

LifeMap Discovery
The database of embryonic development, stem cell research and regenerative medicine

Cells/anatomical compartments in embryo or adult related to Insulinoma:
id TissueAnatomical CompartmentCell Relevance
1 PancreasIslets of LangerhansMature Beta Cells Potential therapeutic candidate, affected by disease

Animal Models for Insulinoma or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Insulinoma:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053787.3PAX4, IAPP, WFS1, CASR, GCK, SLC2A2
2MP:00053796.5SLC2A2, INSM1, PAX4, GIPR, SSTR5, WFS1
3MP:00053765.8CASR, WFS1, SSTR5, IAPP, GIPR, SCT

Publications for Insulinoma

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53PubMed
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Articles related to Insulinoma:

(show top 50)    (show all 735)
idTitleAuthorsYear
1
Canine insulinoma as a model for studying molecular genetics of tumorigenesis and metastasis. (23707578)
2013
2
An insulinoma presenting with hypochondriac delusions and food refusal. (23835036)
2013
3
Giant pancreatic insulinoma. The bigger the worse? Report of two cases and literature review. (23333851)
2013
4
Sphingosine kinase 1 knockdown reduces insulin synthesis and secretion in a rat insulinoma cell line. (22155656)
2012
5
Management of insulinoma: Tunisians' experience. (22695091)
2012
6
Insulinoma-released exosomes or microparticles are immunostimulatory and can activate autoreactive T cells spontaneously developed in nonobese diabetic mice. (21734072)
2011
7
Pregnenolone sulfate activates basic region leucine zipper transcription factors in insulinoma cells: role of voltage-gated Ca2+ channels and transient receptor potential melastatin 3 channels. (21948387)
2011
8
Successful laparoscope resection of ectopic insulinoma in duodenohepatic ligament. (21358311)
2011
9
Metabolic acidosis as a complication of intravenous dextrose administration in a patient with insulinoma. (20972720)
2010
10
Clinical profile of insulinoma: analysis from a tertiary care referral center in India. (20953750)
2010
11
Multiple endocrine neoplasia type 1 deletion in pancreatic alpha-cells leads to development of insulinomas in mice. (20555035)
2010
12
Insulinoma and squamous cell carcinoma with peripheral polyneuropathy in an aged Sprague-Dawley rat. (21205453)
2010
13
Calcium influx into MIN6 insulinoma cells induces expression of Egr-1 involving extracellular signal-regulated protein kinase and the transcription factors Elk-1 and CREB. (18783846)
2009
14
Localization of insulinoma associated protein 2, IA-2 in mouse neuroendocrine tissues using two novel monoclonal antibodies. (19233214)
2009
15
A new luminescence assay for autoantibodies to mammalian cell-prepared insulinoma-associated protein 2. (18535195)
2008
16
Insulinoma and gastrinoma syndromes from a single intrapancreatic neuroendocrine tumor. (18252785)
2008
17
Re: "Islet hyperplasia in adults: challenge to preoperatively diagnose non-insulinoma pancreatogenic hypoglycemia syndrome". (17171485)
2007
18
Karyotype of the human insulinoma CM cell line--beta cell model in vitro? (17492774)
2007
19
Crystal structure of the major diabetes autoantigen insulinoma-associated protein 2 reveals distinctive immune epitopes. (17192463)
2007
20
Rapid intraoperative insulin assay: a novel method to differentiate insulinoma from nesidioblastosis in the pediatric patient. (17958895)
2007
21
The role of intra-arterial calcium stimulation test with hepatic venous sampling (IACS) in the management of occult insulinomas. (17431724)
2007
22
The insulinoma-associated 1: a novel promoter for targeted cancer gene therapy for small-cell lung cancer. (16052225)
2006
23
Islet hyperplasia in adults: challenge to preoperatively diagnose non-insulinoma pancreatogenic hypoglycemia syndrome. (16555022)
2006
24
Hypercalcitoninemia in a patient with a recurrent goitre and insulinoma: a case report. (16804794)
2006
25
Melatonin stimulates inositol-1,4,5-trisphosphate and Ca2+ release from INS1 insulinoma cells. (16150114)
2005
26
Effect of PK11195, a peripheral benzodiazepine receptor agonist, on insulinoma cell death and insulin secretion. (15909116)
2005
27
Malignant insulinoma in ectopic pancreatic tissue. (16432300)
2005
28
Insulinoma in Iran: a 20-year review. (16438457)
2005
29
The PDZ/coiled-coil domain containing protein PIST modulates insulin secretion in MIN6 insulinoma cells by interacting with somatostatin receptor subtype 5. (16263117)
2005
30
Insulin determination by specific and unspecific immunoassays in patients with insulinoma evaluated by the arterial stimulation and venous sampling test. (15248832)
2004
31
The diagnosis and treatment of insulinoma and gastrinoma]. (15045936)
2004
32
Locating an insulinoma by surgical exploration. (15524227)
2004
33
Abnormalities of proinsulin processing in functioning insulinomas: clinical implications. (15473873)
2004
34
Pancreatic beta-cell-specific ablation of the multiple endocrine neoplasia type 1 (MEN1) gene causes full penetrance of insulinoma development in mice. (12941803)
2003
35
Activation of nuclear factor-kappaB by depolarization and Ca(2+) influx in MIN6 insulinoma cells. (12475794)
2002
36
An insulinoma for which secretin test and selective arterial calcium injection test were useful. (12413006)
2002
37
A novel insulinoma tumor suppressor gene locus on chromosome 22q with potential prognostic implications. (11739439)
2001
38
Functional coupling between 'R-type' Ca2+ channels and insulin secretion in the insulinoma cell line INS-1. (11179973)
2001
39
Proteins spontaneously released by rat insulinoma (RIN) cells are anchored on cell membrane by a glycosyl-phosphatidyl-inositol link and inhibit increased RIN cell adhesion of lymphocytes from type 1 diabetic patients and non-obese diabetic mice in vitro. (8985653)
1996
40
Correlations of plasma concentrations of B-cell polypeptides with pathologic features of insulinomas. (15251555)
1996
41
Insulinoma cells in culture show pronounced sensitivity to alloxan-induced oxidative stress. (7672482)
1995
42
HLA-DR-restricted T cell lines from newly diagnosed type 1 diabetic patients specific for insulinoma and normal islet beta cell proteins: lack of reactivity to glutamic acid decarboxylase. (7554382)
1995
43
Intracellular degradation of the C-peptide of proinsulin, in a human insulinoma: identification of sites of cleavage and evidence for a role for cathepsin B. (7716141)
1995
44
Expression, intracellular localization, and gene transcription regulation of the secretory protein 7B2 in endocrine pancreatic cell lines and human insulinomas. (7517367)
1994
45
Detection of the human glucagon-like peptide 1(7-36) amide receptor on insulinoma-derived cell membranes. (8112494)
1994
46
GLUT2 surface expression and intracellular transport via the constitutive pathway in pancreatic beta cells and insulinoma: evidence for a block in trans-Golgi network exit by brefeldin A. (8276890)
1993
47
Control of plasma glucose with somatostatin analogue (SMS 201-995) during surgical removal of insulinomas. (8121865)
1993
48
Unresponsiveness of insulinoma cells to secretin: significance of the secretin test in patients with insulinoma. (2166285)
1990
49
Forced diuresis to reduce nephrotoxicity of streptozotocin in the treatment of advanced metastatic insulinoma. (3034373)
1987
50
Control of refractory fasting hypoglycemia in a patient with suspected insulinoma with diphenylhydantoin. (4358644)
1974

Variations for Insulinoma

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Expression for genes affiliated with Insulinoma

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Insulinoma

Search GEO for disease gene expression data for Insulinoma.

Pathways for genes affiliated with Insulinoma

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51PathCards, 13EMD Millipore, 56Reactome, 61Thomson Reuters, 54QIAGEN, 52PharmGKB, 39NCBI BioSystems Database, 31KEGG
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Pathways related to Insulinoma according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.6SLC2A2, IAPP
29.5IAPP, GIPR, SCT
3
Show member pathways
9.5SCT, GIPR, IAPP
49.0SLC2A2, GCK
5
Show member pathways
9.0SLC2A2, GCK
6
Show member pathways
8.7SCT, GIPR, IAPP, SSTR5, CASR
7
Show member pathways
7.9INSM1, PAX4, IAPP, GCK, SLC2A2
8
Show member pathways
7.9SLC2A2, GCK, IAPP, PAX4, INSM1

Compounds for genes affiliated with Insulinoma

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52PharmGKB, 46Novoseek, 12DrugBank, 62Tocris Bioscience, 30IUPHAR, 25HMDB
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Compounds related to Insulinoma according to GeneCards/GeneDecks:

(show all 33)
idCompoundScoreTop Affiliating Genes
1repaglinide52 46 1212.0PAX4, IAPP
2secretoneurin4610.0SCT, SCG2
3i-app469.7GCK, IAPP
4ibmx46 62 3011.5SCT, IAPP, SLC2A2
5norepinephrine46 25 1211.4IAPP, SCG2, SCT
6alloxan469.4SLC2A2, GCK
73-o-methylglucose469.4GCK, SLC2A2
8preproinsulin469.4GCK, SLC2A2
9ribonucleic acid469.4CASR, SSTR5, GIPR
10histamine46 30 2511.3SCT, SCG2, IAPP
11acth469.3SCT, GIPR, SSTR5, CASR
12cyclic amp46 2510.3CASR, IAPP, SCG2, SCT
13proglucagon469.3SLC2A2, GCK
14incretin469.2SLC2A2, GCK, IAPP
15katp469.2SLC2A2, GCK
16sulfonylurea469.2SLC2A2, GCK, IAPP
17dopamine46 30 25 1212.2SSTR5, IAPP, SCG2, SCT
18streptozotocin469.2IAPP, GCK, SLC2A2
19acetylcholine46 52 30 25 1213.1IAPP, SCG2, SCT
20fructose46 1210.0SLC2A2, GCK
21mannitol46 1210.0SLC2A2, GCK, SCT
22c-peptide468.8SCT, IAPP, GCK, SLC2A2
23h2o2468.7KCNA5, IAPP, GCK, SLC2A2
24hydrogen46 259.6SCT, CASR, GCK, SLC2A2
25adenylate468.6GCK, CASR, IAPP, SCG2, SCT
26oligonucleotide468.5SCT, IAPP, GCK, SLC2A2
27cysteine468.5SCT, SCG2, IAPP, CASR, GCK
28arginine468.4SLC2A2, GCK, CASR, IAPP, SCT
29potassium46 25 1210.4KCNA5, SCT, CASR, GCK, SLC2A2
30atp46 309.2SLC2A2, GCK, IAPP, SCG2, SCT
31forskolin46 52 1210.0SLC2A2, GCK, CASR, IAPP, SCG2, SCT
32glucose467.6SCT, SCG2, PAX4, IAPP, CASR, GCK
33calcium46 52 25 1210.5KCNA5, SCT, SCG2, IAPP, SSTR5, WFS1

GO Terms for genes affiliated with Insulinoma

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17Gene Ontology
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Biological processes related to Insulinoma according to GeneCards/GeneDecks:

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1regulation of potassium ion transportGO:0432669.5GCK, KCNA5
2positive regulation of cell differentiationGO:0455979.4INSM1, PAX4
3calcium ion importGO:0705099.3GCK, CASR
4hexose transportGO:0086459.0GCK, SLC2A2
5positive regulation of insulin secretionGO:0320249.0GIPR, GCK
6glucose homeostasisGO:0425938.9GCK, SSTR5, WFS1
7regulation of insulin secretionGO:0507968.4SLC2A2, GCK, SSTR5, KCNA5
8endocrine pancreas developmentGO:0310187.7SLC2A2, GCK, IAPP, GIPR, PAX4, INSM1

Products for genes affiliated with Insulinoma

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Sources for Insulinoma

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet