MCID: INS001
MIFTS: 59

Insulinoma malady

Rare diseases, Gastrointestinal diseases, Endocrine diseases, Cancer diseases categories
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Summaries for Insulinoma

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8Disease Ontology, 65Wikipedia, 33MalaCards
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Wikipedia:65 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:8 An adenoma that is a tumor in the pancreas that produces too much insulin.

Aliases & Classifications for Insulinoma

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8Disease Ontology, 43NIH Rare Diseases, 47OMIM, 10DISEASES, 45Novoseek, 49Orphanet, 62UMLS, 58SNOMED-CT, 40NCIt, 35MeSH, 26ICD10 via Orphanet, 36MESH via Orphanet, 59SNOMED-CT via Orphanet, 63UMLS via Orphanet
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Classifications:



Aliases & Descriptions:

insulinoma 8 43 47 10 45 49 62
islet cell adenoma 8 62
insulin-producing tumor of islet cells 8
adenoma islet cell 45
insulinoma nos 8
insuloma 49


External Ids:

Disease Ontology8 DOID:3892
NCIt40 C3140
MeSH35 D007340
ICD10 via Orphanet26 E16.8
MESH via Orphanet36 D007340
SNOMED-CT via Orphanet59 25324008, 302822000
UMLS via Orphanet63 C0021670

Related Diseases for Insulinoma

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17GeneCards, 18GeneDecks
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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:
42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Drug clinical trials:

Search ClinicalTrials for Insulinoma

Search NIH Clinical Center for Insulinoma

Genetic Tests for Insulinoma

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Anatomical Context for Insulinoma

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31LifeMap Discovery™, 33MalaCards
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MalaCards organs/tissues related to Insulinoma:

33
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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37MGI
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MGI Mouse Phenotypes related to Insulinoma:

37
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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52PubMed
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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, 15Gene 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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50PathCards, 12EMD Millipore, 55Reactome, 60Thomson Reuters, 53QIAGEN, 51PharmGKB, 38NCBI BioSystems Database, 30KEGG
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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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51PharmGKB, 45Novoseek, 11DrugBank, 61Tocris Bioscience, 29IUPHAR, 24HMDB
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Compounds related to Insulinoma according to GeneCards/GeneDecks:

(show all 33)
idCompoundScoreTop Affiliating Genes
1repaglinide51 45 1112.0PAX4, IAPP
2secretoneurin4510.0SCT, SCG2
3i-app459.7GCK, IAPP
4ibmx45 61 2911.5SCT, IAPP, SLC2A2
5norepinephrine45 24 1111.4IAPP, SCG2, SCT
6alloxan459.4SLC2A2, GCK
73-o-methylglucose459.4GCK, SLC2A2
8preproinsulin459.4GCK, SLC2A2
9ribonucleic acid459.4CASR, SSTR5, GIPR
10histamine45 29 2411.3SCT, SCG2, IAPP
11acth459.3SCT, GIPR, SSTR5, CASR
12cyclic amp45 2410.3CASR, IAPP, SCG2, SCT
13proglucagon459.3SLC2A2, GCK
14incretin459.2SLC2A2, GCK, IAPP
15katp459.2SLC2A2, GCK
16sulfonylurea459.2SLC2A2, GCK, IAPP
17dopamine45 29 24 1112.2SSTR5, IAPP, SCG2, SCT
18streptozotocin459.2IAPP, GCK, SLC2A2
19acetylcholine45 51 29 24 1113.1IAPP, SCG2, SCT
20fructose45 1110.0SLC2A2, GCK
21mannitol45 1110.0SLC2A2, GCK, SCT
22c-peptide458.8SCT, IAPP, GCK, SLC2A2
23h2o2458.7KCNA5, IAPP, GCK, SLC2A2
24hydrogen45 249.6SCT, CASR, GCK, SLC2A2
25adenylate458.6GCK, CASR, IAPP, SCG2, SCT
26oligonucleotide458.5SCT, IAPP, GCK, SLC2A2
27cysteine458.5SCT, SCG2, IAPP, CASR, GCK
28arginine458.4SLC2A2, GCK, CASR, IAPP, SCT
29potassium45 24 1110.4KCNA5, SCT, CASR, GCK, SLC2A2
30atp45 299.2SLC2A2, GCK, IAPP, SCG2, SCT
31forskolin45 51 1110.0SLC2A2, GCK, CASR, IAPP, SCG2, SCT
32glucose457.6SCT, SCG2, PAX4, IAPP, CASR, GCK
33calcium45 51 24 1110.5KCNA5, SCT, SCG2, IAPP, SSTR5, WFS1

GO Terms for genes affiliated with Insulinoma

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16Gene 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
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet