MCID: INS001
MIFTS: 49

Insulinoma malady

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

Aliases & Classifications for Insulinoma

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Sources:
30LifeMap Discovery®, 9Disease Ontology, 41NIH Rare Diseases, 11DISEASES, 43Novoseek, 47Orphanet, 60UMLS, 55SNOMED-CT, 33MeSH, 38NCIt, 34MESH via Orphanet, 26ICD10 via Orphanet, 61UMLS via Orphanet
See all sources

Insulinoma, Aliases & Descriptions:

Name: Insulinoma 30 9 41 11 43 47 60
Islet Cell Adenoma 9 60
Insulin-Producing Tumor of Islet Cells 9
 
Adenoma Islet Cell 43
Islet Cell Tumor 60
Insuloma 47


Classifications:



Characteristics (Orphanet epidemiological data):

47
insulinoma:
Inheritance: Autosomal dominant,Not applicable; Prevalence: 1-9/1000000 (Ireland),1-9/1000000 (Worldwide),1-9/1000000 (Japan); Age of onset: All ages


External Ids:

Disease Ontology9 DOID:3892
MeSH33 D007340
NCIt38 C3140
Orphanet47 97279
MESH via Orphanet34 D007340
ICD10 via Orphanet26 E16.8
UMLS via Orphanet61 C0021670

Summaries for Insulinoma

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Wikipedia:63 An insulinoma is a tumor of the pancreas that is derived from beta cells and secretes insulin. It is a... more...

MalaCards based summary: Insulinoma, also known as islet cell adenoma, is related to glucagonoma and hypoglycemia. An important gene associated with Insulinoma is IAPP (islet amyloid polypeptide), and among its related pathways are Selected targets of HNF1 and ADP signalling through P2Y purinoceptor 12. The compounds repaglinide and secretoneurin have been mentioned in the context of this disorder. Affiliated tissues include pancreas, testes and liver, 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.

Related Diseases for Insulinoma

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

(show top 50)    (show all 88)
idRelated DiseaseScoreTop Affiliating Genes
1glucagonoma30.9SSTR5, SCT
2hypoglycemia30.7IAPP, SCT, GCK, SLC2A2
3gastrinoma30.4CASR, IAPP, SCT, SSTR5
4neuroendocrine tumor30.4INSM1, SCT, SCG2
5primary hyperparathyroidism30.4SCT, CASR
6hyperparathyroidism30.4SCT, CASR
7hypercalcemia30.3SCT, CASR, IAPP
8hyperinsulinism30.3SCT, PAX4, GCK, IAPP
9multiple endocrine neoplasia 130.2CASR, SCT
10pheochromocytoma30.0IAPP, SCG2, SCT, SSTR5
11pituitary adenoma29.8SSTR5, CASR, SCG2
12glucose intolerance29.7IAPP, GCK, SLC2A2
13adenocarcinoma29.6SCG2, SCT, CASR, IAPP
14adenoma29.3SSTR5, GIPR, SCG2, SCT, CASR
15hyperglycemia29.2PAX4, SCT, IAPP, GCK, SLC2A2
16pancreatitis10.9
17metastatic insulinoma10.7
18multiple endocrine neoplasia10.6
19hepatitis10.5
20obesity10.4
21osteomalacia10.4CASR
22duodenitis10.3
23hyperinsulinemic hypoglycemia10.3
24seizure disorder10.3
25fasting hypoglycemia10.2
26gastroduodenitis10.2
27hypokalemia10.2
28diarrhea10.2
29pancreatic adenoma10.2
30islet cell tumor10.2
31adult-onset non-insulinoma persistent hyperinsulinemic hypoglycemia10.2
32primary hyperoxaluria10.1CASR, IAPP, SCT
33mody, type ii10.1GCK, IAPP
34maturity-onset diabetes of the young10.1GCK, PAX4
35medullary thyroid carcinoma, familial10.1CASR, IAPP, SSTR5
36hyperproinsulinemia10.1
37pancreatic cancer10.1
38duodenal ulcer10.1
39acanthosis nigricans10.1
40zollinger-ellison syndrome10.1
41neuropathy10.1
42familial hypocalciuric hypercalcemia10.1
43neonatal diabetes mellitus10.0GCK, SLC2A2
44small cell cancer of the lung, somatic10.0SCG2, INSM1, SCT
45rheumatoid arthritis9.9
46colorectal cancer9.9
47hepatocellular carcinoma9.9
48retinoblastoma9.9
49lung cancer9.9
50paroxysmal nonkinesigenic dyskinesia9.9

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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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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MalaCards organs/tissues related to Insulinoma:

31
Pancreas, Testes, Liver, Pancreatic islet, T cells, Spleen, Pituitary, Brain, B cells, Bone, Lymph node, Retina, Lung, Hypothalamus, Appendix, Temporal lobe

LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database

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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MGI Mouse Phenotypes related to Insulinoma:

35
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053787.7PAX4, IAPP, WFS1, CASR, GCK, SLC2A2
2MP:00053796.7SLC2A2, INSM1, PAX4, GIPR, SSTR5, WFS1
3MP:00053766.0CASR, WFS1, SSTR5, IAPP, GIPR, SCT

Publications for Insulinoma

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Articles related to Insulinoma:

(show top 50)    (show all 787)
idTitleAuthorsYear
1
mReg2 inhibits nuclear entry of apoptosis-inducing factor in mouse insulinoma cells. (25370781)
2014
2
Somatostatin analog inhibits the growth of insulinoma cells by p27-mediated G1 cell cycle arrest. (24694834)
2014
3
Insulinoma presenting with long-standing depression, primary hypogonadism, and sertoli cell only syndrome. (24455334)
2013
4
Canine insulinoma as a model for studying molecular genetics of tumorigenesis and metastasis. (23707578)
2013
5
An insulinoma presenting with hypochondriac delusions and food refusal. (23835036)
2013
6
Giant pancreatic insulinoma. The bigger the worse? Report of two cases and literature review. (23333851)
2013
7
Sphingosine kinase 1 knockdown reduces insulin synthesis and secretion in a rat insulinoma cell line. (22155656)
2012
8
Management of insulinoma: Tunisians' experience. (22695091)
2012
9
Insulinoma-released exosomes or microparticles are immunostimulatory and can activate autoreactive T cells spontaneously developed in nonobese diabetic mice. (21734072)
2011
10
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
11
Successful laparoscope resection of ectopic insulinoma in duodenohepatic ligament. (21358311)
2011
12
Metabolic acidosis as a complication of intravenous dextrose administration in a patient with insulinoma. (20972720)
2010
13
Clinical profile of insulinoma: analysis from a tertiary care referral center in India. (20953750)
2010
14
Multiple endocrine neoplasia type 1 deletion in pancreatic alpha-cells leads to development of insulinomas in mice. (20555035)
2010
15
Insulinoma and squamous cell carcinoma with peripheral polyneuropathy in an aged Sprague-Dawley rat. (21205453)
2010
16
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
17
Localization of insulinoma associated protein 2, IA-2 in mouse neuroendocrine tissues using two novel monoclonal antibodies. (19233214)
2009
18
A new luminescence assay for autoantibodies to mammalian cell-prepared insulinoma-associated protein 2. (18535195)
2008
19
Insulinoma and gastrinoma syndromes from a single intrapancreatic neuroendocrine tumor. (18252785)
2008
20
Re: "Islet hyperplasia in adults: challenge to preoperatively diagnose non-insulinoma pancreatogenic hypoglycemia syndrome". (17171485)
2007
21
Karyotype of the human insulinoma CM cell line--beta cell model in vitro? (17492774)
2007
22
Crystal structure of the major diabetes autoantigen insulinoma-associated protein 2 reveals distinctive immune epitopes. (17192463)
2007
23
Rapid intraoperative insulin assay: a novel method to differentiate insulinoma from nesidioblastosis in the pediatric patient. (17958895)
2007
24
The role of intra-arterial calcium stimulation test with hepatic venous sampling (IACS) in the management of occult insulinomas. (17431724)
2007
25
The insulinoma-associated 1: a novel promoter for targeted cancer gene therapy for small-cell lung cancer. (16052225)
2006
26
Islet hyperplasia in adults: challenge to preoperatively diagnose non-insulinoma pancreatogenic hypoglycemia syndrome. (16555022)
2006
27
Melatonin stimulates inositol-1,4,5-trisphosphate and Ca2+ release from INS1 insulinoma cells. (16150114)
2005
28
Effect of PK11195, a peripheral benzodiazepine receptor agonist, on insulinoma cell death and insulin secretion. (15909116)
2005
29
Malignant insulinoma in ectopic pancreatic tissue. (16432300)
2005
30
Insulinoma in Iran: a 20-year review. (16438457)
2005
31
The PDZ/coiled-coil domain containing protein PIST modulates insulin secretion in MIN6 insulinoma cells by interacting with somatostatin receptor subtype 5. (16263117)
2005
32
Insulin determination by specific and unspecific immunoassays in patients with insulinoma evaluated by the arterial stimulation and venous sampling test. (15248832)
2004
33
The diagnosis and treatment of insulinoma and gastrinoma]. (15045936)
2004
34
Locating an insulinoma by surgical exploration. (15524227)
2004
35
Pancreatic beta-cell-specific ablation of the multiple endocrine neoplasia type 1 (MEN1) gene causes full penetrance of insulinoma development in mice. (12941803)
2003
36
Activation of nuclear factor-kappaB by depolarization and Ca(2+) influx in MIN6 insulinoma cells. (12475794)
2002
37
An insulinoma for which secretin test and selective arterial calcium injection test were useful. (12413006)
2002
38
A novel insulinoma tumor suppressor gene locus on chromosome 22q with potential prognostic implications. (11739439)
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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Search GEO for disease gene expression data for Insulinoma.

Pathways for genes affiliated with Insulinoma

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Pathways related to Insulinoma according to GeneCards Suite gene sharing:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.6SCT, GIPR, IAPP
29.6GIPR, IAPP, SCT
39.3GCK, SLC2A2
4
Show member pathways
9.3SLC2A2, GCK
5
Show member pathways
8.8CASR, IAPP, GIPR, SCT, SSTR5
6
Show member pathways
7.9SLC2A2, INSM1, PAX4, IAPP, GCK
7
Show member pathways
7.9PAX4, INSM1, IAPP, GCK, SLC2A2

Compounds for genes affiliated with Insulinoma

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Compounds related to Insulinoma according to GeneCards Suite gene sharing:

(show all 33)
idCompoundScoreTop Affiliating Genes
1repaglinide49 43 1212.1PAX4, IAPP
2secretoneurin4310.1SCG2, SCT
3i-app439.9GCK, IAPP
4alloxan439.7SLC2A2, GCK
53-o-methylglucose439.7GCK, SLC2A2
6preproinsulin439.7SLC2A2, GCK
7ibmx43 59 2811.7IAPP, SLC2A2, SCT
8proglucagon439.6SLC2A2, GCK
9incretin439.5IAPP, GCK, SLC2A2
10norepinephrine43 24 1211.5IAPP, SCG2, SCT
11sulfonylurea439.5SLC2A2, GCK, IAPP
12katp439.5GCK, SLC2A2
13streptozotocin439.5SLC2A2, GCK, IAPP
14ribonucleic acid439.5SSTR5, GIPR, CASR
15acth439.4CASR, SSTR5, GIPR, SCT
16histamine43 28 2411.4SCT, SCG2, IAPP
17cyclic amp43 2410.4SCG2, IAPP, CASR, SCT
18fructose43 1210.3GCK, SLC2A2
19dopamine43 28 24 1212.3IAPP, SCG2, SCT, SSTR5
20mannitol43 1210.3SLC2A2, SCT, GCK
21acetylcholine43 49 28 24 1213.2IAPP, SCG2, SCT
22c-peptide439.2IAPP, GCK, SCT, SLC2A2
23h2o2439.0IAPP, GCK, SLC2A2, KCNA5
24hydrogen43 2410.0SCT, CASR, GCK, SLC2A2
25adenylate438.9IAPP, SCT, CASR, GCK, SCG2
26oligonucleotide438.9SLC2A2, GCK, SCT, IAPP
27cysteine438.8CASR, SCG2, IAPP, GCK, SCT
28arginine438.8CASR, IAPP, SCT, SLC2A2, GCK
29potassium43 24 1210.8SCT, CASR, KCNA5, GCK, SLC2A2
30atp43 289.6SLC2A2, GCK, IAPP, SCT, SCG2
31forskolin43 49 1210.4GCK, CASR, IAPP, SCT, SCG2, SLC2A2
32glucose438.1SCT, SCG2, PAX4, IAPP, CASR, GCK
33calcium43 49 24 1210.9KCNA5, SCG2, GCK, IAPP, SSTR5, WFS1

GO Terms for genes affiliated with Insulinoma

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

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1regulation of potassium ion transportGO:00432669.7GCK, KCNA5
2calcium ion importGO:00705099.6GCK, CASR
3hexose transportGO:00086459.3GCK, SLC2A2
4positive regulation of insulin secretionGO:00320249.2GIPR, GCK
5glucose homeostasisGO:00425939.2GCK, SSTR5, WFS1
6positive regulation of cell differentiationGO:00455979.2INSM1, PAX4
7regulation of insulin secretionGO:00507968.7SLC2A2, GCK, SSTR5, KCNA5
8endocrine pancreas developmentGO:00310187.8SLC2A2, GCK, IAPP, GIPR, PAX4, INSM1

Products for genes affiliated with Insulinoma

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Sources for Insulinoma

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet