MCID: INS001
MIFTS: 57

Insulinoma malady

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

Summaries for Insulinoma

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Wikipedia:65 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 insulin-producing tumor of islet cells, is related to hypoglycemia and glucagonoma. 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:10 An adenoma that is a tumor in the pancreas that produces too much insulin.

Aliases & Classifications for Insulinoma

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Sources:
32LifeMap Discovery®, 10Disease Ontology, 43NIH Rare Diseases, 12DISEASES, 45Novoseek, 49Orphanet, 62UMLS, 57SNOMED-CT, 35MeSH, 40NCIt, 36MESH via Orphanet, 28ICD10 via Orphanet, 63UMLS via Orphanet
See all sources

Insulinoma, Aliases & Descriptions:

Name: Insulinoma 32 10 43 12 45 49 62
Insulin-Producing Tumor of Islet Cells 10 62
Islet Cell Adenoma 10 62
 
Adenoma Islet Cell 45
Insuloma 49


Classifications:



Characteristics (Orphanet epidemiological data):

49
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 Ontology10 DOID:3892
MeSH35 D007340
NCIt40 C3140
Orphanet49 97279
MESH via Orphanet36 D007340
ICD10 via Orphanet28 E16.8
UMLS via Orphanet63 C0021670

Related Diseases for Insulinoma

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

(show top 50)    (show all 89)
idRelated DiseaseScoreTop Affiliating Genes
1hypoglycemia31.0SCT, IAPP, GCK, SLC2A2
2glucagonoma30.7SSTR5, SCT
3gastrinoma30.5SCT, IAPP, SSTR5, CASR
4hyperinsulinism30.4GCK, IAPP, PAX4, SCT
5neuroendocrine tumor30.3INSM1, SCT, SCG2
6pheochromocytoma30.3SCT, SCG2, IAPP, SSTR5
7hyperparathyroidism30.2SCT, CASR
8primary hyperparathyroidism30.1CASR, SCT
9hypercalcemia30.1CASR, IAPP, SCT
10multiple endocrine neoplasia 129.9SCT, CASR
11pituitary adenoma29.9CASR, SSTR5, SCG2
12glucose intolerance29.9SLC2A2, GCK, IAPP
13adenocarcinoma29.8CASR, IAPP, SCG2, SCT
14adenoma29.8SCT, SCG2, GIPR, SSTR5, CASR
15hyperglycemia29.8SLC2A2, GCK, IAPP, PAX4, SCT
16pancreatitis10.9
17metastatic insulinoma10.7
18multiple endocrine neoplasia10.6
19hepatitis10.5
20obesity10.4
21duodenitis10.3
22hyperinsulinemic hypoglycemia10.3
23seizure disorder10.3
24fasting hypoglycemia10.2
25gastroduodenitis10.2
26hypokalemia10.2
27diarrhea10.2
28hereditary paraganglioma-pheochromocytoma syndromes10.2
29pancreatic adenoma10.2
30islet cell tumor10.2
31hyperproinsulinemia10.1
32pancreatic cancer10.1
33duodenal ulcer10.1
34acanthosis nigricans10.1
35zollinger-ellison syndrome10.1
36neuropathy10.1
37familial hypocalciuric hypercalcemia10.1
38adult-onset non-insulinoma persistent hyperinsulinemic hypoglycemia10.1
39osteomalacia10.0CASR
40primary hyperoxaluria10.0SCT, IAPP, CASR
41mody, type ii10.0GCK, IAPP
42maturity-onset diabetes of the young10.0GCK, PAX4
43medullary thyroid carcinoma, familial10.0IAPP, SSTR5, CASR
44neonatal diabetes mellitus10.0SLC2A2, GCK
45small cell cancer of the lung, somatic10.0INSM1, SCT, SCG2
46metabolic syndrome x10.0SLC2A2, GCK, IAPP
47carcinoid syndrome10.0SCT, SCG2, IAPP, SSTR5
48diabetes mellitus, noninsulin-dependent10.0GCK, IAPP, PAX4
49diabetes mellitus, insulin-dependent10.0SCT, PAX4, IAPP, WFS1, GCK
50rheumatoid arthritis9.9

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:

33
Pancreas, Testes, Liver, Pancreatic islet, T cells, Spleen, Pituitary, Brain, B cells, Bone, Lymph node, Retina, Lung, Hypothalamus, Appendix, 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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MGI Mouse Phenotypes related to Insulinoma:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053787.7IAPP, SSTR5, WFS1, CASR, GCK, SLC2A2
2MP:00053796.7SLC2A2, INSM1, PAX4, GIPR, SSTR5, WFS1
3MP:00053766.0KCNA5, INSM1, SCT, GIPR, IAPP, SSTR5

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/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.7SLC2A2, IAPP
2
Show member pathways
9.6SCT, GIPR, IAPP
39.6IAPP, GIPR, SCT
49.3SLC2A2, GCK
5
Show member pathways
9.3SLC2A2, GCK
6
Show member pathways
8.8SCT, 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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Compounds related to Insulinoma according to GeneCards/GeneDecks:

(show all 33)
idCompoundScoreTop Affiliating Genes
1repaglinide51 45 1312.1PAX4, IAPP
2secretoneurin4510.1SCT, SCG2
3i-app459.9GCK, IAPP
4alloxan459.7SLC2A2, GCK
53-o-methylglucose459.7GCK, SLC2A2
6preproinsulin459.7GCK, SLC2A2
7ibmx45 61 3011.7SCT, IAPP, SLC2A2
8proglucagon459.6SLC2A2, GCK
9incretin459.5SLC2A2, GCK, IAPP
10norepinephrine45 26 1311.5IAPP, SCG2, SCT
11sulfonylurea459.5SLC2A2, GCK, IAPP
12katp459.5SLC2A2, GCK
13streptozotocin459.5IAPP, GCK, SLC2A2
14ribonucleic acid459.5CASR, SSTR5, GIPR
15acth459.4SCT, GIPR, SSTR5, CASR
16histamine45 30 2611.4SCT, SCG2, IAPP
17cyclic amp45 2610.4CASR, IAPP, SCG2, SCT
18fructose45 1310.3SLC2A2, GCK
19dopamine45 30 26 1312.3SSTR5, IAPP, SCG2, SCT
20mannitol45 1310.3SLC2A2, GCK, SCT
21acetylcholine45 51 30 26 1313.2IAPP, SCG2, SCT
22c-peptide459.2SCT, IAPP, GCK, SLC2A2
23h2o2459.0KCNA5, IAPP, GCK, SLC2A2
24hydrogen45 2610.0SCT, CASR, GCK, SLC2A2
25adenylate458.9GCK, CASR, IAPP, SCG2, SCT
26oligonucleotide458.9SCT, IAPP, GCK, SLC2A2
27cysteine458.8SCT, SCG2, IAPP, CASR, GCK
28arginine458.8SLC2A2, GCK, CASR, IAPP, SCT
29potassium45 26 1310.8KCNA5, SCT, CASR, GCK, SLC2A2
30atp45 309.6SLC2A2, GCK, IAPP, SCG2, SCT
31forskolin45 51 1310.4SLC2A2, GCK, CASR, IAPP, SCG2, SCT
32glucose458.1SCT, SCG2, PAX4, IAPP, CASR, GCK
33calcium45 51 26 1310.9KCNA5, SCT, SCG2, IAPP, SSTR5, WFS1

GO Terms for genes affiliated with Insulinoma

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

Products for genes affiliated with Insulinoma

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Sources for Insulinoma

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4CDC
15ExPASy
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet