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MCID: GLC036

Glucagonoma malady
27 genes, 5 tissues, 356 related diseases, 10 phenotypes, 23 articles, clinical trials.

Summaries for Glucagonoma

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44Wikipedia, 22MalaCards
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Wikipedia: A glucagonoma is a rare tumor of the alpha cells of the pancreas that results in up to a 1000-fold...44 more...

MalaCards: Glucagonoma, also known as weight loss, necrolytic migratory erythema (nme), diabetes, stomatitis, and diarrhea, is related to diabetic ketoacidosis and somatostatinoma. An important gene associated with Glucagonoma is MADD (MAP-kinase activating death domain), and among its related pathways are Synthesis, Secretion, and Inactivation of Glucagon-like Peptide-1 (GLP-1) and Class B/2 (Secretin family receptors). The compounds mibg and carbachol have been mentioned in the context of this disorder. Affiliated tissues include lymph node, pancreas and t cells, and related mouse phenotypes are endocrine/exocrine gland and adipose tissue.

Aliases & Descriptions for Glucagonoma

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30NIH Rare Diseases, 32Novoseek , 43UMLS
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glucagonoma 30 32 43
weight loss, necrolytic migratory erythema (nme), diabetes, stomatitis, and diarrhea 30
glucagonoma syndrome 30

Related Diseases for Glucagonoma

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13GeneCards, 14GeneDecks
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Diseases related to glucagonoma by text searches and GeneDecks gene sharing:

(show top 50)    (show all 353)
idRelated DiseaseScoreTop Affiliating Genes
1diabetic ketoacidosis27.4INS, CALCA, SST
2somatostatinoma25.8GCG, VIP, CHGA, MEN1, ENO2, CALCA
3anemia25.2SCT, CHGA, INS, MEN1, GH1, GHRH
4insulinoma20.6PCSK2, PCSK1, GCG, VIP, SCG5, SCT
5diabetes mellitus20.3PCSK1, GCG, SCT, CHGA, INS, INSR
6pancreatitis18.1PCSK2, PCSK1, GCG, VIP, SCG5, SCT
7somatostatin analog, resistance to13.6SST, SSTR5
8intestinal neuroendocrine tumor13.6CHGA, SST
9middle ear cancer13.5CHGA, ENO2
10gastric small cell carcinoma13.5CHGA, ENO2
11duodenal gastrinoma13.4SCT, MEN1, GAST
12supratentorial neoplasm13.4CHGA, ENO2
13female breast carcinoma13.4CHGB, CHGA, ENO2
14central neurocytoma13.4CHGA, ENO2, GHRH
15pancreatic gastrinoma13.4PDX1, GAST
16pancreatic islet cell tumors13.4SST, GAST
17duodenal ulcer13.4SCT, GAST
18duodenum cancer13.4MEN1, SST, GAST
19familial isolated hyperparathyroidism13.4MEN1, GAST
20merkel cell carcinoma13.4CHGA, ENO2, SST
21postcholecystectomy syndrome13.3CCK, GAST
22duodenogastric reflux13.3SCT, CCK, GAST
23somatotrophinoma13.3MEN1, GH1, GHRH
24esthesioneuroblastoma13.3ENO2, SST, SSTR2
25pancreatic cholera13.3VIP, SST, GAST
26pituitary carcinoma13.3CHGA, GHRH, SST, SSTR2
27carney complex13.3CHGA, MEN1, GH1
28cushing's syndrome13.3SCG5, GH1, SST
29gastritis13.3SCT, GHRL, GAST
30neurofibromatosis13.3MEN1, ENO2, SST
31adrenocorticotropic hormone deficiency13.3PCSK1, GHRH
32pituitary-dependent cushing's disease13.2GH1, SST
33mccune albright syndrome13.2MEN1, GH1, SST
34steatorrhea13.2GCG, SCT, CCK, SST
35parathyroid carcinoma13.2SCT, MEN1, CALCA
36acinar cell carcinoma13.2CHGA, GAST
37oncogenic osteomalacia13.2CALCA, SSTR2, SSTR5
38ganglioneuroblastoma13.2VIP, CHGA, ENO2, SST
39pancreatic agenesis13.2INS, PDX1
40ganglioneuroma13.2VIP, CHGA, ENO2, SST
41hereditary pancreatitis13.2GCG, SCT, CCK, SST
42wdha syndrome13.2VIP, CALCA, SST
43gastrointestinal neuroendocrine tumor13.1CHGA, ENO2, GHRL, SST
44achalasia13.1VIP, ENO2, GAST
45acoustic neuroma13.1CCK, SST, GAST
46functioning pituitary adenoma13.1GH1, SST, SSTR2, SSTR5
47constipation13.1VIP, CHGA, CCK, SST
48apudoma13.1CALCA, SST, GAST
49permanent neonatal diabetes mellitus13.1INS, PDX1
50alcoholism13.1SCT, CCK, GHRH, GHRL

Graphical network of the top 20 diseases related to glucagonoma:



Graphical network of diseases related to glucagonoma

Clinical Features for Glucagonoma

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Drugs & Therapeutics for Glucagonoma

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4CenterWatch, 29NIH Clinical Center, 5ClinicalTrials, 43UMLS, 28NDF-RT
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Glucagonoma

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Anatomical Context for Glucagonoma

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22MalaCards
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MalaCards organs/tissues related to glucagonoma:

22
Lymph node, Pancreas, T cells, B cells, Pancreatic islet

Phenotypes for genes affiliated with Glucagonoma

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25MGI
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MGI Mouse Phenotypes related to glucagonoma:

25
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1endocrine/exocrine gland phenotypeMP:00053799.7PCSK2, MEN1, GHRH, SCG5, PCSK1
2adipose tissue phenotypeMP:00053758.5GHRL, GH1, INSR, INS, SCG5, PCSK1
3reproductive system phenotypeMP:00053898.5PCSK1, PCSK2, MEN1, ADCY10, GHRH, GH1
4nervous system phenotypeMP:00036318.2SSTR2, GHRH, CCK, MADD, MEN1, INS
5digestive/alimentary phenotypeMP:00053817.9GAST, PDX1, SSTR2, SST, GHRL, INS
6growth/size phenotypeMP:00053787.6SCG5, PDX1, SSTR5, SST, GHRH, GH1
7liver/biliary system phenotypeMP:00053707.5INS, SCG5, INSR, MEN1, GH1, GHRH
8behavior/neurological phenotypeMP:00053867.0PCSK2, SST, PCSK1, VIP, SCG5, SCT
9cardiovascular system phenotypeMP:00053856.4PCSK2, ADCY10, SSTR2, CALCA, MEN1, INSR
10homeostasis/metabolism phenotypeMP:00053763.9SCG5, ADCY10, PDX1, MEN1, SSTR5, CALCA

Publications for genes affiliated with Glucagonoma

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35PubMed
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Articles related to glucagonoma:

(show all 23)
idTitleAuthorsYearAffiliating Genes
1Glucagonoma without glucagonoma syndrome]. (20499510)ColoviA8 R.... LatinciA8 S.2010SST
2Glucagonoma diagnosed by arterial stimulation and ven ous sampling (ASVS). (19525592)Okauchi Y.... Shimomura I.2009MEN1
3Malignant glucagonoma. New options of treatment. (16761630)Appetecchia M.... Terzoli E.2006CHGA
4Malignant glucagonoma of the pancreas diagnoses throu gh anemia and diabetes mellitus. (12918465)Koike N.... Iwamoto Y.2003EPO
5Ghrelin expression in islet cell tumors: augmented ex pression of ghrelin in a case of glucagonoma with multiple endocrine neoplasm t ype I. (12414844)Iwakura H.... Nakao K.2002GHRL
6Immunohistochemical assessment of an asymptomatic glucagonoma in a patient with hypergastrinemia and marked antral angiodysplasia. (11277423)Weitgasser R.... Hacker G.W.2001SCT
7Glucagonoma--somatostatinoma (10444813)Schusdziarra V.1999SST
8In vivo and in vitro effects of somatostatin and insulin on glucagon release in a human glucagonoma. (9231061)Waeber G.... Haefliger J.A.1997SST
9Calcium reverses octreotide inhibition of insulin and glucagon levels in patients with insulinoma and glucagonoma. (8813473)Thompson C.S.... Woltering E.A.1996SST, INS, SCT
10Glucagonoma: case report and literature review (9201330)das Neves M.M.... Herani Filho A.K.1996SST
11The glucagonoma syndrome. Clinical and pathologic features in 21 patients. (8606627)Wermers R.A.... Lloyd R.V.1996CALCA, VIP, GAST
12Case report: diabetic ketoacidosis in a patient with glucagonoma. (7771503)Anthony L.B.... May M.E.1995SST
13Functional characterization of somatostatin receptors expressed on hamster glucagonoma cells. (7840180)Fehmann H.C.... Goke B.1995SST, ADCY10
14The long-acting somatostatin analogue octreotide alleviates symptoms by reducing posttranslational conversion of prepro-glucagon to glucagon in a patient with malignant glucagonoma, but does not prevent tumor growth. (8186658)JockenhAPvel F.... Reinwein D.1994SST, CHGA
15Molecular and functional characterization of insulin receptors present on hamster glucagonoma cells. (7520401)Fehmann H.C.... GAPke B.1994INSR
16An immunohistochemical study of glucagonoma conducted on the metastatic lymph nodes from a patient with recurrent metastatic glucagonoma: report of a case. (7894192)Kawakita N.... Urakawa T.1994DYT10
17Chromogranin-B, a putative precursor of eight novel rat glucagonoma peptides through processing at mono-, di-, or tribasic residues. (1954895)Nielsen E.... Madsen O.D.1991CHGB
18Glucagonomas of transgenic mice express a wide range of general neuroendocrine markers and bioactive peptides. (1678563)Rindi G.... Polak J.M.1991GCG, CHGA, SCG5
19Treatment of necrolytic migratory erythema in glucagonoma syndrome. (1761771)Shepherd M.E.... Smith E.B.1991SST
20Protein HMG-17 is hyper-expressed in rat glucagonoma. Single-step isolation and sequencing. (2169420)Nielsen E.... Madsen O.D.1990HMGN2
21Somatostatin analogue in treatment of coexisting glucagonoma and pancreatic pseudocyst: dissociation of responses. (2168587)Moattari A.R.... Vinik A.I.1990SST
22Somatostatin analogue and tissue cultures in the study of a human malignant glucagonoma. (2164620)Ahlman H.... Theodorsson E.1990SST
23Isolation of human pancreastatin fragment containing the active sequence from a glucagonoma. (2830133)Sekiya K.... Bloom S.R.1988CHGA

Expression for genes affiliated with Glucagonoma

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1BioGPS
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Expression patterns in normal tissues for genes affiliated with Glucagonoma

Pathways for genes affiliated with Glucagonoma

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38Reactome, 34PharmGKB, 10EMD Millipore, 20KEGG, 3Cell Signaling Technology
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Pathways related to glucagonoma according to GeneDecks:

idPathwayScoreTop Affiliating Genes
1Synthesis, Secretion, and Inactivation of Glucagon-like Peptide-1 (GLP-1)3810.0PCSK1, GCG
2Class B/2 (Secretin family receptors)389.6GHRH, CALCA, SCT
3IRS activation389.5INSR, INS
4Anti-diabetic Drug Potassium Channel Inhibitors Pathway, Pharmacodynamics349.4INS, PDX1, INSR
5Selected targets of CREB1109.2PCSK1, CCK, INS, CHGA, GCG
6Type II diabetes mellitus209.1PDX1, INS, INSR
7Glucose / Energy Metabolism38.9ENO2, INSR, INS, GCG, PDX1

Compounds for genes affiliated with Glucagonoma

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32Novoseek , 9DrugBank, 34PharmGKB, 18HMDB, 42Tocris Bioscience
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Compounds related to glucagonoma according to GeneDecks:

(show top 50)    (show all 220)
idCompoundScoreTop Affiliating Genes
1mibg32 9.9ENO2
2carbachol32 9 9 11.7GCG, SCT, CCK, SST, GAST
3proglucagon32 9.5PDX1, GCG, CHGA, SCG5, PCSK1, PCSK2
4lanreotide32 9.5SST, SSTR2, GHRH, GH1, SSTR5, CHGA
5111in-pentetreotide32 9.5SSTR5, SSTR2, SST, CALCA, CHGA
6peptidylglycine32 9.4PCSK2, PCSK1, CHGB, CHGA, GAST
7propranolol32 34 9 18 9 13.4VIP, CHGA, CALCA, GHRH, SCT, GAST
8glucose32 9.4PCSK2, PCSK1, GCG, GAST, PDX1, SSTR2
9gnrh32 9.1PCSK1, INS, GH1, GHRL, GHRH, GCG
10preproinsulin32 9.1INS, INSR, PDX1, PCSK1, PCSK2
11acipimox32 9.0GCG, INS, GH1, GHRL, SST, GHRH
12c-peptide32 8.9CCK, GHRL, PDX1, INSR, SCT, PCSK2
13i-app32 8.8GCG, INS, CALCA, GAST, PDX1, PCSK1
14pramlintide32 9 9 10.8CALCA, GHRL, CCK, INS, GCG
15naloxone32 34 9 9 11.6CCK, GH1, GHRH, GHRL, SST, ADCY10
16oxyntomodulin32 42 9.6PCSK2, PCSK1, GCG, VIP, SCT, CHGA
17clonidine32 9 9 10.6ADCY10, SST, GH1, CALCA, CHGA, SCT
18ribonucleic acid32 8.5PCSK2, PCSK1, CHGA, GH1, GHRH, GHRL
19pirenzepine32 9 9 10.4GCG, CCK, GH1, GHRH, GHRL, SST
20isoproterenol32 9 9 10.4ADCY10, CHGA, GH1, SST, GAST, SCT
21nifedipine32 9 9 10.3GAST, GHRH, GHRL, SST, DYT10, GH1
22gaba32 42 9.2VIP, ENO2, INSR, CHGA, SCT, GHRL
23bromocriptine32 9 9 10.1GH1, GHRH, VIP, ADCY10, EPO, SST
24guanine32 9 18 9 10.9SSTR2, GCG, VIP, SCT, CHGA, ADCY10
25epinephrine32 9 18 9 10.9GCG, VIP, SCT, CHGA, INS, INSR
26acth32 7.8GHRH, CCK, INS, SCT, SCG5, VIP
27thyroxine32 18 8.7GHRH, SST, GHRL, GH1, CALCA, INSR
285-hydroxytryptamine32 7.7GAST, VIP, GCG, ENO2, CHGA, CCK
29atropine32 9 9 9.6GHRL, SST, ADCY10, GAST, GCG, VIP
30dopamine32 9 18 9 10.4ADCY10, SSTR5, SSTR2, SST, GHRL, GHRH
31calcium32 9 18 9 10.4PCSK2, CHGB, SCT, SCG5, MEN1, VIP
32vitamin d32 7.4GHRL, EPO, CALCA, MEN1, INSR, CHGA
33testosterone32 9 18 9 10.4GAST, EPO, SST, CALCA, CCK, ENO2
34glutamate32 7.3GHRH, DYT10, SST, ADCY10, INSR, INS
35hydrocortisone32 9 9 9.3SST, INS, GHRL, GAST, GH1, CALCA
36arginine32 7.3VIP, GCG, PCSK1, PCSK2, GH1, SST
37histamine32 18 8.1CALCA, CCK, CHGA, SCT, SCG5, VIP
38thymidine32 18 8.0SCT, INSR, VIP, ENO2, GH1, GAST
39acetylcholine32 9 18 9 9.9GAST, ADCY10, SST, GHRL, GH1, CALCA
40lactate32 6.7SST, CHGA, VIP, GCG, INS, ENO2
41estrogen32 6.6CHGA, INSR, CHGB, VIP, GCG, MEN1
42forskolin32 42 9 9 9.5CCK, ENO2, CHGA, SCT, VIP, GCG
43dexamethasone32 42 34 9 9 10.5SCG5, VIP, PCSK2, CHGB, CHGA, GAST
44norepinephrine32 9 18 9 9.4GAST, ADCY10, SST, GHRL, GH1, CALCA
45serine32 6.3INSR, MEN1, GH1, GHRL, DYT10, PDX1
46adenylate32 6.2GAST, ADCY10, SSTR2, SST, DYT10, GHRL
47ibmx32 6.1PCSK1, GCG, VIP, SCT, INS, INSR
48vegf32 5.9PDX1, EPO, SSTR2, GHRL, GAST, DYT10
49tyrosine32 5.3GHRL, SCT, VIP, ENO2, SCG5, PCSK2
50octreotide32 42 9 9 8.0CCK, GCG, VIP, SCT, CHGA, INS

GO Terms for genes affiliated with Glucagonoma

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12Gene Ontology
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Cellular components related to glucagonoma according to GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1perikaryonGO:0432049.7ENO2, CCK, GHRH
2terminal buttonGO:0431959.3GHRH, CALCA, CCK
3secretory granuleGO:0301418.5CHGA, PCSK2, PCSK1, GCG, SCG5, CHGB
4extracellular spaceGO:0056156.6EPO, SST, GHRL, PCSK2, PCSK1, GCG
5extracellular regionGO:0055766.0EPO, GAST, GCG, SST, GHRL, VIP

Biological processes related to glucagonoma according to GeneDecks:

(show all 17)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of circadian sleep/wake cycle, non-REM sleepGO:04601010.2GHRL, GHRH
2somatostatin signaling pathwayGO:03817010.2SSTR2, SSTR5
3negative regulation of appetiteGO:03209910.1GCG, CCK
4positive regulation of growth hormone secretionGO:06012410.0GHRH, GHRL
5positive regulation of multicellular organism growthGO:0400189.9GHRH, GHRL, GH1
6positive regulation of cAMP biosynthetic processGO:0308199.9GCG, CALCA, GHRH
7exocrine pancreas developmentGO:0310179.8INSR, PDX1
8positive regulation of respiratory burstGO:0602679.7INSR, INS
9growth hormone secretionGO:0302529.7GHRH, GHRL
10regulation of insulin secretionGO:0507969.6GCG, INS, SSTR5, PCSK1
11negative regulation of cell proliferationGO:0082859.5MEN1, SST, SSTR2, SSTR5, PDX1
12glucose homeostasisGO:0425939.5SSTR5, INSR, INS, PDX1
13glucose metabolic processGO:0060069.4INS, ENO2, PDX1, GHRL
14cell-cell signalingGO:0072679.0INS, CALCA, SSTR2, SST, GHRH, PCSK1
15positive regulation of DNA replicationGO:0457408.9EPO, INSR, INS
16G-protein coupled receptor signaling pathwayGO:0071868.4INSR, GAST, SSTR5, GCG, VIP, INS
17positive regulation of cell proliferationGO:0082848.2VIP, INS, INSR, CCK, GHRH, EPO

Molecular functions related to glucagonoma according to GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1somatostatin receptor activityGO:00499410.1SSTR2, SSTR5
2growth hormone-releasing hormone activityGO:0166089.9GHRL, GHRH
3insulin bindingGO:0435599.6PCSK1, INSR
4hormone activityGO:0051796.8CALCA, GAST, EPO, SST, GH1, CCK

Sources for Glucagonoma

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2CDC
11FMA
18HMDB
19ICD9CM
20KEGG
24MeSH
25MGI
27NCIt
28NDF-RT
31NINDS
32Novoseek
33OMIM
35PubMed
36QIAGEN
43UMLS
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