MEA
MCID: MLT074
MIFTS: 59

Multiple Endocrine Neoplasia (MEA) malady

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

Summaries for Multiple Endocrine Neoplasia

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22Genetics Home Reference, 66Wikipedia, 34MalaCards
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Genetics Home Reference:22 Multiple endocrine neoplasia is a group of disorders that affect the body's network of hormone-producing glands (the endocrine system). Hormones are chemical messengers that travel through the bloodstream and regulate the function of cells and tissues throughout the body. Multiple endocrine neoplasia typically involves tumors (neoplasia) in at least two endocrine glands; tumors can also develop in other organs and tissues. These growths can be noncancerous (benign) or cancerous (malignant). If the tumors become cancerous, the condition can be life-threatening.

MalaCards: Multiple Endocrine Neoplasia, also known as adenomatosis, familial endocrine, is related to multiple endocrine neoplasia type 2a and zollinger-ellison syndrome. An important gene associated with Multiple Endocrine Neoplasia is RET (ret proto-oncogene), and among its related pathways are G protein signaling H RAS regulation pathway and Signaling events regulated by Ret tyrosine kinase. The compounds 111in-pentetreotide and sunitinib have been mentioned in the context of this disorder. Affiliated tissues include thyroid, pituitary and testes, and related mouse phenotypes are limbs/digits/tail and hearing/vestibular/ear.

Wikipedia:66 The term multiple endocrine neoplasia (MEN) encompasses several distinct syndromes featuring tumors of... more...

Aliases & Classifications for Multiple Endocrine Neoplasia

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22Genetics Home Reference, 46Novoseek, 63UMLS
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Classifications:



Aliases & Descriptions:

multiple endocrine neoplasia 22 46 63
adenomatosis, familial endocrine 22
familial endocrine adenomatosis 22
multiple endocrine adenomatosis 22
endocrine neoplasia, multiple 22
multiple endocrine neoplasms 22
men 22
mea 22


Related Diseases for Multiple Endocrine Neoplasia

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18GeneCards, 19GeneDecks
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Diseases in the Multiple Endocrine Neoplasia Type 2a family:

Multiple Endocrine Neoplasia Type 4 multiple endocrine neoplasia

Diseases related to Multiple Endocrine Neoplasia via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 197)
idRelated DiseaseScoreTop Affiliating Genes
1multiple endocrine neoplasia type 2a31.7GDNF, SDHD, SDHC, NF1, CALCA, VHL
2zollinger-ellison syndrome31.4MEN1, CHGA
3pheochromocytoma31.4VHL, RET, SDHD
4gastrinoma31.4MEN1, CHGA
5familial medullary thyroid carcinoma31.3GDNF, RET, CALCA
6neuroendocrine tumor31.3CHGA, CALCA
7insulinoma31.2CALCA, MEN1, CHGA
8primary hyperparathyroidism31.2CHGA, PRKAR1A, RET, MEN1, CALCA
9hyperparathyroidism31.1RET, PRKAR1A, CHGA, SDHC, SDHD, MEN1
10hirschsprung's disease31.1GDNF, RET
11wermer syndrome31.1CHGA, PRKAR1A, MEN1, CDKN1B
12neuroma31.0GDNF, RET, CALCA
13glucagonoma30.9CHGA, MEN1
14papillary thyroid carcinoma30.9CALCA, MEN1, RET
15thyroid cancer30.9GNAS, RET, CALCA, CDKN1B
16phaeochromocytoma30.8SDHD, SDHC, CHGA, RET, CALCA
17cushing's syndrome30.8PRKAR1A
18prolactinoma30.8GNAS, CHGA, PRKAR1A, MEN1
19adenoma30.8GDNF, CDKN1B, CALCA, VHL, MEN1, RET
20acromegaly30.8MEN1, PRKAR1A, GNAS
21parathyroid carcinoma30.8RET, MEN1
22pituitary tumors30.8GNAS, CHGA, PRKAR1A, MEN1, CDKN1B
23von hippel-lindau disease30.7NF1, VHL, MEN1, RET, SDHC, SDHD
24ganglioneuroma30.7CHGA, RET
25neurofibromatosis30.7SDHD, NF1
26pituitary adenoma30.7MEN1, RET, PRKAR1A, CHGA, GNAS, GDNF
27neuroendocrine carcinoma30.6CHGA, MEN1, CALCA
28pancreatic islet cell tumors30.6CHGA, RET, MEN1, CALCA
29thyroid medullary carcinoma30.5CALCA, VHL, MEN1, RET, PRKAR1A, CHGA
30vipoma30.5MEN1, CHGA
31hypercalcemia30.5CHGA, PRKAR1A, MEN1, CALCA
32adrenal adenoma30.5MEN1, PRKAR1A, GNAS
33paraganglioma30.5NF1, CALCA, VHL, MEN1, RET, CHGA
34ependymoma30.5NF1, MEN1
35carney complex30.5GNAS, PRKAR1A, MEN1, CDKN1B
36lipomatosis30.4MEN1
37parathyroid adenoma30.3CDKN1B, CALCA, MEN1, RET, PRKAR1A, CHGA
38renal dysplasia30.3GDNF, RET
39somatostatinoma30.3CALCA, CHGA
40glomus tumor30.3VHL, SDHD
41peutz-jeghers syndrome30.3VHL, PRKAR1A
42carcinoid syndrome30.2GNAS, SDHD, CHGA, RET, MEN1, CALCA
43gastrointestinal stromal tumor30.2NF1, SDHC
44adenocarcinoma30.1CHGA, MEN1, VHL, CDKN1B
45thyroiditis11.1
46pancreatitis10.9
47wagenmann-froboese syndrome10.7
48amyloidosis10.7
49lichen amyloidosis10.7
50sipple syndrome10.6

Graphical network of the top 20 diseases related to Multiple Endocrine Neoplasia:



Diseases related to multiple endocrine neoplasia

Symptoms for Multiple Endocrine Neoplasia

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Drugs & Therapeutics for Multiple Endocrine Neoplasia

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

Search CenterWatch for Multiple Endocrine Neoplasia

Drug clinical trials:

Search ClinicalTrials for Multiple Endocrine Neoplasia

Search NIH Clinical Center for Multiple Endocrine Neoplasia

Search CenterWatch for Multiple Endocrine Neoplasia

Genetic Tests for Multiple Endocrine Neoplasia

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Anatomical Context for Multiple Endocrine Neoplasia

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34MalaCards
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MalaCards organs/tissues related to Multiple Endocrine Neoplasia:

34
Thyroid, Pituitary, Testes, Bone, Pancreas, Skin, Pancreatic islet, Prostate, Adrenal gland, Liver, Brain, Kidney, Colon, Tongue, Lung, Endothelial, Spinal cord, Ovary, Adipocyte, Breast, Lymph node, Thymus

Animal Models for Multiple Endocrine Neoplasia or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Multiple Endocrine Neoplasia:

38 (show all 24)
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053718.7NF1, VHL, RET, GNAS
2MP:00053778.6CDKN1B, NF1, CALCA, GNAS
3MP:00053818.5GDNF, PRKAR1A, RET, MEN1, VHL, CDKN1B
4MP:00053708.5GNAS, MEN1, VHL, NF1, CDKN1B
5MP:00053898.2GDNF, CHGA, PRKAR1A, RET, MEN1, VHL
6MP:00053828.1GNAS, PRKAR1A, MEN1, VHL, NF1, CDKN1B
7MP:00053888.0GNAS, PRKAR1A, RET, CALCA, NF1, CDKN1B
8MP:00053808.0SDHD, PRKAR1A, RET, MEN1, VHL, CALCA
9MP:00107718.0GNAS, VHL, CALCA, NF1, CDKN1B
10MP:00053908.0GNAS, PRKAR1A, VHL, CALCA, NF1
11MP:00020067.8SDHD, PRKAR1A, RET, MEN1, VHL, CALCA
12MP:00053977.6GDNF, GNAS, PRKAR1A, RET, VHL, NF1
13MP:00053677.5GDNF, GNAS, CHGA, RET, VHL, CALCA
14MP:00053697.5GDNF, GNAS, PRKAR1A, RET, MEN1, VHL
15MP:00028737.5GNAS, SDHD, PRKAR1A, RET, VHL, CALCA
16MP:00053877.4GDNF, GNAS, PRKAR1A, RET, MEN1, VHL
17MP:00053867.4GDNF, GNAS, SDHD, RET, CALCA, NF1
18MP:00036317.2GDNF, GNAS, SDHD, RET, MEN1, CALCA
19MP:00053846.8GDNF, GNAS, SDHD, PRKAR1A, RET, MEN1
20MP:00053766.5PRKAR1A, CHGA, SDHD, GNAS, RET, MEN1
21MP:00053796.5CHGA, SDHD, GNAS, GDNF, PRKAR1A, RET
22MP:00053786.4GDNF, GNAS, SDHD, CHGA, PRKAR1A, RET
23MP:00053856.1CDKN1B, GDNF, GNAS, SDHD, CHGA, PRKAR1A
24MP:00107685.6CHGA, SDHC, SDHD, GNAS, GDNF, PRKAR1A

Publications for Multiple Endocrine Neoplasia

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53PubMed
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Articles related to Multiple Endocrine Neoplasia:

(show top 50)    (show all 984)
idTitleAuthorsYear
1
Tanycytic ependymoma of the filum terminale associated with multiple endocrine neoplasia type 1: first reported case. (23562332)
2013
2
Multiple endocrine neoplasia: the enigma of MEN. (23880753)
2013
3
A novel germline mutation of the MEN1 gene caused multiple endocrine neoplasia type 1 in a Chinese young man and 1 year follow-up. (24302194)
2013
4
A novel menin gene deletional mutation in a little series of Italian patients affected by apparently sporadic multiple endocrine neoplasia type 1 syndrome. (22490989)
2012
5
Multiple endocrine neoplasia type 1 presenting as refractory epilepsy and polyneuropathy--a case report. (22138026)
2012
6
Novel association of thymic carcinoid with a germline mutation in a kindred with multiple endocrine neoplasia 1 (MEN1). (22549346)
2012
7
Coexistence of Multiple Endocrine Neoplasia Type 2B and Chilaiditi Sign: A Case Report. (23119190)
2012
8
Functioning glucagonoma associated with primary hyperparathyroidism: multiple endocrine neoplasia type 1 or incidental association? (23259638)
2012
9
Neural hyperplasia in maxillary bone of multiple endocrine neoplasia type 2B patient. (22099857)
2011
10
A new mutation in the menin gene causes the multiple endocrine neoplasia type 1 syndrome with adrenocortical carcinoma. (21069576)
2011
11
Intracranial ependymoma associated with multiple endocrine neoplasia type 1. (20142633)
2010
12
Pathogenicity of DNA variants and double mutations in multiple endocrine neoplasia type 2 and von Hippel-Lindau syndrome. (19906784)
2010
13
Multiple endocrine neoplasia type 1 deletion in pancreatic alpha-cells leads to development of insulinomas in mice. (20555035)
2010
14
Unusual features of medullary thyroid carcinoma in multiple endocrine neoplasia type 2A. (19332941)
2009
15
Multiple endocrine neoplasia type 2a and germ line C634G RET mutation diagnosed in an 80-year-old patient. (19201392)
2009
16
First description of parathyroid disease in multiple endocrine neoplasia 2A syndrome. (19034701)
2008
17
Early diagnosis of multiple endocrine neoplasia type 2B: a challenge for physicians. (19169500)
2008
18
Adrenal involvement in multiple endocrine neoplasia type 1: results of 7 years prospective screening. (17235589)
2007
19
The impact of clinical and genetic screenings on the management of the multiple endocrine neoplasia type 1. (17823710)
2007
20
Management of nonfunctioning pancreatic endocrine tumors in the context of multiple endocrine neoplasia type 1 syndrome. (17925918)
2007
21
Management of pancreatic endocrine tumors in multiple endocrine neoplasia type 1. (16680581)
2006
22
Clinical testing for multiple endocrine neoplasia type 1 in a DNA diagnostic laboratory. (15714081)
2005
23
Do all patients with RET mutations associated with multiple endocrine neoplasia type 2 require surgery? (16929368)
2005
24
Adrenal ganglioneuromas in children with multiple endocrine neoplasia type 2: a report of two cases. (15827098)
2005
25
Heterotropic cardiac calcification: a rare presentation of multiple endocrine neoplasia. (16324211)
2005
26
Menin missense mutants associated with multiple endocrine neoplasia type 1 are rapidly degraded via the ubiquitin-proteasome pathway. (15254225)
2004
27
Pancreatic beta-cell-specific ablation of the multiple endocrine neoplasia type 1 (MEN1) gene causes full penetrance of insulinoma development in mice. (12941803)
2003
28
Multiple endocrine neoplasia type 1 (MEN1) germline mutations in familial isolated primary hyperparathyroidism. (12699448)
2003
29
Frequent occurrence of an intron 4 mutation in multiple endocrine neoplasia type 1. (12050235)
2002
30
Multiple endocrine neoplasia. (11786689)
2001
31
A false-positive diagnosis of C-cell hyperplasia in a member of a family with multiple endocrine neoplasia type 2A and familial colonic polyposis. (9591014)
1998
32
Papillary thyroid carcinoma, parathyroid adenoma, and unexplained hypercalcitoninemia: an unusual presentation of multiple endocrine neoplasia type 2A? (9777749)
1998
33
The molecular genetics of multiple endocrine neoplasia type 2A and related syndromes. (9046972)
1997
34
Exclusion of the phosphoinositide-specific phospholipase C beta 3 (PLCB3) gene as a candidate for multiple endocrine neoplasia type 1. (9003510)
1997
35
Mutation of RET proto-oncogene in Japanese patients with multiple endocrine neoplasia type 2B and sporadic medullary thyroid carcinoma. (9447290)
1997
36
A family of multiple endocrine neoplasia type 2A: genetic analysis and clinical features. (8732448)
1996
37
Diagnosis and management of pheochromocytomas in patients with multiple endocrine neoplasia type 2-relevance of specific mutations in the RET proto-oncogene. (8810737)
1996
38
Prophylactic surgery for multiple endocrine neoplasia type IIa after genetic diagnosis: is parathyroid transplantation indicated? (8678956)
1996
39
Cutaneous macular amyloidosis associated with multiple endocrine neoplasia 2A. (8959910)
1996
40
Prolactinoma presenting in identical twins with multiple endocrine neoplasia type 1. (8846498)
1996
41
Multiple endocrine neoplasia]. (7752481)
1995
42
Rudolf-Virchow-Preis 1995. The role of RET proto-oncogene mutation analysis in the diagnosis of multiple endocrine neoplasia type 2 (MEN 2) gene carriers and in the discrimination of sporadic and familial medullary thyroid carcinomas and pheochromocytomas. (8600671)
1995
43
Candidate genes for multiple endocrine neoplasia type 1. (7673854)
1995
44
Germ line mutation in the RET proto-oncogene associated with familial multiple endocrine neoplasia type 2B: a case report. (7596047)
1995
45
A metastatic neuroendocrine anaplastic small cell tumor in a patient with multiple endocrine neoplasia type 1 syndrome. Assessment of disease status and response to doxorubicin, cyclophosphamide, etoposide chemotherapy through scintigraphic imaging with 111In-pentetreotide. (7922988)
1994
46
A cluster of CpG islands at D10S94, near the locus responsible for multiple endocrine neoplasia type 2A (MEN2A). (1351867)
1992
47
Influence of multiple endocrine neoplasia type 1 on gastric endocrine cells in patients with the Zollinger-Ellison syndrome. (1358767)
1992
48
Pathological features of multiple endocrine neoplasia type IIb in childhood. (1973536)
1990
49
Search for the gene for multiple endocrine neoplasia type 2A. (2281187)
1990
50
Progress toward resolving the possible linkage of multiple endocrine neoplasia type 2A to haptoglobin and group-specific loci: use of restriction fragment length polymorphisms extends exclusion region. (2873080)
1986

Variations for Multiple Endocrine Neoplasia

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Expression for genes affiliated with Multiple Endocrine Neoplasia

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

Search GEO for disease gene expression data for Multiple Endocrine Neoplasia.

Pathways for genes affiliated with Multiple Endocrine Neoplasia

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51PathCards, 61Thomson Reuters, 39NCBI BioSystems Database, 5Cell Signaling Technology, 13EMD Millipore, 56Reactome
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Pathways related to Multiple Endocrine Neoplasia according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.8GDNF, RET
29.8GDNF, RET
39.4VHL, PRKAR1A, RET
49.3GNAS, CALCA
59.3GNAS, CALCA
69.1GNAS, PRKAR1A
79.1PRKAR1A, GNAS
8
Show member pathways
8.8GNAS, PRKAR1A, CDKN1B
9
Show member pathways
Calcium Regulation in the Cardiac Cell39
8.8GNAS, CALCA, PRKAR1A
10
Show member pathways
8.2CDKN1B, CALCA, MEN1, PRKAR1A, GNAS

Compounds for genes affiliated with Multiple Endocrine Neoplasia

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

(show top 50)    (show all 55)
idCompoundScoreTop Affiliating Genes
1111in-pentetreotide4610.0CHGA, CALCA
2sunitinib46 52 1212.0VHL, RET
3cinacalcet46 30 1211.9PRKAR1A, CALCA
4fluoride469.7NF1, CALCA, CHGA
5Fumaric acid259.6SDHC, SDHD
61,25 dihydroxy vitamin d3469.6CDKN1B, CALCA, GDNF
7bromodeoxyuridine469.6GDNF, CHGA, CDKN1B
8methionine469.5CHGA, RET, NF1, CDKN1B
9mibg469.5SDHD, CHGA, CALCA
10l-amino acid469.5GDNF, CHGA, PRKAR1A
11paraffin469.5CDKN1B, NF1, RET, CHGA
12ly294002469.5CDKN1B, VHL, RET, GDNF
13fumarate46 1210.4SDHD, SDHC, VHL
14acth469.4GNAS, CHGA
15ubiquinone469.3SDHC, SDHD
16testosterone46 62 25 1212.3CDKN1B, CALCA, RET, CHGA
17vitamin d469.2CHGA, PRKAR1A, MEN1, CALCA
18octreotide46 62 30 1212.2CALCA, CHGA, GNAS
19iron-sulfur469.2SDHC, SDHD
20catecholamine469.1RET, CHGA, SDHD, GDNF
21phosphatidylinositol469.1GDNF, RET, VHL, NF1, CDKN1B
22isoproterenol46 1210.1CALCA, CHGA, GNAS
23succinic acid30 25 1211.0SDHC, SDHD
24guanine46 25 1211.0GNAS, CHGA, CALCA
25atp46 3010.0CDKN1B, NF1, RET, CHGA, GDNF
26succinate468.8SDHD, SDHC, RET, VHL, NF1
27iron46 259.8GDNF, SDHD, SDHC, VHL
28progesterone46 30 62 25 1212.8CDKN1B, NF1, CHGA, GNAS
29dexamethasone46 52 30 1211.8GDNF, CHGA, PRKAR1A, CDKN1B
30norepinephrine46 25 1210.8CALCA, CHGA, GNAS, GDNF
31alanine468.6GDNF, GNAS, RET, NF1, CDKN1B
32retinoic acid46 259.6GDNF, SDHC, CHGA, RET, NF1, CDKN1B
33estrogen468.4GDNF, CHGA, MEN1, VHL, CALCA, NF1
34lipid468.4GDNF, GNAS, CHGA, RET, NF1
35dopamine46 30 25 1211.2GDNF, GNAS, CHGA, RET, CALCA, NF1
36steroid468.2GNAS, SDHC, CHGA, PRKAR1A, NF1
37arginine468.2GNAS, CHGA, PRKAR1A, RET, NF1, CDKN1B
38nitric oxide46 25 1210.2GDNF, GNAS, CHGA, VHL, CALCA, CDKN1B
39cysteine468.0GDNF, GNAS, CHGA, PRKAR1A, RET, NF1
40glucose468.0GDNF, GNAS, CHGA, PRKAR1A, VHL, CDKN1B
41oligonucleotide468.0GDNF, GNAS, CHGA, RET, VHL, NF1
42glutamate468.0GDNF, GNAS, CHGA, PRKAR1A, CALCA, CDKN1B
43vegf468.0GDNF, SDHC, CHGA, RET, MEN1, VHL
44adenylate468.0GDNF, GNAS, CHGA, PRKAR1A, CALCA, NF1
45serine467.7GDNF, GNAS, CHGA, RET, MEN1, VHL
46forskolin46 52 129.7GDNF, GNAS, CHGA, PRKAR1A, CALCA, NF1
47tyrosine467.7GDNF, GNAS, CHGA, RET, MEN1, VHL
48oxygen46 258.5GDNF, GNAS, SDHD, SDHC, CHGA, VHL
49cyclic amp46 258.4CDKN1B, GDNF, GNAS, CHGA, PRKAR1A, RET
50calcium46 52 25 1210.1GDNF, GNAS, CHGA, PRKAR1A, RET, MEN1

GO Terms for genes affiliated with Multiple Endocrine Neoplasia

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17Gene Ontology
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Cellular components related to Multiple Endocrine Neoplasia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1protein complexGO:0432349.4CDKN1B, MEN1, PRKAR1A
2mitochondrial respiratory chain complex IIGO:0057499.0SDHC, SDHD

Biological processes related to Multiple Endocrine Neoplasia according to GeneCards/GeneDecks:

(show all 19)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of extrinsic apoptotic signaling pathway in absence of ligandGO:200124110.2RET, NF1
2negative regulation of cyclin-dependent protein serine/threonine kinase activityGO:04573610.2CDKN1B, MEN1
3response to painGO:04826510.1CALCA, RET
4positive regulation of adenylate cyclase activityGO:04576210.1NF1, CALCA
5enteric nervous system developmentGO:04848410.0GDNF, RET
6negative regulation of osteoclast differentiationGO:04567110.0CALCA, NF1
7MAPK cascadeGO:0001659.9NF1, MEN1, RET
8sympathetic nervous system developmentGO:0484859.9GDNF, NF1
9metanephros developmentGO:0016569.9NF1, GDNF
10neural crest cell migrationGO:0017559.8RET, GDNF
11positive regulation of Ras GTPase activityGO:0323209.6NF1, GNAS
12cognitionGO:0508909.5NF1, GNAS
13tricarboxylic acid cycleGO:0060999.5SDHC, SDHD
14activation of adenylate cyclase activityGO:0071909.4CALCA, GNAS
15positive regulation of cAMP biosynthetic processGO:0308199.3GNAS, CALCA
16cellular response to glucagon stimulusGO:0713779.2GNAS, PRKAR1A
17adenylate cyclase-activating G-protein coupled receptor signaling pathwayGO:0071899.2GNAS, CALCA
18response to drugGO:0424939.1CDKN1B, RET, GNAS
19water transportGO:0068338.8GNAS, PRKAR1A

Molecular functions related to Multiple Endocrine Neoplasia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1succinate dehydrogenase activityGO:0001049.0SDHC, SDHD
2protein bindingGO:0055157.2PRKAR1A, RET, MEN1, VHL, CALCA, NF1

Products for genes affiliated with Multiple Endocrine Neoplasia

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  • Antibodies
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Sources for Multiple Endocrine Neoplasia

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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