MCID: MLT074
MIFTS: 61

Multiple Endocrine Neoplasia malady

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

Summaries for Multiple Endocrine Neoplasia

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Genetics Home Reference:23 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 based summary: Multiple Endocrine Neoplasia, also known as adenomatosis, familial endocrine, is related to pheochromocytoma 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:65 The term multiple endocrine neoplasia (MEN) encompasses several distinct syndromes featuring tumors of... more...

Aliases & Classifications for Multiple Endocrine Neoplasia

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Multiple Endocrine Neoplasia, Aliases & Descriptions:

Name: Multiple Endocrine Neoplasia 23 45 62
Adenomatosis, Familial Endocrine 23 62
Endocrine Adenomatosis, Multiple 62
Familial Endocrine Adenomatosis 23
Multiple Endocrine Adenomatosis 23
 
Endocrine Neoplasia, Multiple 23
Multiple Endocrine Neoplasms 23
Mea 23
Men 23


Classifications:



Related Diseases for Multiple Endocrine Neoplasia

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Diseases in the Multiple Endocrine Neoplasia family:

Multiple Endocrine Neoplasia, Type Iv Multiple Endocrine Neoplasia Iia
Multiple Endocrine Neoplasia Iib Multiple Endocrine Neoplasia 1

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

(show top 50)    (show all 187)
idRelated DiseaseScoreTop Affiliating Genes
1pheochromocytoma31.5VHL, RET, SDHD
2zollinger-ellison syndrome31.4MEN1, CHGA
3gastrinoma31.3MEN1, CHGA
4hyperparathyroidism31.3RET, PRKAR1A, CHGA, SDHC, SDHD, MEN1
5neuroendocrine tumor31.3CHGA, CALCA
6primary hyperparathyroidism31.3CHGA, PRKAR1A, RET, MEN1, CALCA
7multiple endocrine neoplasia 131.3CHGA, PRKAR1A, MEN1, CDKN1B
8insulinoma31.2CALCA, MEN1, CHGA
9neuroma31.0GDNF, RET, CALCA
10multiple endocrine neoplasia iia31.0GDNF, SDHD, SDHC, NF1, CALCA, VHL
11adenoma31.0GDNF, CDKN1B, CALCA, VHL, MEN1, RET
12thyroid cancer31.0GNAS, RET, CALCA, CDKN1B
13glucagonoma30.9CHGA, MEN1
14phaeochromocytoma30.9SDHD, SDHC, CHGA, RET, CALCA
15parathyroid carcinoma30.9RET, MEN1
16pituitary tumors30.9GNAS, CHGA, PRKAR1A, MEN1, CDKN1B
17prolactinoma30.8GNAS, CHGA, PRKAR1A, MEN1
18pituitary adenoma30.8MEN1, RET, PRKAR1A, CHGA, GNAS, GDNF
19acromegaly30.8MEN1, PRKAR1A, GNAS
20cushing's syndrome30.8PRKAR1A
21islet cell tumor30.7CHGA, RET, MEN1, CALCA
22von hippel-lindau syndrome30.7NF1, VHL, MEN1, RET, SDHC, SDHD
23ganglioneuroma30.7CHGA, RET
24paraganglioma30.7NF1, CALCA, VHL, MEN1, RET, CHGA
25neurofibromatosis30.6SDHD, SDHC, RET, VHL, NF1
26ependymoma30.6NF1, MEN1
27medullary thyroid carcinoma, familial30.6GDNF, RET, CALCA
28hypercalcemia30.6CHGA, PRKAR1A, MEN1, CALCA
29adrenal adenoma30.6MEN1, PRKAR1A, GNAS
30vipoma30.5CALCA
31parathyroid adenoma30.4CDKN1B, CALCA, MEN1, RET, PRKAR1A, CHGA
32breast cancer30.3CDKN1B, NF1, CALCA, VHL, MEN1, PRKAR1A
33carcinoid syndrome30.3GNAS, SDHD, CHGA, RET, MEN1, CALCA
34lipomatosis30.3MEN1
35malignant peripheral nerve sheath tumor30.3NF1
36renal dysplasia30.3GDNF, RET
37somatostatinoma30.3CALCA, CHGA
38glomus tumor30.3VHL, SDHD
39peutz-jeghers syndrome30.2VHL, PRKAR1A
40neurofibromatosis, type 230.2SDHD, NF1
41gastrointestinal stromal tumor30.2NF1, SDHC
42adenocarcinoma30.2CHGA, MEN1, VHL, CDKN1B
43hemangioma30.1SDHD, SDHC, RET, MEN1, VHL, NF1
44adrenal cortical adenoma30.1SDHD, SDHC, CHGA, PRKAR1A, MEN1, VHL
45thyroiditis11.1
46pancreatitis11.0
47multiple endocrine neoplasia iib10.7
48amyloidosis10.7
49duodenitis10.6
50multiple endocrine neoplasia, type iv10.5

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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Drug clinical trials:

Search ClinicalTrials for Multiple Endocrine Neoplasia

Search NIH Clinical Center for Multiple Endocrine Neoplasia

Genetic Tests for Multiple Endocrine Neoplasia

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Anatomical Context for Multiple Endocrine Neoplasia

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MalaCards organs/tissues related to Multiple Endocrine Neoplasia:

33
Thyroid, Pituitary, Testes, Bone, Pancreas, Skin, Pancreatic islet, Brain, Colon, Kidney, Liver, Adrenal gland, Breast, Prostate, Tongue, Lymph node, Thymus, Spinal cord, Adipocyte, Lung, Ovary, Endothelial

Animal Models for Multiple Endocrine Neoplasia or affiliated genes

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MGI Mouse Phenotypes related to Multiple Endocrine Neoplasia:

37 (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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Articles related to Multiple Endocrine Neoplasia:

(show top 50)    (show all 1037)
idTitleAuthorsYear
1
Molecular diagnosis and comprehensive treatment of multiple endocrine neoplasia type 2 in Southeastern Chinese. (25628771)
2015
2
Tanycytic ependymoma of the filum terminale associated with multiple endocrine neoplasia type 1: first reported case. (23562332)
2013
3
Multiple endocrine neoplasia: the enigma of MEN. (23880753)
2013
4
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
5
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
6
Multiple endocrine neoplasia type 1 presenting as refractory epilepsy and polyneuropathy--a case report. (22138026)
2012
7
Novel association of thymic carcinoid with a germline mutation in a kindred with multiple endocrine neoplasia 1 (MEN1). (22549346)
2012
8
Coexistence of Multiple Endocrine Neoplasia Type 2B and Chilaiditi Sign: A Case Report. (23119190)
2012
9
Functioning glucagonoma associated with primary hyperparathyroidism: multiple endocrine neoplasia type 1 or incidental association? (23259638)
2012
10
Neural hyperplasia in maxillary bone of multiple endocrine neoplasia type 2B patient. (22099857)
2011
11
A new mutation in the menin gene causes the multiple endocrine neoplasia type 1 syndrome with adrenocortical carcinoma. (21069576)
2011
12
Intracranial ependymoma associated with multiple endocrine neoplasia type 1. (20142633)
2010
13
Pathogenicity of DNA variants and double mutations in multiple endocrine neoplasia type 2 and von Hippel-Lindau syndrome. (19906784)
2010
14
Multiple endocrine neoplasia type 1 deletion in pancreatic alpha-cells leads to development of insulinomas in mice. (20555035)
2010
15
Unusual features of medullary thyroid carcinoma in multiple endocrine neoplasia type 2A. (19332941)
2009
16
Multiple endocrine neoplasia type 2a and germ line C634G RET mutation diagnosed in an 80-year-old patient. (19201392)
2009
17
First description of parathyroid disease in multiple endocrine neoplasia 2A syndrome. (19034701)
2008
18
Early diagnosis of multiple endocrine neoplasia type 2B: a challenge for physicians. (19169500)
2008
19
Adrenal involvement in multiple endocrine neoplasia type 1: results of 7 years prospective screening. (17235589)
2007
20
The impact of clinical and genetic screenings on the management of the multiple endocrine neoplasia type 1. (17823710)
2007
21
Management of nonfunctioning pancreatic endocrine tumors in the context of multiple endocrine neoplasia type 1 syndrome. (17925918)
2007
22
Management of pancreatic endocrine tumors in multiple endocrine neoplasia type 1. (16680581)
2006
23
Clinical testing for multiple endocrine neoplasia type 1 in a DNA diagnostic laboratory. (15714081)
2005
24
Do all patients with RET mutations associated with multiple endocrine neoplasia type 2 require surgery? (16929368)
2005
25
Adrenal ganglioneuromas in children with multiple endocrine neoplasia type 2: a report of two cases. (15827098)
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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Search GEO for disease gene expression data for Multiple Endocrine Neoplasia.

Pathways for genes affiliated with Multiple Endocrine Neoplasia

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Pathways related to Multiple Endocrine Neoplasia according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.8RET, GDNF
29.8RET, GDNF
39.4PRKAR1A, RET, VHL
49.3GNAS, CALCA
59.3CALCA, GNAS
69.1GNAS, PRKAR1A
79.1PRKAR1A, GNAS
8
Show member pathways
8.8CDKN1B, PRKAR1A, GNAS
9
Show member pathways
Calcium Regulation in the Cardiac Cell38
8.8CALCA, PRKAR1A, GNAS
10
Show member pathways
8.2CDKN1B, CALCA, MEN1, PRKAR1A, GNAS

Compounds for genes affiliated with Multiple Endocrine Neoplasia

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Compounds related to Multiple Endocrine Neoplasia according to GeneCards/GeneDecks:

(show top 50)    (show all 55)
idCompoundScoreTop Affiliating Genes
1111in-pentetreotide4510.0CHGA, CALCA
2sunitinib45 51 1312.0VHL, RET
3cinacalcet45 30 1311.9PRKAR1A, CALCA
4fluoride459.7NF1, CALCA, CHGA
5Fumaric acid269.6SDHC, SDHD
61,25 dihydroxy vitamin d3459.6CDKN1B, CALCA, GDNF
7bromodeoxyuridine459.6GDNF, CHGA, CDKN1B
8methionine459.5CHGA, RET, NF1, CDKN1B
9mibg459.5SDHD, CHGA, CALCA
10l-amino acid459.5GDNF, CHGA, PRKAR1A
11paraffin459.5CDKN1B, NF1, RET, CHGA
12ly294002459.5CDKN1B, VHL, RET, GDNF
13fumarate45 1310.4SDHD, SDHC, VHL
14acth459.4GNAS, CHGA
15ubiquinone459.3SDHC, SDHD
16testosterone45 61 26 1312.3CDKN1B, CALCA, RET, CHGA
17vitamin d459.2CHGA, PRKAR1A, MEN1, CALCA
18octreotide45 61 30 1312.2CALCA, CHGA, GNAS
19iron-sulfur459.2SDHC, SDHD
20catecholamine459.1RET, CHGA, SDHD, GDNF
21phosphatidylinositol459.1GDNF, RET, VHL, NF1, CDKN1B
22isoproterenol45 1310.1CALCA, CHGA, GNAS
23succinic acid30 26 1311.0SDHC, SDHD
24guanine45 26 1311.0GNAS, CHGA, CALCA
25atp45 3010.0CDKN1B, NF1, RET, CHGA, GDNF
26succinate458.8SDHD, SDHC, RET, VHL, NF1
27iron45 269.8GDNF, SDHD, SDHC, VHL
28progesterone45 30 61 26 1312.8CDKN1B, NF1, CHGA, GNAS
29dexamethasone45 51 30 1311.8GDNF, CHGA, PRKAR1A, CDKN1B
30norepinephrine45 26 1310.8CALCA, CHGA, GNAS, GDNF
31alanine458.6GDNF, GNAS, RET, NF1, CDKN1B
32retinoic acid45 269.6GDNF, SDHC, CHGA, RET, NF1, CDKN1B
33estrogen458.4GDNF, CHGA, MEN1, VHL, CALCA, NF1
34lipid458.4GDNF, GNAS, CHGA, RET, NF1
35dopamine45 30 26 1311.2GDNF, GNAS, CHGA, RET, CALCA, NF1
36steroid458.2GNAS, SDHC, CHGA, PRKAR1A, NF1
37arginine458.2GNAS, CHGA, PRKAR1A, RET, NF1, CDKN1B
38nitric oxide45 26 1310.2GDNF, GNAS, CHGA, VHL, CALCA, CDKN1B
39cysteine458.0GDNF, GNAS, CHGA, PRKAR1A, RET, NF1
40glucose458.0GDNF, GNAS, CHGA, PRKAR1A, VHL, CDKN1B
41oligonucleotide458.0GDNF, GNAS, CHGA, RET, VHL, NF1
42glutamate458.0GDNF, GNAS, CHGA, PRKAR1A, CALCA, CDKN1B
43vegf458.0GDNF, SDHC, CHGA, RET, MEN1, VHL
44adenylate458.0GDNF, GNAS, CHGA, PRKAR1A, CALCA, NF1
45serine457.7GDNF, GNAS, CHGA, RET, MEN1, VHL
46forskolin45 51 139.7GDNF, GNAS, CHGA, PRKAR1A, CALCA, NF1
47tyrosine457.7GDNF, GNAS, CHGA, RET, MEN1, VHL
48oxygen45 268.5GDNF, GNAS, SDHD, SDHC, CHGA, VHL
49cyclic amp45 268.4CDKN1B, GDNF, GNAS, CHGA, PRKAR1A, RET
50calcium45 51 26 1310.1GDNF, GNAS, CHGA, PRKAR1A, RET, MEN1

GO Terms for genes affiliated with Multiple Endocrine Neoplasia

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

idNameGO IDScoreTop Affiliating Genes
1protein complexGO:00432349.4CDKN1B, MEN1, PRKAR1A
2mitochondrial respiratory chain complex IIGO:00057499.0SDHC, 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:0200124110.2RET, NF1
2negative regulation of cyclin-dependent protein serine/threonine kinase activityGO:004573610.2CDKN1B, MEN1
3response to painGO:004826510.1CALCA, RET
4positive regulation of adenylate cyclase activityGO:004576210.1NF1, CALCA
5enteric nervous system developmentGO:004848410.0GDNF, RET
6negative regulation of osteoclast differentiationGO:004567110.0CALCA, NF1
7MAPK cascadeGO:00001659.9NF1, MEN1, RET
8sympathetic nervous system developmentGO:00484859.9GDNF, NF1
9metanephros developmentGO:00016569.9NF1, GDNF
10neural crest cell migrationGO:00017559.8RET, GDNF
11positive regulation of Ras GTPase activityGO:00323209.6NF1, GNAS
12cognitionGO:00508909.5NF1, GNAS
13tricarboxylic acid cycleGO:00060999.5SDHC, SDHD
14activation of adenylate cyclase activityGO:00071909.4CALCA, GNAS
15positive regulation of cAMP biosynthetic processGO:00308199.3GNAS, CALCA
16cellular response to glucagon stimulusGO:00713779.2GNAS, PRKAR1A
17adenylate cyclase-activating G-protein coupled receptor signaling pathwayGO:00071899.2GNAS, CALCA
18response to drugGO:00424939.1CDKN1B, RET, GNAS
19water transportGO:00068338.8GNAS, PRKAR1A

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

idNameGO IDScoreTop Affiliating Genes
1succinate dehydrogenase activityGO:00001049.0SDHC, SDHD
2protein bindingGO:00055157.2PRKAR1A, RET, MEN1, VHL, CALCA, NF1

Products for genes affiliated with Multiple Endocrine Neoplasia

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Sources for Multiple Endocrine Neoplasia

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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