FIPA
MCID: FML168
MIFTS: 29

Familial Isolated Pituitary Adenoma (FIPA) malady

Gastrointestinal, Genetic categories

Summaries for Familial Isolated Pituitary Adenoma

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21Genetics Home Reference, 33MalaCards
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Genetics Home Reference:21 Familial isolated pituitary adenoma (FIPA) is an inherited condition characterized by development of a noncancerous tumor in the pituitary gland (called a pituitary adenoma). The pituitary gland, which is found at the base of the brain, produces hormones that control many important body functions.

MalaCards: Familial Isolated Pituitary Adenoma, also known as FIPA, is related to adenoma and prolactinoma. An important gene associated with Familial Isolated Pituitary Adenoma is AIP (aryl hydrocarbon receptor interacting protein), and among its related pathways are AhR pathway and Development A2B receptor- action via G-protein alpha s. The compounds octreotide and forskolin have been mentioned in the context of this disorder. Affiliated tissues include brain and pituitary, and related mouse phenotypes are tumorigenesis and endocrine/exocrine gland.

Aliases & Classifications for Familial Isolated Pituitary Adenoma

Sources:
61UMLS, 43NIH Rare Diseases, 21Genetics Home Reference
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Genetic
Anatomical: Gastrointestinal


Aliases & Descriptions:

familial isolated pituitary adenoma 43 21
fipa 43 21
familial isolated pituitary adenoma syndrome 43
pituitary adenoma, familial isolated 61
multiple gastrointestinal atresias 61


Related Diseases for Familial Isolated Pituitary Adenoma

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Familial Isolated Pituitary Adenoma:



Diseases related to familial isolated pituitary adenoma

Clinical Features for Familial Isolated Pituitary Adenoma

Drugs & Therapeutics for Familial Isolated Pituitary Adenoma

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Familial Isolated Pituitary Adenoma

Anatomical Context for Familial Isolated Pituitary Adenoma

Sources:
33MalaCards
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MalaCards organs/tissues related to Familial Isolated Pituitary Adenoma:

33
Brain, Pituitary

Animal Models for Familial Isolated Pituitary Adenoma or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
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MGI Mouse Phenotypes related to Familial Isolated Pituitary Adenoma:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020068.7CDKN1B, AIP, PRKAR1A, SF1
2MP:00053797.5CDKN1B, AIP, SSTR5, PRKAR1A, GNAS, SF1
3MP:00053767.3SF1, GNAS, PRKAR1A, SSTR5, AIP, CDKN1B

Publications for Familial Isolated Pituitary Adenoma

Sources:
51PubMed
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Articles related to Familial Isolated Pituitary Adenoma:

(show all 16)
idTitleAuthorsYear
1
Clinical experience in the screening and management of a large kindred with familial isolated pituitary adenoma due to an aryl hydrocarbon receptor interacting protein (AIP) mutation. (24423289)
2013
2
Familial isolated pituitary adenomas (FIPA) and the pituitary adenoma predisposition due to mutations in the aryl hydrocarbon receptor interacting protein (AIP) gene. (23371967)
2013
3
Familial isolated pituitary adenoma caused by a Aip gene mutation not described before in a family context. (24078436)
2013
4
Cyclin-dependent kinase inhibitor 1B (CDKN1B) gene variants in AIP mutation-negative familial isolated pituitary adenoma kindreds. (22291433)
2012
5
Genetic analysis in a patient presenting with meningioma and familial isolated pituitary adenoma (FIPA) reveals selective involvement of the R81X mutation of the AIP gene in the pathogenesis of the pituitary tumor. (22527616)
2012
6
Familial isolated pituitary adenoma: evidence for genetic heterogeneity. (20616498)
2010
7
Familial isolated pituitary adenomas experience at a single center: clinical importance of AIP mutation screening. (21340155)
2010
8
The R304X mutation of the aryl hydrocarbon receptor interacting protein gene in familial isolated pituitary adenomas: Mutational hot-spot or founder effect? (20354355)
2010
9
Characterization of aryl hydrocarbon receptor interacting protein (AIP) mutations in familial isolated pituitary adenoma families. (20506337)
2010
10
Genetic, molecular and clinical features of familial isolated pituitary adenomas. (19407507)
2009
11
Update on familial pituitary tumors: from multiple endocrine neoplasia type 1 to familial isolated pituitary adenoma. (19153518)
2009
12
Silent familial isolated pituitary adenomas: histopathological and clinical case report. (18317953)
2008
13
The clinical, pathological, and genetic features of familial isolated pituitary adenomas. (17893250)
2007
14
Aryl hydrocarbon receptor-interacting protein gene mutations in familial isolated pituitary adenomas: analysis in 73 families. (17244780)
2007
15
Clinical characterization of familial isolated pituitary adenomas. (16787992)
2006
16
AIP-Related Familial Isolated Pituitary Adenomas (22720333)
1993

Genetic Variations for Familial Isolated Pituitary Adenoma

Expression for genes affiliated with Familial Isolated Pituitary Adenoma

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Familial Isolated Pituitary Adenoma

Search GEO for disease gene expression data for Familial Isolated Pituitary Adenoma.

Pathways for genes affiliated with Familial Isolated Pituitary Adenoma

Sources:
38NCBI BioSystems Database, 12EMD Millipore, 54Reactome
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Compounds for genes affiliated with Familial Isolated Pituitary Adenoma

Sources:
45Novoseek, 60Tocris Bioscience, 29IUPHAR, 11DrugBank, 50PharmGKB, 24HMDB
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Compounds related to Familial Isolated Pituitary Adenoma according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1octreotide45 60 29 1112.0SSTR5, GNAS
2forskolin45 50 1111.0GNAS, PRKAR1A, CDKN1B
3thymidine45 249.9GNAS, SSTR5, CDKN1B
4glutamine458.8GNAS, SF1, CDKN1B
5cyclic amp45 249.2CNC, GNAS, PRKAR1A, CDKN1B

GO Terms for genes affiliated with Familial Isolated Pituitary Adenoma

Sources:
16Gene Ontology
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Biological processes related to Familial Isolated Pituitary Adenoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1water transportGO:0068339.4PRKAR1A, GNAS
2cellular response to glucagon stimulusGO:0713779.1PRKAR1A, GNAS
3regulation of insulin secretionGO:0507968.8SSTR5, PRKAR1A, GNAS

Products for genes affiliated with Familial Isolated Pituitary Adenoma

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  • Lysates
  • Antibodies

Sources for Familial Isolated Pituitary Adenoma

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet