PXA
MCID: PLM020
MIFTS: 55

Pleomorphic Xanthoastrocytoma (PXA) malady

Neuronal diseases, Cancer diseases categories

Summaries for Pleomorphic Xanthoastrocytoma

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63Wikipedia, 32MalaCards
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Wikipedia:63 Pleomorphic Xanthoastrocytoma is a neoplasm of the brain that occurs often in children and teenagers.... more...

MalaCards: Pleomorphic Xanthoastrocytoma, also known as PXA, is related to ganglioglioma and astrocytoma. An important gene associated with Pleomorphic Xanthoastrocytoma is SYP (synaptophysin), and among its related pathways are Small cell lung cancer and Development IGF-RI signaling. The compounds estrogen and vincristine have been mentioned in the context of this disorder. Affiliated tissues include brain, spinal cord and parietal lobe, and related mouse phenotypes are embryogenesis and renal/urinary system.

Aliases & Classifications for Pleomorphic Xanthoastrocytoma

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8Disease Ontology, 42NIH Rare Diseases, 48Orphanet, 60UMLS, 39NCIt, 56SNOMED-CT, 57SNOMED-CT via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer diseases
Anatomical: Neuronal diseases


Characteristics (Orphanet epidemiological data):

48
pleomorphic xanthoastrocytoma:
Inheritance: Sporadic; Age of onset: Variable


Aliases & Descriptions:

pleomorphic xanthoastrocytoma 8 42 48 60
pxa 42 48
pleomorphic xantho-astrocytoma 8


External Ids:

Disease Ontology8 DOID:4852
NCIt39 C4323
SNOMED-CT56 189924002, 78838008
SNOMED-CT via Orphanet57 78838008

Related Diseases for Pleomorphic Xanthoastrocytoma

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17GeneCards, 18GeneDecks
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Diseases related to Pleomorphic Xanthoastrocytoma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 162)
idRelated DiseaseScoreTop Affiliating Genes
1ganglioglioma30.9SYP, GFAP, TSC1, TUBB
2astrocytoma30.7MDM2, MGMT, EGFR, SYP, CDKN2B, CDKN2A
3glioblastoma multiforme30.5TP53, EGFR, CDKN2A, CDKN2B, CDK4, GFAP
4giant cell glioblastoma30.4EGFR, GFAP, MDM2
5oligodendroglioma30.4CDKN2B, GFAP, CDKN2A, EGFR, MGMT, BRAF
6atypical teratoid rhabdoid tumor30.2SMARCB1
7dysembryoplastic neuroepithelial tumor30.2SYP
8fibrous histiocytoma30.2CD34
9meningioma30.2SMARCB1, TSC1, GFAP, CD34, CDKN2B, CD68
10neurofibromatosis10.6
11cerebritis10.5
12neuronitis10.5
13meningitis10.4
14arteriovenous malformation10.3
15moyamoya disease10.2
16temporal lobe epilepsy10.2
17cerebellar astrocytoma10.2
18histiocytoma10.2
19glomus tumor10.0SYP
20chordoma10.0GFAP
21gliomatosis cerebri10.0GFAP
22angiomyolipoma10.0TSC1
23gastrointestinal stromal tumor10.0CD34
24wilms tumor10.0SMARCB1
25choroid plexus carcinoma10.0SMARCB1
26ganglioneuroblastoma10.0SYP
27gemistocytic astrocytoma10.0GFAP
28synovial sarcoma10.0CD34
29mycosis fungoides10.0CDKN2B
30angiosarcoma10.0CD34
31skin melanoma10.0CDKN2B
32leiomyoma10.0CD34
33plasma cell leukemia10.0CDK4
34kaposi's sarcoma10.0CD34
35medulloepithelioma10.0SYP
36melanotic neuroectodermal tumor10.0SYP
37uterine sarcoma10.0MDM2
38peutz-jeghers syndrome10.0TSC1
39cervical adenocarcinoma10.0CDKN2A
40dermatofibrosarcoma10.0MDM2
41merkel cell carcinoma10.0SYP
42hemangioblastoma10.0SYP, GFAP
43rhabdoid tumor10.0SMARCB1, SYP
44hemangioma10.0CD68, CD34
45spitz nevus10.0BRAF
46biliary tract neoplasm10.0EGFR
47leukoplakia10.0MGMT
48congenital mesoblastic nephroma10.0SMARCB1
49pineocytoma10.0GFAP, SYP
50teratoma10.0GFAP, SMARCB1

Graphical network of the top 20 diseases related to Pleomorphic Xanthoastrocytoma:



Diseases related to pleomorphic xanthoastrocytoma

Clinical Features for Pleomorphic Xanthoastrocytoma

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Drugs & Therapeutics for Pleomorphic Xanthoastrocytoma

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

Search CenterWatch for Pleomorphic Xanthoastrocytoma

Drug clinical trials:

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Search NIH Clinical Center for Pleomorphic Xanthoastrocytoma

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Genetic Tests for Pleomorphic Xanthoastrocytoma

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Anatomical Context for Pleomorphic Xanthoastrocytoma

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32MalaCards
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MalaCards organs/tissues related to Pleomorphic Xanthoastrocytoma:

32
Brain, Spinal cord, Parietal lobe, Temporal lobe, Cerebellum, Pineal, Retina, Testes

Animal Models for Pleomorphic Xanthoastrocytoma or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Pleomorphic Xanthoastrocytoma:

36 (show all 20)
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000538010.4TSC1
2MP:00053679.9TSC1, EGFR
3MP:00036319.7TSC1, GFAP, CDK4, SYP
4MP:00053919.6EGFR, GFAP, SMARCB1
5MP:00053909.1CD68, TSC1
6MP:00053798.7MGMT, CDKN2A, CDKN2B, CDK4, TP53
7MP:00053898.7BRAF, EGFR, CDKN2A, CDKN2B, TSC1, TP53
8MP:00053768.5MGMT, CDK4, CD34, GFAP, TSC1, SMARCB1
9MP:00053698.5BRAF, MDM2, CDKN2A, CDK4, GFAP, TSC1
10MP:00107718.4MDM2, CDKN2A, CDKN2B, CDK4, CD34, TSC1
11MP:00053708.2BRAF, MDM2, EGFR, CDKN2A, CDK4, TSC1
12MP:00053818.1BRAF, MDM2, EGFR, CDKN2A, CDK4, GFAP
13MP:00053857.8BRAF, MDM2, EGFR, CDK4, GFAP, TSC1
14MP:00053787.6MDM2, EGFR, CDKN2A, CDK4, GFAP, TSC1
15MP:00030127.4MGMT, MDM2, EGFR, SYP, CDKN2A, CD68
16MP:00053847.1BRAF, MDM2, EGFR, CDKN2A, CDKN2B, CDK4
17MP:00107686.9BRAF, MGMT, MDM2, EGFR, CDKN2A, CDKN2B
18MP:00053976.8BRAF, MDM2, EGFR, CDKN2A, CD68, CDKN2B
19MP:00020066.8TP53, BRAF, MGMT, MDM2, EGFR, CDKN2A
20MP:00053876.6CDKN2A, EGFR, MDM2, BRAF, CD68, CDKN2B

Publications for Pleomorphic Xanthoastrocytoma

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50PubMed
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Articles related to Pleomorphic Xanthoastrocytoma:

(show top 50)    (show all 190)
idTitleAuthorsYear
1
Immunohistochemistry is highly sensitive and specific for detection of BRAF V600E mutation in pleomorphic xanthoastrocytoma. (24252190)
2013
2
Primary multicentric anaplastic pleomorphic xanthoastrocytoma with atypical features. (23827171)
2013
3
Prognostic factors and therapeutic outcomes in 22 patients with pleomorphic xanthoastrocytoma. (23908701)
2013
4
Pleomorphic xanthoastrocytoma: long-term results of surgical treatment and analysis of prognostic factors. (23514331)
2013
5
Salvage therapy with BRAF inhibitors for recurrent pleomorphic xanthoastrocytoma: a retrospective case series. (23756728)
2013
6
BRAF-Mutated Pleomorphic Xanthoastrocytoma is Associated with Temporal Location, Reticulin Fiber Deposition and CD34 Expression. (24345274)
2013
7
Establishment of a primary pleomorphic xanthoastrocytoma cell line: in vitro responsiveness to some chemotherapeutics. (21629132)
2012
8
Pleomorphic xanthoastrocytoma: a new differential diagnosis for a pediatric pineal neoplasm. (22203031)
2012
9
Pleomorphic xanthoastrocytoma in the lateral ventricle with extensive subarachnoid dissemination: report of a case and review of the literature. (22340580)
2012
10
Pleomorphic xanthoastrocytoma and oligodendroglioma: collision of 2 morphologically and genetically distinct anaplastic components. (21214337)
2011
11
Analysis of BRAF V600E mutation in 1,320 nervous system tumors reveals high mutation frequencies in pleomorphic xanthoastrocytoma, ganglioglioma and extra-cerebellar pilocytic astrocytoma. (21274720)
2011
12
Periventricular pleomorphic xanthoastrocytoma (PXA): an uncommon tumor at an atypical site. (21755146)
2011
13
Pigmented pleomorphic xanthoastrocytoma: a rare variant and literature review. (20573030)
2011
14
Primary spinal pleomorphic xanthoastrocytoma. (21045509)
2010
15
Anaplastic pleomorphic xanthoastrocytoma with a component of anaplastic astrocytoma presenting as skull base tumor followed by downward extracranial extension. Case report. (21206189)
2010
16
Malignant progression of a pleomorphic xanthoastrocytoma in a child. (20799153)
2010
17
Pleomorphic xanthoastrocytoma in children and adolescents. (20582976)
2010
18
Temporal pleomorphic xanthoastrocytoma with glycogen accumulation--case report. (18666439)
2008
19
Pleomorphic xanthoastrocytoma with an intracystic hemorrhage : a case report and literature review. (19096580)
2007
20
Malignant transformation of a pleomorphic xanthoastrocytoma. (16369863)
2006
21
Malignant transformation of pleomorphic xanthoastrocytoma. (15912255)
2006
22
Giant cell glioblastoma and pleomorphic xanthoastrocytoma. (15043454)
2004
23
Genetic alterations commonly found in diffusely infiltrating cerebral gliomas are rare or absent in pleomorphic xanthoastrocytomas. (12484572)
2002
24
Ultrastructural features of pleomorphic xanthoastrocytoma: a comparative study with glioblastoma multiforme. (11783911)
2001
25
Pleomorphic xanthoastrocytoma derived from glioneuronal malformation in a child with intractable epilepsy. (10805198)
2000
26
Pleomorphic xanthoastrocytoma with CT and MRI appearance of meningioma. (9987765)
1999
27
Hugh Cairns, Dorothy Russell and the first pleomorphic xanthoastrocytoma? (10616587)
1999
28
Fatal pleomorphic xanthoastrocytoma with meningeal gliomatosis. (9602336)
1998
29
Atypical pleomorphic xanthoastrocytoma. (10192056)
1998
30
Diffuse leptomeningeal spread of pleomorphic xanthoastrocytoma. (18639021)
1998
31
Prognostic markers of pleomorphic xanthoastrocytoma. (9822932)
1998
32
Pleomorphic xanthoastrocytoma with gangliogliomatous component. (9521020)
1997
33
Composite pleomorphic xanthoastrocytoma and ganglioglioma: report of four cases and review of the literature. (9236832)
1997
34
Pleomorphic xanthoastrocytoma: clinical, imaging and pathological features of four cases. (9107467)
1997
35
Anaplastic pleomorphic xanthoastrocytoma. (9083703)
1997
36
Pleomorphic xanthoastrocytoma with neurofibromatosis type 1: case report. (8916131)
1996
37
PET-FDG of pleomorphic xanthoastrocytoma. (7799091)
1995
38
Pleomorphic xanthoastrocytoma in the spinal cord. Case report. (8113873)
1994
39
MRI of pleomorphic xanthoastrocytoma: case report. (7991088)
1994
40
Pleomorphic xanthoastrocytoma and desmoplastic infantile ganglioglioma--have these neoplasms a common origin? (7889337)
1994
41
Pleomorphic xanthoastrocytoma: the birth of a diagnosis and a concept. (8293186)
1993
42
Pleomorphic xanthoastrocytoma, a distinctive astroglial tumor: neuroradiologic and pathologic features. (8279337)
1993
43
Increased mitotic activity as a negative prognostic indicator in pleomorphic xanthoastrocytoma. Case report. (8410257)
1993
44
Pleomorphic xanthoastrocytoma. Ultrastructural, immunohistochemical, and DNA cytofluorometric study of a case. (1855184)
1991
45
The pleomorphic xanthoastrocytoma and its differential diagnosis: a study of five cases. (1743696)
1991
46
Pleomorphic xanthoastrocytoma with desmoplastic reaction: angiomatous variant. Report of two cases. (1962743)
1990
47
Glioblastoma multiforme masquerading as a pleomorphic xanthoastrocytoma. (2547522)
1989
48
Glioblastoma multiforme masquerading as a pleomorphic xanthoastrocytoma. (2844403)
1988
49
Does the pleomorphic xanthoastrocytoma exist? Problems in the application of immunological techniques to the classification of brain tumors. (3213427)
1988
50
Pleomorphic xanthoastrocytoma. Ultrastructural and immunohistochemical study of a case with a rapidly fatal outcome following surgery. (6194876)
1983

Genetic Variations for Pleomorphic Xanthoastrocytoma

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Expression for genes affiliated with Pleomorphic Xanthoastrocytoma

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Pleomorphic Xanthoastrocytoma

Search GEO for disease gene expression data for Pleomorphic Xanthoastrocytoma.

Pathways for genes affiliated with Pleomorphic Xanthoastrocytoma

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Sources:
29KEGG, 12EMD Millipore, 51QIAGEN, 37NCBI BioSystems Database, 53Reactome, 55SinoBiological, 49PharmGKB, 4Cell Signaling Technology, 52R&D Systems
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Pathways related to Pleomorphic Xanthoastrocytoma according to GeneCards/GeneDecks:

(show all 36)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.5TP53, CDK4, CDKN2B
2
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9.5TP53, TSC1, MDM2
39.4TP53, CDK4, MDM2
49.4MDM2, CDKN2A, TP53
59.4MDM2, CDKN2A, TP53
69.4MDM2, CDKN2A, TP53
7
Hide members
9.4TP53, CDKN2A, MDM2
89.4TUBB, BRAF, EGFR
9
Hide members
9.3GFAP, SYNM, TUBB
10
Hide members
9.3MDM2, CDKN2A, CDKN2B, CDK4
119.3TP53, EGFR, MDM2
12
Hide members
9.2TP53, CDK4, CDKN2B, BRAF
13
Hide members
9.2MDM2, CDK4, TSC1, TP53
149.2CDKN2A, CDKN2B, CDK4, TP53
15
Hide members
9.2CDKN2A, CDKN2B, CDK4, TP53
169.1TP53, CDK4, MGMT
17
Transcription P53 signaling pathway
Hide members
9.1MDM2, CDKN2A, CDK4, TP53
18
Hide members
9.1MDM2, CDKN2A, CDK4, TP53
199.1TP53, GFAP, CDK4, EGFR
209.1TP53, CDKN2A, MDM2, BRAF
21
Hide members
9.0BRAF, MDM2, EGFR, TP53
22
Hide members
9.0BRAF, MDM2, EGFR, TP53
239.0TP53, EGFR, MDM2, BRAF
249.0MDM2, EGFR, CDKN2A, TP53
25
Hide members
8.9MGMT, MDM2, CDKN2A, CDK4
26
Hide members
8.9BRAF, MDM2, CDKN2B, TSC1, TP53
278.9TP53, CDK4, CDKN2B, CDKN2A, MDM2
288.8MDM2, EGFR, CDKN2B, CDK4, TP53
298.8MDM2, EGFR, CDK4, TSC1, TP53
308.8TP53, TSC1, CDK4, EGFR, MDM2
31
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8.6BRAF, MDM2, EGFR, CDKN2A, TP53
328.4BRAF, MDM2, EGFR, CDK4, TSC1, TP53
33
Hide members
8.4BRAF, MDM2, EGFR, CDKN2A, CDK4, TP53
348.1BRAF, MDM2, EGFR, CDKN2A, CDKN2B, CDK4
35
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7.6BRAF, MDM2, EGFR, CDKN2A, CDKN2B, CDK4
367.5TP53, BRAF, MGMT, MDM2, EGFR, CDKN2A

Compounds for genes affiliated with Pleomorphic Xanthoastrocytoma

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Sources:
44Novoseek, 49PharmGKB, 11DrugBank, 24HMDB, 59Tocris Bioscience, 28IUPHAR, 2BitterDB
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Compounds related to Pleomorphic Xanthoastrocytoma according to GeneCards/GeneDecks:

(show top 50)    (show all 114)
idCompoundScoreTop Affiliating Genes
1estrogen4410.7GFAP
2vincristine44 49 1111.9TUBB, MGMT
3reticulin449.7GFAP, CD34, SYP
4depsipeptide449.6TP53, CDKN2A, TUBB
5dutp449.6SYP, GFAP, TP53, MDM2
6serine449.6TUBB, SYP, CDKN2B, TSC1, GFAP
7sodium bisulfite449.5CDKN2A, MGMT, CDKN2B
8roscovitine449.4CDK4, CDKN2A, TP53, MDM2
9hematoxylin449.4CDKN2A, GFAP, CD34, SYP
10acnu449.4CDKN2A, TP53, MGMT
117,12-dimethylbenz(a)anthracene449.4MDM2, CDKN2A, MGMT
12silver449.3GFAP, CD34, SYP, CDKN2A
13olomoucine44 1110.3CDKN2A, CDK4, TP53
145-methylcytosine44 2410.2TP53, CDKN2A, MGMT, CDKN2B
155-methylthioadenosine449.2CDKN2B, CDKN2A, EGFR
16cytarabine44 49 1111.2CDKN2B, TP53, CDKN2A, GFAP
17valine449.1BRAF, MDM2, TUBB, CDKN2A, CDK4
18crcs449.1BRAF, CDKN2A, TP53, MGMT
19chlorambucil44 1110.1TP53, MDM2, MGMT
20geldanamycin44 49 59 1112.1BRAF, CDK4, EGFR, MDM2, TP53
21flavopiridol44 1110.1CDK4, CDKN2A, EGFR, MDM2, TP53
22adpribose449.1EGFR, CDK4, TP53, MDM2
23temozolomide44 1110.0EGFR, TP53, MGMT, CDKN2A
24docetaxel44 49 59 1112.0TP53, CDKN2A, EGFR, MDM2, TUBB
25o6-methylguanine448.9MGMT, TP53, BRAF, CDKN2A, CDKN2B
26cyclic amp44 249.9BRAF, MDM2, GFAP, CDK4, CDKN2A, SYP
27pd 98,059448.8MDM2, TSC1, GFAP, CDK4, CDKN2B, EGFR
28camptothecin44 59 1110.8MDM2, CDKN2A, CDK4, MGMT, TP53
29bcnu448.8TP53, MGMT, CDKN2A, EGFR, GFAP
305-aza-2deoxycytidine448.8MDM2, CDKN2A, TP53, MGMT, CDKN2B
31carboplatin44 49 1110.8MGMT, MDM2, CDK4, TP53, EGFR
32curcumin448.7EGFR, MGMT, TP53, CDK4, MDM2
33testosterone44 59 11 2411.6TUBB, MDM2, EGFR, CDKN2A, CDK4, GFAP
34alanine448.6TSC1, CDKN2A, TUBB, BRAF, GFAP, CDK4
35threonine448.5GFAP, TSC1, BRAF, TUBB, MDM2, CDKN2A
36agar448.5BRAF, MDM2, GFAP, CD34, CDK4, CDKN2A
375fluorouracil448.5EGFR, CDK4, CDKN2A, MDM2, TP53, MGMT
38irinotecan44 49 1110.5MGMT, MDM2, EGFR, CDKN2A, TP53, BRAF
39genistein44 28 59 2 11 2413.5MGMT, GFAP, CDK4, CDKN2A, SYP, EGFR
40doxorubicin44 49 1110.4MGMT, TP53, CDK4, CDKN2A, EGFR, MDM2
41paclitaxel44 49 1110.2TUBB, GFAP, MDM2, TP53, CDK4, CDKN2A
42thymidylate448.1CDKN2A, EGFR, MDM2, CDK4, MGMT, TUBB
43oligonucleotide448.1MGMT, MDM2, TUBB, BRAF, CDK4, TP53
44thymidine44 249.0MGMT, MDM2, CDK4, CD34, GFAP, TUBB
45tyrosine447.7CD34, TUBB, GFAP, CDKN2B, CDK4, BRAF
46cisplatin44 49 59 1110.3GFAP, CDK4, CDKN2A, BRAF, EGFR, MGMT
47retinoic acid44 248.2TP53, GFAP, CDK4, BRAF, MGMT, EGFR
48progesterone44 59 28 11 2411.2CD34, BRAF, MGMT, MDM2, EGFR, SYP
49vegf446.9CD34, BRAF, TP53, TSC1, GFAP, CDK4
50paraffin445.7TP53, TUBB, CD68, BRAF, MGMT, MDM2

GO Terms for genes affiliated with Pleomorphic Xanthoastrocytoma

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16Gene Ontology
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Cellular components related to Pleomorphic Xanthoastrocytoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1nuclear bodyGO:0166049.4MDM2, CDKN2A, TP53
2protein complexGO:0432349.0TP53, TSC1, CDKN2A, SYP, MDM2
3membraneGO:0160208.2TSC1, GFAP, CD68, SYNM, EGFR
4nucleoplasmGO:0056547.9MGMT, MDM2, CDKN2A, CDK4, SMARCB1, TP53
5cytosolGO:0058297.5TUBB, BRAF, MDM2, CDKN2A, CDKN2B, CDK4

Biological processes related to Pleomorphic Xanthoastrocytoma according to GeneCards/GeneDecks:

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1replicative senescenceGO:0903999.8TP53, CDKN2A
2positive regulation of cell cycle arrestGO:0711589.7TP53, CDKN2A
3cell cycle arrestGO:0070509.4TP53, TSC1, CDKN2B, CDKN2A
4cellular response to organic substanceGO:0713109.3CD68, SYP
5positive regulation of gene expressionGO:0106289.3CD34, MDM2, BRAF
6negative regulation of cell proliferationGO:0082859.1CDKN2A, CDKN2B, TSC1, SMARCB1, TP53
7mitotic cell cycleGO:0002788.9TUBB, CDKN2A, CDKN2B, CDK4
8positive regulation of transcription from RNA polymerase II promoterGO:0459448.9EGFR, CDKN2A, CDKN2B, SMARCB1, TP53

Molecular functions related to Pleomorphic Xanthoastrocytoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1MDM2/MDM4 family protein bindingGO:0973719.7TP53, CDKN2A
2cyclin-dependent protein serine/threonine kinase inhibitor activityGO:0048619.6CDKN2A, CDKN2B
3protein complex bindingGO:0324039.6CDK4, SYP, TUBB
4p53 bindingGO:0020399.2TP53, SMARCB1, CDKN2A, MDM2
5structural constituent of cytoskeletonGO:0052009.2GFAP, SYNM, TUBB
6identical protein bindingGO:0428028.8BRAF, MDM2, EGFR, SYP, TP53

Products for genes affiliated with Pleomorphic Xanthoastrocytoma

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Sources for Pleomorphic Xanthoastrocytoma

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet