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, Testes, Retina

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
Pleomorphic xanthoastrocytoma: magnetic resonance imaging findings in a series of cases with histopathological confirmation. (23249973)
2013
2
The coexistence of pleomorphic xanthoastrocytoma and arteriovenous malformation. A case report. (24114645)
2013
3
Case of pleomorphic xanthoastrocytoma with anaplastic features in the pineal gland. (23460303)
2013
4
Possible differentiation of cerebral glioblastoma into pleomorphic xanthoastrocytoma: an unusual case in an infant. (22546030)
2012
5
Pleomorphic xanthoastrocytoma arising in neurofibromatosis Type 1. (22551920)
2012
6
Malignant potential of pleomorphic xanthoastrocytoma. (22137880)
2012
7
Pleomorphic xanthoastrocytoma - a clinico-pathological review. (22101999)
2011
8
Temporal lobe pleomorphic xanthoastrocytoma and chronic epilepsy: long-term surgical outcomes. (21741164)
2011
9
Pleomorphic xanthoastrocytoma of the pineal region. (20655751)
2010
10
Subtelomeric analysis of pediatric astrocytoma: subchromosomal instability is a distinctive feature of pleomorphic xanthoastrocytoma. (19099200)
2009
11
Rapid progression of early delayed radiation effect in pleomorphic xanthoastrocytoma. (20062573)
2009
12
Prevalence of mutated TP53 on cDNA (but not on DNA template) in pleomorphic xanthoastrocytoma with positive TP53 immunohistochemistry. (19665069)
2009
13
Pleomorphic xanthoastrocytoma as a component of a temporal lobe cystic ganglioglioma: a case report. (19408095)
2009
14
Pleomorphic xanthoastrocytoma associated with long-standing Taylor-type IIB-focal cortical dysplasia in an adult. (18657915)
2009
15
Pleomorphic xanthoastrocytoma in elderly patients may portend a poor prognosis. (18255294)
2008
16
Pleomorphic xanthoastrocytoma: a comparative pathological study between conventional and anaplastic types. (18184267)
2008
17
Atypical teratoid/rhabdoid tumor arising in the setting of a pleomorphic xanthoastrocytoma. (17431546)
2007
18
Malignant progression in pleomorphic xanthoastrocytoma: personal experience and review of the literature. (17189643)
2007
19
A neuroepithelial tumor showing combined histological features of dysembryoplastic neuroepithelial tumor and pleomorphic xanthoastrocytoma--a case report and review of the literature. (17702498)
2007
20
Unusual recurrence of pleomorphic xanthoastrocytoma. (17439101)
2006
21
Diffuse malignant transformation of pleomorphic xanthoastrocytoma 21 years later: a matter of time? (16369862)
2006
22
Pediatric cerebellar pleomorphic xanthoastrocytoma with anaplastic features: a case of long-term survival after multimodality therapy. (16570197)
2006
23
A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination. (18095120)
2006
24
Multicentric pleomorphic xanthoastrocytoma in a patient with neurofibromatosis type 1. Case report and review of the literature. (15739569)
2005
25
Pleomorphic xanthoastrocytoma with anaplastic features presenting without GFAP immunoreactivity: implications for differential diagnosis. (16193842)
2005
26
Cerebellar pleomorphic xanthoastrocytoma in a patient with neurofibromatosis type 1. (15289955)
2004
27
Pleomorphic xanthoastrocytoma of the cerebellum: illustrated review. (15455217)
2004
28
Pleomorphic xanthoastrocytoma: a developmental glioneuronal tumor with prominent glioproliferative changes. (15015766)
2004
29
Molecular genetic analysis of anaplastic pleomorphic xanthoastrocytoma. (12732498)
2003
30
Expression of macrophage/histiocytic antigens in pleomorphic xanthoastrocytomas. (12899201)
2003
31
The use of chemotherapy to facilitate surgical resection in pleomorphic xanthoastrocytoma: experience in a single case. (11585332)
2001
32
Pleomorphic xanthoastrocytoma: report of a case diagnosed by intraoperative cytopathological examination. (11169891)
2001
33
Clinicopathological study of pleomorphic xanthoastrocytoma: correlation between histological features and prognosis. (11012983)
2000
34
Angiomatous pleomorphic xanthoastrocytoma as a component of ganglioglioma. (17337987)
1999
35
Pleomorphic xanthoastrocytoma with malignant progression. (9817466)
1998
36
Late sequelae of treated pleomorphic xanthoastrocytoma: malignant brain stem astrocytoma occurring 15 years after radiation therapy. (9049863)
1997
37
Pleomorphic xanthoastrocytoma: DNA flow cytometry and outcome analysis of 12 patients. (9392337)
1997
38
Arteriovenous malformation associated with pleomorphic xanthoastrocytoma. (8800336)
1996
39
Pleomorphic xanthoastrocytoma with a gangliomatous component: an immunohistochemical and ultrastructural study. (7732793)
1995
40
Cerebellar pleomorphic xanthoastrocytoma: case report. (7838347)
1994
41
Pleomorphic xanthoastrocytoma. (8498232)
1993
42
Pleomorphic xanthoastrocytoma as a component of a cerebellar ganglioglioma: case report. (1513442)
1992
43
Test and teach. Number Sixty-four. Diagnosis: Pleomorphic xanthoastrocytoma. (2091002)
1990
44
Pleomorphic xanthoastrocytoma. Report of four cases. (2644402)
1989
45
Pleomorphic xanthoastrocytoma in a 62-year-old male. (3447073)
1987
46
Pleomorphic xanthoastrocytoma. Immunohistochemical methods for differentiation from fibrous histiocytomas with similar morphology. (3010749)
1986
47
Pleomorphic xanthoastrocytoma with 18-year survival. Case report. (4056889)
1985
48
A variant of cerebral glioma called pleomorphic xanthoastrocytoma: case report. (4000446)
1985
49
Pleomorphic xanthoastrocytoma: a report of two cases. (6687296)
1983
50
Pleomorphic xanthoastrocytoma: report of a case with light and electron microscopy. (16830446)
1981

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
Hide members
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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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