MCID: PLM032
MIFTS: 61

Pulmonary Blastoma malady

Respiratory diseases, Cancer diseases, Rare diseases categories

Summaries for Pulmonary Blastoma

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9Disease Ontology, 66Wikipedia, 34MalaCards
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Disease Ontology:9 An embryoma and malignant neoplasm of lung that derives from tissues of the lung and derives from pleura (a thin layer of tissue that covers the lungs and lines the interior wall of the chest cavity). it is most commonly effects children.

MalaCards: Pulmonary Blastoma, also known as blastoma of lung, is related to sarcoma and adenocarcinoma, and has symptoms including abnormal pleura/hydrothorax/pleuresia/pleural effusion/chylothorax, fever/chilling and weight loss/loss of appetite/break in weight curve/general health alteration. An important gene associated with Pulmonary Blastoma is NKX2-1 (NK2 homeobox 1), and among its related pathways are Apoptotic cleavage of cellular proteins and Cell adhesion Endothelial cell contacts by junctional mechanisms. The compounds s 100 and panitumumab have been mentioned in the context of this disorder. Affiliated tissues include lung, fetal lung and ovary, and related mouse phenotypes are pigmentation and limbs/digits/tail.

Wikipedia:66 Pleuropulmonary blastoma (PPB) is a rare cancer originating in the lung or pleural cavity. It occurs... more...

Aliases & Classifications for Pulmonary Blastoma

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Sources:
9Disease Ontology, 46Novoseek, 50Orphanet, 63UMLS, 59SNOMED-CT, 41NCIt, 36MeSH, 27ICD10 via Orphanet, 60SNOMED-CT via Orphanet, 64UMLS via Orphanet, 37MESH via Orphanet
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer diseases, Rare diseases
Anatomical: Respiratory diseases


Characteristics (Orphanet epidemiological data):

50
pulmonary blastoma:
Inheritance: Multigenic/multifactorial; Age of onset: Adulthood; Age of death: Adult


Aliases & Descriptions:

pulmonary blastoma 9 46 50 63
blastoma of lung 9
pneumoblastoma 50


External Ids:

Disease Ontology9 DOID:4765
NCIt41 C3732
MeSH36 D018202
ICD10 via Orphanet27 C34
SNOMED-CT59 189815007, 43149009
SNOMED-CT via Orphanet60 43149009
UMLS via Orphanet64 C0206629
MESH via Orphanet37 D018202

Related Diseases for Pulmonary Blastoma

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Sources:
18GeneCards, 19GeneDecks
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Diseases related to Pulmonary Blastoma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 103)
idRelated DiseaseScoreTop Affiliating Genes
1sarcoma30.1CGB, KRAS, VIM, TP53
2adenocarcinoma30.0TP53, VIM, SYP, NKX2-1, KRAS, EGFR
3melanoma30.0TP53, VIM, NKX2-1, KRAS, CTNNB1, CGB
4blastoma11.2
5pleuropulmonary blastoma10.5
6classic pulmonary blastoma10.5
7epithelial predominant pulmonary blastoma10.3
8spindle cell carcinoma10.2
9embryoma10.2
10pneumothorax10.2
11turcot syndrome10.1CTNNB1
12ovarian small cell carcinoma10.1TTF1
13superior vena cava syndrome10.1CGB
14subependymal giant cell astrocytoma10.1SYP
15melanotic neuroectodermal tumor10.1SYP
16pineoblastoma10.1SYP
17dysembryoplastic neuroepithelial tumor10.1SYP
18choroid plexus papilloma10.1TP53
19bronchitis10.1
20hypercalcemia10.1
21small cell carcinoma10.1
22bronchogenic carcinoma10.1
23laryngitis10.1
24thyroiditis10.1
25embryonal sarcoma10.1
26pilocytic astrocytoma10.1SYP
27granulosa cell tumor of the ovary10.1NKX2-1, CTNNB1
28adenomatoid tumor10.1VIM
29giant cell glioblastoma10.1EGFR
30thymus cancer10.1SYP, NKX2-1
31merkel cell carcinoma10.1NKX2-1, SYP
32benign mesothelioma10.1TP53, NKX2-1
33tubular adenocarcinoma10.1SYP, CTNNB1
34teratoma10.1CGB, NKX2-1
35neuroendocrine carcinoma10.1SYP, NKX2-1
36pseudosarcomatous fibromatosis10.1CTNNB1, VIM
37nephroblastoma10.1CTNNB1, TP53
38sex cord-gonadal stromal tumor10.1VIM, NKX2-1
39glomus tumor10.0VIM, SYP
40abrikosov's tumor10.0VIM, NKX2-1
41fibromatosis10.0VIM, CTNNB1
42adrenal adenoma10.0CTNNB1, SYP
43meningioma10.0VIM, SYP
44sertoli cell tumor10.0SYP, VIM
45granular cell tumor10.0VIM, SYP
46gallbladder cancer10.0KRAS, TP53
47lynch syndrome10.0KRAS, TP53
48rhabdoid tumor10.0SYP, VIM
49thyroid cancer, anaplastic10.0CTNNB1, NKX2-1, TP53
50synovial sarcoma10.0VIM, CTNNB1

Graphical network of the top 20 diseases related to Pulmonary Blastoma:



Diseases related to pulmonary blastoma

Symptoms for Pulmonary Blastoma

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Sources:
50Orphanet
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Symptoms:

50 (show all 10)
  • abnormal pleura/hydrothorax/pleuresia/pleural effusion/chylothorax
  • fever/chilling
  • weight loss/loss of appetite/break in weight curve/general health alteration
  • repeat respiratory infections
  • respiratory distress/dyspnea/respiratory failure/lung volume reduction
  • lung/bronchopulmonary neoplasm/tumor/carcinoma/cancer
  • hemoptysis
  • cough
  • thoracic/chest pain
  • radiologic lung abnormalities/changes

Drugs & Therapeutics for Pulmonary Blastoma

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Pulmonary Blastoma

Drug clinical trials:

Search ClinicalTrials for Pulmonary Blastoma

Search NIH Clinical Center for Pulmonary Blastoma

Search CenterWatch for Pulmonary Blastoma

Genetic Tests for Pulmonary Blastoma

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Anatomical Context for Pulmonary Blastoma

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Sources:
34MalaCards
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MalaCards organs/tissues related to Pulmonary Blastoma:

34
Lung, Fetal lung, Ovary, Thyroid, Liver, Brain, Lymph node, Bronchial epithelium

Animal Models for Pulmonary Blastoma or affiliated genes

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Sources:
38MGI
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MGI Mouse Phenotypes related to Pulmonary Blastoma:

38 (show all 21)
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00011868.5KRAS, EGFR, CTNNB1, TP53
2MP:00053718.3EGFR, CTNNB1, KRAS, TP53
3MP:00030128.2TP53, CTNNB1, NKX2-1, SYP, EGFR
4MP:00020068.2NKX2-1, KRAS, EGFR, CTNNB1, TP53
5MP:00053708.2EGFR, KRAS, CTNNB1, TP53
6MP:00053818.2EGFR, TP53, KRAS, NKX2-1, CTNNB1
7MP:00053798.1EGFR, TP53, KRAS, CTNNB1, NKX2-1
8MP:00053678.1CTNNB1, KRAS, TP53, EGFR
9MP:00028738.0SYP, CTNNB1, TP53, KRAS, EGFR
10MP:00053918.0EGFR, KRAS, VIM, TP53, CTNNB1
11MP:00053698.0TP53, VIM, KRAS, EGFR, CTNNB1
12MP:00053807.9KRAS, EGFR, TP53, CTNNB1, VIM
13MP:00053887.7CTNNB1, EGFR, KRAS, NKX2-1, TP53, VIM
14MP:00053977.7CTNNB1, EGFR, VIM, TP53, KRAS
15MP:00053897.7TP53, EGFR, KRAS, NKX2-1, CTNNB1, VIM
16MP:00053857.7NKX2-1, KRAS, EGFR, CTNNB1, VIM, TP53
17MP:00053847.6VIM, NKX2-1, KRAS, EGFR, TP53, CTNNB1
18MP:00053787.5TP53, VIM, NKX2-1, CTNNB1, EGFR, KRAS
19MP:00053767.5CTNNB1, VIM, EGFR, NKX2-1, TP53, KRAS
20MP:00036317.3TP53, VIM, NKX2-1, KRAS, EGFR, CTNNB1
21MP:00107687.1NKX2-1, KRAS, EGFR, VIM, TP53, CTNNB1

Publications for Pulmonary Blastoma

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Sources:
53PubMed
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Articles related to Pulmonary Blastoma:

(show top 50)    (show all 189)
idTitleAuthorsYear
1
Adult biphasic pulmonary blastoma. (23427368)
2013
2
18F-FDG PET/CT appearances of adult pulmonary blastoma. (23751833)
2013
3
Expression and mutation analysis of EGFR, c-KIT, and I^-catenin in pulmonary blastoma. (21292787)
2011
4
Pulmonary blastoma with yolk sac elements. (21045461)
2010
5
Pulmonary blastoma in a neonate: a lesion distinct from pleuropulmonary blastoma with unique cytogenetic features. (19902198)
2010
6
A rare association; pulmonary blastoma developing on extrapulmonary sequestration basis. (20395246)
2010
7
Pulmonary blastoma metastatic to the ovary. (19047906)
2009
8
Clinical, pathological and immunohistochemical features of a pulmonary blastoma in a horse. (19202177)
2009
9
Biphasic pulmonary blastoma with rapid progression. (19214452)
2009
10
Pulmonary blastoma. (17686364)
2007
11
Combined small cell carcinoma with pulmonary blastoma and adenocarcinoma: case report and clonality analysis. (17046168)
2006
12
A patient with shortness of breath and a left hilar mass. Biphasic pulmonary blastoma. (16683903)
2006
13
Survival of biphasic pulmonary blastoma. (16332433)
2006
14
Solitary brain metastasis from classic biphasic pulmonary blastoma: a case report and review of the literature. (17202827)
2006
15
A rare case of classic biphasic pulmonary blastoma. (16447486)
2005
16
Cyopathologic and histologic features of biphasic pulmonary blastoma: a case report. (15717762)
2005
17
Biphasic pulmonary blastoma presenting as endobronchial polyp with a long stalk. (15942110)
2005
18
Pulmonary blastoma. (11563261)
2001
19
Pulmonary blastoma: report of two cases. (11381509)
2001
20
Extension of a pulmonary blastoma into the left atrium. (11393507)
2001
21
Pulmonary blastoma: report of a case. (10483763)
1999
22
Pulmonary blastoma: diagnostic and therapeutic aspects. (10415291)
1999
23
Case of extra pulmonary, pleuro-pulmonary blastoma in a child: pathological and cytogenetic findings. (9142208)
1997
24
Pulmonary blastoma. (9255019)
1997
25
Resection and adjuvant chemotherapy of pulmonary blastoma. A case report. (8697409)
1996
26
p53 and MDM2 immunostaining in pulmonary blastomas and bronchogenic carcinomas. (8666362)
1996
27
Pulmonary blastoma presenting as a solitary lip metastasis: case report and review of the literature. (7552426)
1995
28
Resection and adjuvant chemotherapy of pulmonary blastoma: a case report. (8635054)
1995
29
Pulmonary blastoma in an adult presenting as a chronic loculated effusion: a diagnostic problem. (8091334)
1994
30
Pulmonary blastoma--a case and review of literature. (8613168)
1994
31
Pleuropulmonary blastoma (pulmonary blastoma of childhood): genetic link with other embryonal malignancies? (8063284)
1994
32
Pulmonary blastoma: a rare case of childhood malignancy. (1323822)
1992
33
Childhood pulmonary blastoma: a pleuropulmonary variant of the adult-type pulmonary blastoma. (1720893)
1991
34
Pulmonary blastoma with a topographic transition from blastic to more differentiated areas. An immunohistochemical assessment of its embryonic nature using stage-specific embryonic antigens. (1661048)
1991
35
Pulmonary blastoma with neuroendocrine differentiation in cell morules resembling neuroepithelial bodies. (2124206)
1990
36
Pulmonary blastoma. (2176642)
1990
37
Pulmonary blastoma--report of a case. (2848950)
1988
38
Computed tomography of pulmonary blastoma in a child. (2991998)
1985
39
Pulmonary blastoma associated with cystic lung disease. (6895712)
1982
40
Pulmonary blastoma. An ultrastructural study of a case and its transplanted tumor in athymic nude mice. (7417940)
1980
41
Pulmonary blastoma. (158135)
1979
42
Cytopathologic findings in pulmonary blastoma. (295162)
1979
43
Pulmonary blastoma. (199865)
1977
44
Pulmonary blastoma: A light and electron microscopic study. (974996)
1976
45
Pulmonary blastoma: report of two cases. (1126201)
1975
46
Pulmonary blastoma. (4364385)
1974
47
Pulmonary blastoma. A case report and review of the literature. (4811746)
1974
48
Pulmonary blastoma. (4355012)
1973
49
Pulmonary blastoma with diverse mesenchymal proliferation. (5075621)
1972
50
Pulmonary blastoma with diverse metastases. (5351776)
1969

Variations for Pulmonary Blastoma

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Expression for genes affiliated with Pulmonary Blastoma

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Sources:
2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Pulmonary Blastoma

Search GEO for disease gene expression data for Pulmonary Blastoma.

Pathways for genes affiliated with Pulmonary Blastoma

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Sources:
51PathCards, 56Reactome, 39NCBI BioSystems Database, 61Thomson Reuters, 31KEGG, 13EMD Millipore, 54QIAGEN, 58SinoBiological, 55R&D Systems, 52PharmGKB, 62Tocris Bioscience
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Pathways related to Pulmonary Blastoma according to GeneCards/GeneDecks:

(show all 39)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.6CTNNB1, VIM
29.6CTNNB1, VIM
39.5CTNNB1, EGFR
49.4KRAS, EGFR
59.4KRAS, EGFR
6
Show member pathways
9.4EGFR, KRAS
7
Show member pathways
EGFR-dependent Endothelin signaling events39
9.4EGFR, KRAS
89.3CTNNB1, TP53
99.3CGB, TP53
10
Show member pathways
9.1EGFR, KRAS, CTNNB1
11
Show member pathways
9.1KRAS, EGFR, CTNNB1
12
Show member pathways
Cytoskeleton remodeling FAK signaling61
Development Endothelin 1 EDNRA transactivation of EGFR61
9.1CTNNB1, EGFR, KRAS
13
Show member pathways
9.1KRAS, CTNNB1, EGFR
148.9CTNNB1, KRAS, TP53
158.9CTNNB1, KRAS, TP53
16
Show member pathways
8.9TP53, CTNNB1, KRAS
178.9KRAS, TP53, CTNNB1
188.8CTNNB1, VIM, TP53
198.7CTNNB1, EGFR, TP53
208.7CTNNB1, EGFR, TP53
218.7EGFR, TP53, CTNNB1
228.7EGFR, CTNNB1, TP53
23
Show member pathways
Development EGFR signaling via PIP361
Development PDGF signaling via MAPK cascades61
Apoptosis and survival Anti apoptotic action of membrane bound ESR161
Signaling of Hepatocyte Growth Factor Receptor39
Development EGFR signaling via small GTPases61
Development Neurotrophin family signaling61
Apoptosis and survival NGF signaling pathway61
Apoptosis and survival Role of CDK5 in neuronal death and survival61
8.6KRAS, TP53, EGFR
24
Show member pathways
8.6TP53, EGFR, KRAS
258.6KRAS, EGFR, TP53
268.6TP53, EGFR, KRAS
27
Show member pathways
MAPK signaling pathway39
8.6TP53, EGFR, KRAS
28
Show member pathways
ErbB receptor signaling network39
ErbB signaling pathway39
8.6EGFR, KRAS, TP53
298.6EGFR, KRAS, TP53
308.5TP53, VIM, EGFR
31
Show member pathways
Signaling Pathways in Glioblastoma39
8.2CTNNB1, TP53, KRAS, EGFR
328.2TP53, KRAS, EGFR, CTNNB1
33
Show member pathways
8.2TP53, KRAS, EGFR, CTNNB1
348.2EGFR, TP53, KRAS, CTNNB1
35
Show member pathways
Signal transduction PTEN pathway61
8.2KRAS, EGFR, CTNNB1, TP53
368.2TP53, EGFR, CTNNB1, KRAS
378.2TP53, EGFR, CTNNB1, KRAS
38
Show member pathways
8.2EGFR, VIM, CTNNB1, TP53
398.1EGFR, KRAS, VIM, TP53

Compounds for genes affiliated with Pulmonary Blastoma

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Sources:
46Novoseek, 52PharmGKB, 12DrugBank, 25HMDB, 62Tocris Bioscience, 30IUPHAR, 3BitterDB
See all sources

Compounds related to Pulmonary Blastoma according to GeneCards/GeneDecks:

(show top 50)    (show all 92)
idCompoundScoreTop Affiliating Genes
1s 100469.7VIM, SYP
2panitumumab46 52 1211.7KRAS, EGFR
3mucicarmine469.7NKX2-1, SYP, VIM
4b 723469.6SYP, VIM, NKX2-1
5hematoxylin469.6NKX2-1, SYP, VIM
6cetuximab46 52 1211.5CTNNB1, EGFR, KRAS
7leucovorin46 52 1211.4CGB, EGFR, KRAS
8gnrh469.3CGB, CTNNB1, NKX2-1, VIM
94-hydroxytamoxifen469.3CTNNB1, VIM, EGFR
10crcs469.3TP53, CTNNB1, KRAS
11bleomycin46 1210.2CTNNB1, CGB, TP53
12roscovitine469.2CTNNB1, VIM, TP53
13imatinib46 52 1211.1EGFR, CTNNB1, KRAS
14nocodazole46 1210.0VIM, CTNNB1, TP53
15gefitinib46 52 1211.0KRAS, EGFR, CTNNB1, VIM
16cyclic amp46 2510.0SYP, NKX2-1, CGB, VIM
17vincristine46 52 1211.0TP53, CGB, VIM
18adpribose469.0TP53, EGFR, CTNNB1
19geldanamycin46 52 62 1211.9EGFR, TP53, CTNNB1
20oxaliplatin46 52 1210.9KRAS, EGFR, TP53
21temozolomide46 129.9TP53, EGFR, VIM
22adriamycin468.9CTNNB1, TP53, VIM
23formaldehyde46 259.9VIM, SYP, NKX2-1
24rapamycin468.9VIM, KRAS, EGFR, CTNNB1
25irinotecan46 52 1210.9EGFR, KRAS, TP53
26genistein46 30 62 3 25 1213.9VIM, SYP, EGFR, CTNNB1
27phosphoinositide468.9VIM, KRAS, EGFR, CTNNB1
28egcg468.8CTNNB1, EGFR, TP53
29etoposide46 52 62 1211.7SYP, CGB, TP53, VIM
30thymidylate468.7EGFR, TP53, VIM
31butyrate468.7TP53, KRAS, CTNNB1, VIM
325fluorouracil468.6KRAS, EGFR, CTNNB1, TP53
33testosterone46 62 25 1211.6EGFR, VIM, CGB, CTNNB1, SYP
34mg 13246 629.6EGFR, CTNNB1, TP53
35ly294002468.6EGFR, TP53, KRAS, CTNNB1
36retinoid468.6TP53, CTNNB1, EGFR, VIM
37sb 20358046 629.5EGFR, VIM, TP53, CTNNB1
38tamoxifen46 52 30 1211.5CTNNB1, EGFR, TP53, VIM
39doxorubicin46 52 1210.5CTNNB1, EGFR, VIM, TP53
40oligonucleotide468.4KRAS, TP53, VIM, NKX2-1, CTNNB1
41h2o2468.3SYP, CTNNB1, TP53, VIM, KRAS
42cycloheximide468.2CTNNB1, TP53, VIM, EGFR
43paclitaxel46 52 1210.1CTNNB1, TP53, VIM, KRAS, EGFR
44cisplatin46 52 62 1210.9EGFR, KRAS, SYP, CTNNB1, CGB, TP53
45progesterone46 30 62 25 1211.5TP53, VIM, CGB, EGFR, NKX2-1, CTNNB1
46paraffin467.4SYP, CTNNB1, EGFR, KRAS, NKX2-1, VIM
47retinoic acid46 258.4VIM, SYP, NKX2-1, KRAS, EGFR, CTNNB1
48serine467.4EGFR, TP53, SYP, CTNNB1, NKX2-1, KRAS
49vegf467.4TP53, CTNNB1, EGFR, KRAS, SYP, VIM
50estrogen467.1VIM, TP53, SYP, NKX2-1, KRAS, EGFR

GO Terms for genes affiliated with Pulmonary Blastoma

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Sources:
17Gene Ontology
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Cellular components related to Pulmonary Blastoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1nucleoplasmGO:0056548.5CTNNB1, NKX2-1, TP53, TTF1

Biological processes related to Pulmonary Blastoma according to GeneCards/GeneDecks:

(show all 11)
idNameGO IDScoreTop Affiliating Genes
1epithelial tube branching involved in lung morphogenesisGO:06044110.0CTNNB1, NKX2-1
2regulation of long-term neuronal synaptic plasticityGO:0481699.8KRAS, SYP
3cerebral cortex cell migrationGO:0217959.8EGFR, NKX2-1
4cellular component disassembly involved in execution phase of apoptosisGO:0069219.6CTNNB1, VIM
5T cell differentiation in thymusGO:0330779.4CTNNB1, TP53
6positive regulation of MAP kinase activityGO:0434069.3EGFR, KRAS
7negative regulation of DNA replicationGO:0081569.3TP53, TTF1
8axon guidanceGO:0074119.1NKX2-1, KRAS, EGFR
9cellular response to drugGO:0356908.7EGFR, TP53
10apoptotic processGO:0069158.6TP53, VIM, CTNNB1, CGB
11positive regulation of transcription from RNA polymerase II promoterGO:0459448.5TP53, NKX2-1, EGFR, CTNNB1

Molecular functions related to Pulmonary Blastoma according to GeneCards/GeneDecks:

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1glycoprotein bindingGO:0019489.3EGFR, VIM
2protein complex bindingGO:0324039.3CTNNB1, KRAS, SYP
3transcription regulatory region DNA bindingGO:0442129.1TP53, NKX2-1, CTNNB1
4protein phosphatase bindingGO:0199038.9CTNNB1, EGFR, TP53
5enzyme bindingGO:0198998.6TP53, EGFR, CTNNB1
6protein kinase bindingGO:0199018.4CTNNB1, EGFR, TP53
7chromatin bindingGO:0036828.3TTF1, TP53, EGFR, CTNNB1
8identical protein bindingGO:0428028.2EGFR, SYP, VIM, TP53

Products for genes affiliated with Pulmonary Blastoma

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Pulmonary Blastoma

About this section
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