MPNST
MCID: MLG077
MIFTS: 64

Malignant Peripheral Nerve Sheath Tumor (MPNST) malady

Neuronal diseases, Cancer diseases categories

Summaries for Malignant Peripheral Nerve Sheath Tumor

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42NIH Rare Diseases, 63Wikipedia, 32MalaCards
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NIH Rare Diseases:42 A malignant peripheral nerve sheath tumor (mpnst) is a tumor that develops from nerve tissue.  the first symptom of mpnst is a lump or mass that increases in size, sometimes causing pain or a tingling sensation.  mpnst is considered an aggressive tumor because there is up to a 65% chance of the tumor regrowing after surgery (a recurrence), and approximately 40% chance of spreading to distant parts of the body (a metastasis), most commonly to the lung.  treatment of mpnst begins with surgery to remove as much of the tumor as possible.  radiation therapy may be used to decrease the chance of a recurrence.  chemotherapy might be used if the whole tumor cannot be removed during surgery, or to treat a metastasis.  mpnsts are quite rare, occurring in 0.001% of the general population.  approximately 25-50% of mpnsts are associated with a genetic condition known as neurofibromatosis type 1. last updated: 10/18/2011

MalaCards: Malignant Peripheral Nerve Sheath Tumor, also known as MPNST, is related to neurofibromatosis and neurofibroma. An important gene associated with Malignant Peripheral Nerve Sheath Tumor is S100B (S100 calcium binding protein B), and among its related pathways are GPCR Pathway and Glioblastoma Multiforme. The compounds estrogen and glucose have been mentioned in the context of this disorder. Affiliated tissues include lung, kidney and bone, and related mouse phenotypes are limbs/digits/tail and embryogenesis.

Wikipedia:63 A malignant peripheral nerve sheath tumor (MPNST) (also known as \"Malignant schwannoma,\"... more...

Aliases & Classifications for Malignant Peripheral Nerve Sheath Tumor

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48Orphanet, 8Disease Ontology, 42NIH Rare Diseases, 10DISEASES, 60UMLS, 44Novoseek, 56SNOMED-CT, 34MeSH, 39NCIt, 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
malignant peripheral nerve sheath tumor:
Inheritance: Sporadic; Age of onset: Variable


Aliases & Descriptions:

malignant peripheral nerve sheath tumor 8 42 10 48 60
mpnst 42 44 48
malignant neoplasm of the peripheral nerve sheath 8
malignant peripheral nerve sheath tumors 44
malignant neurilemmoma 48
malignant neurofibroma 48
malignant schwannoma 48
neurogenic sarcoma 48
neurofibrosarcoma 48


External Ids:

Disease Ontology8 DOID:5940
MeSH34 D018317
NCIt39 C3798
SNOMED-CT via Orphanet57 404037002, 19897006, 77418004

Related Diseases for Malignant Peripheral Nerve Sheath Tumor

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17GeneCards, 18GeneDecks
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Diseases in the Malignant Peripheral Nerve Sheath Tumor family:

Benign Peripheral Nerve Sheath Tumor

Diseases related to Malignant Peripheral Nerve Sheath Tumor via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 223)
idRelated DiseaseScoreTop Affiliating Genes
1neurofibromatosis31.8KIT, MMP13, NGFR, NF1, NF2, PDGFRA
2neurofibroma31.4NF1, NGFR, MMP13, KIT, NF2, TOP2A
3sarcoma31.2S100B, S100A1, PDGFRA, BMP2, TOP2A, NES
4melanoma31.1S100A6, S100A1, SOX10, PDGFRA, BMP2, TOP2A
5plexiform neurofibroma31.1MMP13, NF1, PDGFRA
6synovial sarcoma31.1CALB2, S100B, NES, NF1, MMP13, KIT
7ganglioneuroma31.0S100B, CHGA, NGFR
8perineurioma30.9S100B
9neurofibrosarcoma30.9NF2, NF1
10neurilemmoma30.7NF2, NF1, SMARCB1
11neuroblastoma30.6NGFR
12angiosarcoma30.6PDGFRA, CALB2
13malignant mesothelioma30.6CALB2, S100A1, NF2, CDKN2A
14pheochromocytoma30.6NGFR, NF1, CHGA, CALB2
15gastrointestinal stromal tumor30.6KIT, PDGFRA
16smooth muscle tumor30.4KIT, S100A1, S100B
17liposarcoma30.4CALB2
18neuropathy30.4MYOG, SOX10
19papillary carcinoma30.4CHGA, S100A1, S100A6, CALB2
20rhabdomyosarcoma30.4CALB2, S100B, S100A1, PDGFRA, NES, MYOG
21seminoma30.4CALB2, PDGFRA, TOP2A, KIT
22adenocarcinoma30.4CALB2, S100A6, BMP2, TOP2A, NES, CHGA
23bladder carcinoma30.4MMP13, CDKN2A, NGFR, TOP2A
24lung cancer30.4CHGA, TOP2A, BMP2, PDGFRA
25medulloblastoma29.9PDGFRA, BMP2, NES, NGFR, SMARCB1, S100B
26thymoma29.8CALB2, NGFR
27ovarian cancer29.8CALB2, PDGFRA, TOP2A, NF2, NGFR, MMP13
28leiomyosarcoma29.8SMARCB1, KIT, CALB2, S100B, S100A1, PDGFRA
29epithelioid malignant peripheral nerve sheath tumor11.0
30peripheral nerve schwannoma10.9
31malignant triton tumor10.8
32cervicitis10.5
33malignant glandular tumor of peripheral nerve sheath10.4
34hepatitis a10.4
35hepatitis10.4
36thyroiditis10.4
37malignant cardiac peripheral nerve sheath neoplasm10.3
38malignant oculomotor nerve tumor10.3
39malignant peritoneal mesothelioma10.3
40malignant spindle cell melanoma10.3
41cellular schwannoma10.3
42plexiform schwannoma10.3
43malignant epithelial mesothelioma10.3
44cervix melanoma10.3
45cerebritis10.3
46bladder carcinoma in situ10.3
47endocervicitis10.3
48childhood malignant schwannoma10.3
49meningocele10.3
50intussusception10.3

Graphical network of the top 20 diseases related to Malignant Peripheral Nerve Sheath Tumor:



Diseases related to malignant peripheral nerve sheath tumor

Clinical Features for Malignant Peripheral Nerve Sheath Tumor

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Drugs & Therapeutics for Malignant Peripheral Nerve Sheath Tumor

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Malignant Peripheral Nerve Sheath Tumor

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Anatomical Context for Malignant Peripheral Nerve Sheath Tumor

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32MalaCards
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MalaCards organs/tissues related to Malignant Peripheral Nerve Sheath Tumor:

32
Lung, Kidney, Bone, Breast, Cervix, Brain, Thyroid, Colon, Pancreas, Liver, Skin, Spinal cord, Tongue, Smooth muscle, Prostate, Heart, Uterus, Testes, Eye, Lymph node, Trachea

Animal Models for Malignant Peripheral Nerve Sheath Tumor or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Malignant Peripheral Nerve Sheath Tumor:

36 (show all 22)
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000537110.7SOX10
2MP:000538010.7SOX10, BMP2, NES, NF2, MYOG
3MP:001077110.7S100B, SOX10, PDGFRA, MYOG, CDKN2A, MMP13
4MP:000118610.7NF1
5MP:000537710.6APOD, BMP2, NF2, NF1, NGFR
6MP:000538810.6PDGFRA, PRG2, NF2, NF1, MYOG, NGFR
7MP:000363110.6CALB2, SOX10, APOD, BMP2, NF2, NF1
8MP:000537610.6PDGFRA, APOD, NF2, NF1, MYOG, NGFR
9MP:000539010.6PDGFRA, BMP2, NF2, NF1, MYOG, MMP13
10MP:000287310.5CALB2, S100B, S100A1, SOX10, PDGFRA, BMP2
11MP:000537810.5CHGA, NES, BMP2, PDGFRA, SOX10, NF2
12MP:000538610.5KIT, CALB2, S100B, S100A1, SOX10, PDGFRA
13MP:000536910.5PDGFRA, NF1, NGFR, CDKN2A
14MP:000538110.5SOX10, PDGFRA, BMP2, NGFR, CDKN2A, KIT
15MP:000538510.5S100A1, PDGFRA, BMP2, CHGA, NF1, NGFR
16MP:001076810.5NES, BMP2, APOD, PDGFRA, SOX10, CHGA
17MP:000200610.4PDGFRA, NF2, NF1, CDKN2A, KIT, SMARCB1
18MP:000538410.4SOX10, PDGFRA, BMP2, NES, NF1, NGFR
19MP:000537910.3SOX10, PDGFRA, NF2, NF1, CDKN2A, KIT
20MP:000536710.3KIT, NGFR, NF1, NF2, CHGA, PDGFRA
21MP:000538210.2PDGFRA, BMP2, NF2, NF1, NGFR, KIT
22MP:000537010.0NF2, NF1, NGFR, CDKN2A, KIT, SMARCB1

Publications for Malignant Peripheral Nerve Sheath Tumor

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50PubMed
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Articles related to Malignant Peripheral Nerve Sheath Tumor:

(show top 50)    (show all 433)
idTitleAuthorsYear
1
Malignant peripheral nerve sheath tumor as a cause of chronic cardiac insufficiency in cattle. (23369465)
2013
2
Imaging diagnosis-malignant peripheral nerve sheath tumor presenting as an intra-axial brain mass in a young dog. (23363000)
2013
3
Primary intrapulmonary malignant peripheral nerve sheath tumor mimicking lung cancer. (23991328)
2013
4
Expression profiling of 519 kinase genes in matched malignant peripheral nerve sheath tumor/plexiform neurofibroma samples is discriminatory and identifies mitotic regulators BUB1B, PBK and NEK2 as overexpressed with transformation. (23370767)
2013
5
Malignant peripheral nerve sheath tumor of non-neurofibromatosis type I metastasized to the cerebrospinal axis. (23634272)
2013
6
Clinical and molecular prognostic predictors of malignant peripheral nerve sheath tumor. (23749326)
2013
7
Low-grade malignant triton tumor in the lumbar spine: a rare variant of malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation. (21732991)
2012
8
Malignant peripheral nerve sheath tumor of the superior cervical sympathetic ganglia. (23076858)
2012
9
Ras-driven transcriptome analysis identifies aurora kinase A as a potential malignant peripheral nerve sheath tumor therapeutic target. (22811580)
2012
10
Telomere maintenance mechanisms in malignant peripheral nerve sheath tumors: expression and prognostic relevance. (22516689)
2012
11
Favorable outcome after radical resection and subsequent local irradiation of malignant peripheral nerve sheath tumor in the cervical spine. (23006884)
2012
12
Report of a case of malignant peripheral nerve sheath tumor of the neck with an early local recurrence. (22033963)
2011
13
Orthotopic xenografting of human luciferase-tagged malignant peripheral nerve sheath tumor cells for in vivo testing of candidate therapeutic agents. (21460792)
2011
14
Discovery of a small molecule targeting IRA2 deletion in budding yeast and neurofibromin loss in malignant peripheral nerve sheath tumor cells. (21697395)
2011
15
Malignant peripheral nerve sheath tumor in a patient with juvenile-onset dermatomyositis. (21441814)
2011
16
Malignant transformation in a hybrid schwannoma/perineurioma: addition to the spectrum of a malignant peripheral nerve sheath tumor. (22234123)
2011
17
Cervicothoracic malignant peripheral nerve sheath tumor. (21674466)
2011
18
Malignant peripheral nerve sheath tumor of the vagus nerve in a teenager with the neurofibromatosis 1 gene mutation: a case report. (21843710)
2011
19
Endocervical fibroblastic malignant peripheral nerve sheath tumor (neurofibrosarcoma): report of a novel entity possibly related to endocervical CD34 fibrocytes. (21317712)
2011
20
Lymph node metastasis of malignant peripheral nerve sheath tumor in the absence of widespread disease five years after diagnosis: a rare finding. (21151396)
2010
21
Liposarcomatous differentiation in malignant peripheral nerve sheath tumor: a case report. (19433348)
2010
22
Clinical course of a malignant peripheral nerve sheath tumor in a Siberian tiger (Panthera tigris altaica). (21088186)
2010
23
Abrogating drug resistance in malignant peripheral nerve sheath tumors by disrupting hyaluronan-CD44 interactions with small hyaluronan oligosaccharides. (19470767)
2009
24
Fine needle aspiration cytology of a primary malignant peripheral nerve sheath tumor arising in the parotid gland: a case report. (19697728)
2009
25
Malignant Peripheral Nerve Sheath Tumor: molecular pathogenesis and current management considerations. (18286466)
2008
26
Isolated malignant peripheral nerve sheath tumor of kidney capsule. (19259060)
2008
27
Ras signaling influences permissiveness of malignant peripheral nerve sheath tumor cells to oncolytic herpes. (18988803)
2008
28
Imatinib mesylate inhibits cell invasion of malignant peripheral nerve sheath tumor induced by platelet-derived growth factor-BB. (17558420)
2007
29
Multicentric malignant peripheral nerve sheath tumor. (17825749)
2007
30
Primary pulmonary malignant peripheral nerve sheath tumor: a case report. (17910355)
2007
31
A case of intra-dural malignant peripheral nerve sheath tumor in thoracic spine associated with neurofibromatosis type 1. (16598431)
2006
32
Malignant peripheral nerve sheath tumor originating in neurofibroma of the mesentery. Case report. (16334983)
2005
33
Malignant peripheral nerve sheath tumor of the orbit: case report and literature review. (16145592)
2004
34
Giant malignant peripheral nerve sheath tumor of the neck in a patient with neurofibromatosis-1. (15488983)
2004
35
Primary malignant peripheral nerve sheath tumor of the cauda equina in a child case report. (15001982)
2004
36
Malignant peripheral nerve sheath tumor of the mediastinum: a temporary aortic transection approach. (15457164)
2004
37
Transcriptional mRNA of BMP-2, 3, 4 and 5 in trigeminal nerve, benign and malignant peripheral nerve sheath tumors. (11642720)
2001
38
Multiple recurrences in malignant peripheral nerve sheath tumor of the orbit: a case report and a review of the literature. (11476181)
2001
39
Image interpretation session: 1999. Intraosseous malignant peripheral nerve sheath tumor (malignant schwannoma) in a patient with neurofibromatosis. (10682790)
2000
40
Images in cardiovascular medicine. A malignant peripheral nerve sheath tumor presenting as an intrapericardial mass. (10929747)
2000
41
Histopathologic features of malignant peripheral nerve sheath tumor are not restricted to metastatic malignant melanoma and can be found in primary malignant melanoma also. (11023110)
2000
42
Metastatic malignant melanoma resembling malignant peripheral nerve sheath tumor: report of 16 cases. (10584703)
1999
43
Solitary malignant peripheral nerve sheath tumor of the pancreas. (10228853)
1999
44
Malignant peripheral nerve sheath tumor of the prostate: a rare manifestion of neurofibromatosis type 1. (10379768)
1999
45
Malignant peripheral nerve sheath tumor with perineurial cell differentiation (malignant perineurioma). (10227727)
1999
46
Small round-cell type of malignant peripheral nerve sheath tumor. (9720503)
1998
47
Malignant peripheral nerve sheath tumor: analysis of treatment outcome. (9788415)
1998
48
A resected case of malignant peripheral nerve sheath tumor (MPNST) necessary to distinguish from Askin tumor]. (9884581)
1998
49
Recurrent orbital malignant peripheral nerve sheath tumor 18 years after initial resection. (9430299)
1997
50
Malignant peripheral nerve sheath tumor. An immunohistochemical study of 62 cases. (2435144)
1987

Genetic Variations for Malignant Peripheral Nerve Sheath Tumor

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Expression for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

Search GEO for disease gene expression data for Malignant Peripheral Nerve Sheath Tumor.

Pathways for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

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51QIAGEN
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Pathways related to Malignant Peripheral Nerve Sheath Tumor according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
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10.4PDGFRA, BMP2, NGFR, MMP13
210.4PDGFRA, NF2, NF1, CDKN2A
3
Hide members
10.4KIT, NGFR, PRG2, BMP2, PDGFRA
4
Hide members
10.3KIT, SOX10, PDGFRA, BMP2, PRG2, NF1

Compounds for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

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44Novoseek, 49PharmGKB, 11DrugBank, 28IUPHAR, 24HMDB, 59Tocris Bioscience, 2BitterDB
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Compounds related to Malignant Peripheral Nerve Sheath Tumor according to GeneCards/GeneDecks:

(show top 50)    (show all 74)
idCompoundScoreTop Affiliating Genes
1estrogen4410.8CALB2, NF1, MYOG
2glucose4410.8CDKN2A, NGFR, MYOG
3forskolin44 49 1112.8NGFR, MYOG
4b 7234410.7CALB2, S100B, S100A1, CHGA
5dopamine44 28 11 2413.7CALB2, BMP2, NES, CHGA, NF1, NGFR
6fatty acid4410.7S100A6, TOP2A, NF1, MYOG, CDKN2A
7mucicarmine4410.7CALB2, S100B, S100A1
8agarose4410.7S100B, APOD, CDKN2A, MMP13, KIT
9silver4410.7CALB2, S100A6, S100A1, CHGA, CDKN2A
10serine4410.7S100B, BMP2, TOP2A, NF2, NF1, MYOG
11paclitaxel44 49 1112.7CALB2, S100B, TOP2A, CHGA, CDKN2A
12hematoxylin4410.7CALB2, S100B, S100A1, PRG2, CHGA, NGFR
13matrigel4410.7MMP13, NGFR, CHGA, NES, BMP2
14gaba4410.7CALB2, S100B, NES, CHGA, NGFR
15creatinine4410.7APOD, BMP2, CHGA, CDKN2A
16rapamycin4410.7PDGFRA, BMP2, NF2, NF1, MYOG, KIT
17thymidine44 2411.7CALB2, S100B, PDGFRA, BMP2, TOP2A, NGFR
18steroid4410.7CALB2, S100A6, BMP2, TOP2A, NF1, MYOG
19acetylcholine44 49 28 11 2414.7CALB2, S100B, S100A1, NES, PRG2, CHGA
20actinomycin d4410.7BMP2, TOP2A, PRG2, CDKN2A, MMP13, KIT
21oxygen44 2411.7S100B, BMP2, NES, PRG2, CHGA, MYOG
22p0024410.7TOP2A, CHGA, MMP13, KIT
23bromodeoxyuridine4410.6MYOG, CHGA, NES, S100B
24cyclic amp44 2411.6S100B, CHGA, NF1, NGFR, CDKN2A, MMP13
25ribonucleic acid4410.6BMP2, CHGA, MYOG, MMP13, KIT
26choline44 11 2412.6CALB2, S100B, NES, NGFR
27vegf4410.6S100B, PDGFRA, BMP2, TOP2A, NES, CHGA
28zinc44 2411.6CALB2, S100B, S100A6, S100A1, PRG2, CHGA
29lipid4410.6S100B, APOD, BMP2, NES, PRG2, CHGA
30atp44 2811.6S100B, S100A6, PDGFRA, TOP2A, PRG2, CHGA
31dexamethasone44 49 28 1113.6S100B, BMP2, CDKN2A, MMP13
32testosterone44 59 11 2413.6APOD, S100A6, S100B, CALB2, BMP2, TOP2A
33estracyt4410.6CHGA, PRG2
34oligonucleotide4410.6PRG2, BMP2, APOD, PDGFRA, S100A6, CHGA
35nitric oxide44 11 2412.6CALB2, S100B, S100A1, BMP2, NES, PRG2
36nicotine44 49 28 1113.6BMP2, CHGA, NGFR, CDKN2A
37genistein44 28 59 2 11 2415.6MMP13, CDKN2A, PRG2, TOP2A, BMP2
38cysteine4410.6PRG2, BMP2, S100A1, S100A6, S100B, CHGA
39progesterone44 59 28 11 2414.6CALB2, KIT, CDKN2A, NGFR, S100B, S100A6
40tyrosine4410.5TOP2A, BMP2, PDGFRA, S100A1, S100B, CALB2
41calcium44 49 11 2413.5BMP2, PDGFRA, S100A1, S100A6, S100B, CALB2
42paraffin4410.5CALB2, S100B, S100A6, S100A1, PDGFRA, APOD
43retinoic acid44 2411.5CHGA, NF1, MYOG, NGFR, CDKN2A, MMP13
44arginine4410.5S100B, BMP2, PRG2, CHGA, NF1, CDKN2A
45nacl4410.5S100A6, S100A1, NF1, MMP13
46deoxyribonucleic acid4410.5CDKN2A, NF2, BMP2
47ono 25065910.4PRG2, S100B
48threonine4410.4S100B, BMP2, NF2, NGFR, CDKN2A, KIT
49mog (35-55)5910.3PRG2, S100B
50nogo-66 (1-40)5910.1PRG2, S100B

GO Terms for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

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16Gene Ontology
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Cellular components related to Malignant Peripheral Nerve Sheath Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular regionGO:00557610.3APOD, BMP2, PRG2, CHGA, NGFR, MMP13
2nucleusGO:0056349.9SMARCB1, S100B, S100A6, S100A1, SOX10, PDGFRA

Biological processes related to Malignant Peripheral Nerve Sheath Tumor according to GeneCards/GeneDecks:

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1negative regulation of cell-matrix adhesionGO:00195310.6CDKN2A, NF1, NF2
2positive regulation of fibroblast proliferationGO:04814610.6S100A6, PDGFRA, NGFR
3positive regulation of apoptotic processGO:04306510.6NGFR, NF1, TOP2A, BMP2, S100B
4negative regulation of cell proliferationGO:00828510.5SMARCB1, CDKN2A, MYOG, NF2, BMP2
5negative regulation of protein kinase activityGO:00646910.5CDKN2A, NF1, NF2
6negative regulation of MAPK cascadeGO:04340910.4NF1, NF2
7positive regulation of transcription from RNA polymerase II promoterGO:04594410.4SOX10, BMP2, TOP2A, MYOG, CDKN2A, SMARCB1
8odontogenesis of dentin-containing toothGO:04247510.4PDGFRA, BMP2, NF2
9positive regulation of phospholipase C activityGO:01086310.3KIT, PDGFRA
10extracellular matrix organizationGO:03019810.1MMP13, NF1, BMP2, PDGFRA

Molecular functions related to Malignant Peripheral Nerve Sheath Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1protein homodimerization activityGO:04280310.3KIT, TOP2A, PDGFRA, S100A1, S100A6, S100B
2S100 protein bindingGO:04454810.3S100A1, S100A6, S100B
3protein bindingGO:0055159.8APOD, PDGFRA, SOX10, S100A1, S100A6, S100B

Products for genes affiliated with Malignant Peripheral Nerve Sheath Tumor

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  • Antibodies
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Sources for Malignant Peripheral Nerve Sheath Tumor

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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