TGCT
MCID: PGM001
MIFTS: 60

Pigmented Villonodular Synovitis (TGCT) malady

Rare diseases, Bone diseases, Muscle diseases, Cancer diseases categories
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Summaries for Pigmented Villonodular Synovitis

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43NIH Rare Diseases, 65Wikipedia, 33MalaCards
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NIH Rare Diseases:43 Pigmented villonodular synovitis is a disease in which the tissue lining a joint in the body begins to grow abnormally.  there are two types of pigmented villonodular synovitis: the nodular form (where the abnormal tissue growth forms a single bump) and the diffuse form (where the entire lining of the joint grows unusually).  the additional tissue in the joint can cause pain, limit movements or cause the joint to lock, and in some cases, can destroy the normal joint structure.  the knee is most commonly affected by this condition, though it can occur in other joints such as the hip, shoulder, elbow, ankle, or wrist.  the average age of diagnosis for this condition is 35 years. last updated: 11/28/2011

MalaCards: Pigmented Villonodular Synovitis, also known as localized pigmented villonodular synovitis, is related to synovitis and giant cell tumor. An important gene associated with Pigmented Villonodular Synovitis is IBSP (integrin-binding sialoprotein), and among its related pathways are Syndecan-1-mediated signaling events and Osteopontin Signaling. The compounds pyridinoline and ibandronate have been mentioned in the context of this disorder. Affiliated tissues include bone, lung and testes, and related mouse phenotypes are craniofacial and limbs/digits/tail.

Wikipedia:65 Pigmented villonodular synovitis (PVNS) is a joint disease characterized by inflammation and overgrowth... more...

Aliases & Classifications for Pigmented Villonodular Synovitis

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8Disease Ontology, 43NIH Rare Diseases, 10DISEASES, 45Novoseek, 49Orphanet, 62UMLS, 58SNOMED-CT, 40NCIt, 35MeSH, 36MESH via Orphanet, 59SNOMED-CT via Orphanet, 63UMLS via Orphanet
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Classifications:

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


Aliases & Descriptions:

pigmented villonodular synovitis 8 43 10 49 62
localized pigmented villonodular synovitis 43
diffuse giant cell tumor of tenosynovium 8
diffuse pigmented villonodular synovitis 43
fibrous histiocytoma of tendon sheath 62
synovitis pigmented villonodular 45
tenosynovial giant cell tumors 43
diffuse-type giant cell tumor 49
tenosynovial giant cell tumor 49
villous tenosynovitis 8
diffuse-type gct 49
tsgct 49
tgct 49


External Ids:

Disease Ontology8 DOID:2702
NCIt40 C3401
MeSH35 D013586
MESH via Orphanet36 D013586
SNOMED-CT via Orphanet59 95412009
UMLS via Orphanet63 C0039106

Related Diseases for Pigmented Villonodular Synovitis

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17GeneCards, 18GeneDecks
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Diseases related to Pigmented Villonodular Synovitis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 130)
idRelated DiseaseScoreTop Affiliating Genes
1synovitis32.5CD68, MMP1, TNFRSF11B, TNFSF11
2giant cell tumor31.5TNFSF11, CSF1R, CSF1
3osteonecrosis30.9BGLAP
4arthritis30.8BGLAP, TNFSF11, TNFRSF11B, MMP9, MMP1, ACP5
5rheumatoid arthritis30.7TNFSF11, IBSP, BGLAP, SPP1, TNFRSF11B, MMP9
6osteoarthritis30.1MMP1, MMP9, PTHLH, TNFRSF11B, TNFSF11, IBSP
7melanoma30.0SPP1, IBSP, TNFSF11, TNFRSF11B, MMP9, MMP1
8villonodular synovitis11.7
9tenosynovial giant cell tumor10.8
10malignant giant cell tumor10.6
11neuropathy10.6
12hemarthrosis10.5
13carpal tunnel syndrome10.5
14tuberculosis10.5
15synovial chondromatosis10.5
16male germ cell tumor, somatic10.4
17neurofibromatosis10.3
18malignant giant cell tumor of the tendon sheath10.3
19nodular tenosynovitis10.3
20chondroblastoma10.3
21peroneal neuropathy10.3
22ulnar neuropathy10.3
23noonan syndrome10.3
24systemic lupus erythematosus10.3
25bone giant cell tumor10.3
26cervicitis10.3
27conjunctivitis10.3
28hemangioma10.3
29lipoma10.3
30lupus erythematosus10.3
31paraplegia10.3
32sarcoma10.3
33noonan-like/multiple giant cell lesion syndrome10.3
34soft tissue sarcoma10.3
35spastic paraparesis10.3
36spasticity10.3
37polyploidy10.3
38germ cell tumors10.2
39testicular germ cell tumor10.2
40multicentric reticulohistiocytosis10.1CD68
41camurati-engelmann disease10.1BGLAP
42cherubism10.1TNFSF11
43periapical granuloma10.1TNFSF11
44pseudohypoparathyroidism type 1b10.1PTHLH, BGLAP
45osteopetrosis autosomal dominant type 210.1BGLAP, TNFSF11
46paget disease, juvenile10.1TNFSF11, TNFRSF11B
47polyostotic osteolytic dysplasia, hereditary expansile10.1TNFSF11, TNFRSF11B
48fibrous dysplasia10.1IBSP, BGLAP
49pseudohypoparathyroidism10.1PTHLH, BGLAP
50osteosclerosis10.1TNFRSF11B, TNFSF11

Graphical network of the top 20 diseases related to Pigmented Villonodular Synovitis:



Diseases related to pigmented villonodular synovitis

Symptoms for Pigmented Villonodular Synovitis

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Drugs & Therapeutics for Pigmented Villonodular Synovitis

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42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Drug clinical trials:

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Genetic Tests for Pigmented Villonodular Synovitis

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Anatomical Context for Pigmented Villonodular Synovitis

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33MalaCards
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MalaCards organs/tissues related to Pigmented Villonodular Synovitis:

33
Bone, Lung, Testes

Animal Models for Pigmented Villonodular Synovitis or affiliated genes

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37MGI
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Publications for Pigmented Villonodular Synovitis

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52PubMed
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Articles related to Pigmented Villonodular Synovitis:

(show top 50)    (show all 488)
idTitleAuthorsYear
1
Low-dose external beam radiotherapy as a postoperative treatment for patients with diffuse pigmented villonodular synovitis of the knee: 4 recurrences in 23 patients followed for mean 9 years. (22489888)
2012
2
Hip joint pigmented villonodular synovitis in a young girl: a case report. (22433958)
2012
3
Pigmented villonodular synovitis of the ankle presenting as a persisting ankle effusion. (22764668)
2012
4
The management of diffuse-type giant cell tumour (pigmented villonodular synovitis) and giant cell tumour of tendon sheath (nodular tenosynovitis). (22733940)
2012
5
One step open synovectomy without adjuvant therapy for diffuse pigmented villonodular synovitis of the knee in a soccer player. (23738243)
2011
6
Destructive synovial hemangioma of the hip resembling pigmented villonodular synovitis. (20570091)
2011
7
Pigmented villonodular synovitis of the temporomandibular joint: case report and review of the literature. (21310347)
2011
8
Radiation therapy for treatment of pigmented villonodular synovitis: results of a national patterns of care study. (20488628)
2010
9
Arthroscopic treatment of pigmented villonodular synovitis involving bilateral shoulders. (20806759)
2010
10
Pigmented villonodular synovitis: an overdiagnosed entity. (19817903)
2009
11
Tenosynovial giant cell tumor and pigmented villonodular synovitis: a proposal for unification of these clinically distinct but histologically and genetically identical lesions. (17225151)
2007
12
Pigmented villonodular synovitis of the elbow in a 6-year-old girl: a case report. (17429130)
2007
13
Radial and ulnar neuropathy due to pigmented villonodular synovitis of the elbow. (17126239)
2006
14
Pigmented villonodular synovitis. (16311109)
2006
15
Similarities between giant cell tumor of bone, giant cell tumor of tendon sheath, and pigmented villonodular synovitis concerning ultrastructural cytochemical features of multinucleated giant cells and mononuclear stromal cells. (16825116)
2006
16
Clustered localized pigmented villonodular synovitis. (15944639)
2005
17
Pigmented villonodular synovitis of the knee: disease of the popliteus tendon and posterolateral compartment. (16012505)
2005
18
Pigmented villonodular synovitis: a disease in evolution. (15290750)
2004
19
Incidental detection of pigmented villonodular synovitis on FDG PET. (12897655)
2003
20
Pigmented villonodular synovitis of the anserine bursa. (12817733)
2003
21
Pigmented villonodular synovitis of the knee presenting as sciatica. (12943290)
2003
22
Tuberculosis of the hip as the presenting sign of HIV and simulating pigmented villonodular synovitis. (12107577)
2002
23
Langerhans cell granulomatosis manifested as pigmented villonodular synovitis. (12132822)
2001
24
Arthroscopic treatment of pigmented villonodular synovitis of the shoulder. (11154374)
2001
25
Giant cell tumor of tendon sheath, tenosynovial giant cell tumor, and pigmented villonodular synovitis: defining the presentation, surgical therapy and recurrence. (10671695)
2000
26
Dynamic MR-imaging in the diagnosis of pigmented villonodular synovitis of the knee. (11093605)
2000
27
Magnetic resonance imaging evaluation and arthroscopic resection of localized pigmented villonodular synovitis of the knee. (10791586)
2000
28
Lipoma induced by pigmented villonodular synovitis of the ankle. (10359296)
1999
29
An unusual case of pigmented villonodular synovitis of the spine: benign aggressive and/or malignant? (10371292)
1999
30
Pigmented villonodular synovitis of the foot and ankle: a review of six cases. (10353773)
1999
31
Pigmented villonodular synovitis and related lesions: the spectrum of imaging findings. (9888766)
1999
32
Cytokine and matrix metalloproteinase expression in pigmented villonodular synovitis may mediate bone and cartilage destruction. (9807705)
1998
33
Diffuse pigmented villonodular synovitis of the knee associated with chronic ACL deficiency. (9606698)
1998
34
Total knee arthroplasty in patients who have pigmented villonodular synovitis. (9469312)
1998
35
Pigmented villonodular synovitis of the elbow. (9524343)
1998
36
Articular pigmented villonodular synovitis of the MP joint of the hand. (8543887)
1995
37
Diagnostic features of diffuse pigmented villonodular synovitis of the knee. (8118978)
1994
38
Pigmented villonodular synovitis of the temporomandibular joint. (8228609)
1993
39
Pigmented villonodular synovitis of the knee demonstrated on bone scan. Correlation with US, CT, and MRI. (1424384)
1992
40
Evaluation of the hip with pigmented villonodular synovitis. A case report. (1735210)
1992
41
Pigmented villonodular synovitis and synovial cysts of the spine [ comment]. (1595437)
1992
42
Case report 578: Pigmented villonodular synovitis of the shoulder. (2321043)
1990
43
Pigmented villonodular synovitis in a vertebra. A case report. (2365726)
1990
44
Diffuse pigmented villonodular synovitis of the temporomandibular joint diagnosed by fine-needle aspiration cytology. (2791838)
1989
45
Case report 563: Pigmented villonodular synovitis (PVNS) of knee. (2814557)
1989
46
Pigmented villonodular synovitis of the knee: results from 13 cases. (2706054)
1989
47
A case of aggressive pigmented villonodular synovitis. (3421087)
1988
48
Lipid microspherules in synovial fluid of patients with pigmented villonodular synovitis. (3190786)
1988
49
Pigmented villonodular synovitis of the hip--association with osteochondromatosis. (3440329)
1987
50
Pigmented villonodular synovitis of the temporomandibular joint: a case report. (10994414)
1982

Variations for Pigmented Villonodular Synovitis

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Expression for genes affiliated with Pigmented Villonodular Synovitis

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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Pigmented Villonodular Synovitis

Search GEO for disease gene expression data for Pigmented Villonodular Synovitis.

Pathways for genes affiliated with Pigmented Villonodular Synovitis

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50PathCards, 38NCBI BioSystems Database, 53QIAGEN, 60Thomson Reuters, 55Reactome, 30KEGG
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Pathways related to Pigmented Villonodular Synovitis according to GeneCards/GeneDecks:

(show all 23)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.9MMP9, MMP1
29.9SPP1, MMP9
3
Show member pathways
RANKL/RANK Signaling Pathway38
Apoptosis and survival APRIL and BAFF signaling60
9.8ACP5, TNFRSF11B, TNFSF11
49.7CD68, CSF1
5
Show member pathways
Apoptosis and survival TNFR1 signaling pathway60
TWEAK Signaling Pathway38
Apoptosis Modulation by HSP7038
HIV-1 Nef- Negative effector of Fas and TNF-alpha38
9.7MMP9, TNFRSF11B, TNFSF11
69.7MMP1, MMP9, BGLAP
79.7MMP9, PTHLH, SPP1
89.6SPP1, BGLAP, MMP9
99.5CSF1, CSF1R, SPP1
109.5SPP1, TNFSF11, TNFRSF11B, ACP5
119.5TNFRSF11B, TNFSF11, BGLAP, SPP1
129.5CSF1R, CSF1, PDGFRB
139.5PDGFRB, CSF1, CSF1R
149.4IBSP, TNFSF11, TNFRSF11B, PDGFRB
15
Show member pathways
9.4SPP1, IBSP, MMP9, MMP1
169.3LAPTM5, ACP5, CD68
179.3PTHLH, TNFSF11, IBSP, BGLAP, SPP1
189.2PDGFRB, CSF1R, MMP1, MMP9
199.1TNFSF11, TNFRSF11B, ACP5, CSF1R, CSF1
209.0SPP1, IBSP, CSF1R, CSF1, PDGFRB
21
Show member pathways
9.0TNFSF11, TNFRSF11B, CSF1R, CSF1, PDGFRB
22
Show member pathways
8.9PDGFRB, CSF1, CSF1R, TNFSF11, SPP1
238.6CSF1, ACP5, MMP1, TNFSF11, ATP6V1B2

Compounds for genes affiliated with Pigmented Villonodular Synovitis

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45Novoseek, 11DrugBank, 51PharmGKB, 24HMDB, 3BitterDB, 61Tocris Bioscience, 29IUPHAR
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Compounds related to Pigmented Villonodular Synovitis according to GeneCards/GeneDecks:

(show top 50)    (show all 174)
idCompoundScoreTop Affiliating Genes
1pyridinoline4510.6TNFRSF11B
2ibandronate45 1110.8TNFRSF11B, IBSP, BGLAP, TNFSF11
3deoxypyridinoline459.7TNFSF11, IBSP, ACP5, BGLAP, TNFRSF11B
4pamidronate45 51 1111.6BGLAP, TNFSF11, TNFRSF11B, PTHLH, MMP1
5tartrate459.6CSF1, PTHLH, TNFSF11, BGLAP, ACP5
6alendronate45 51 1111.6PTHLH, TNFRSF11B, TNFSF11, MMP1, BGLAP
7vitamin k2459.5BGLAP, TNFSF11, TNFRSF11B, CSF1
8clodronate45 51 1111.5MMP1, TNFSF11, TNFRSF11B, PTHLH, BGLAP, ACP5
9titanium459.5SPP1, IBSP, ACP5, TNFRSF11B, TNFSF11, BGLAP
1025-hydroxyvitamin d459.4PTHLH, TNFSF11, TNFRSF11B, ACP5, BGLAP, SPP1
11phosphorus459.4BGLAP, PTHLH, SPP1, TNFSF11, ACP5, TNFRSF11B
12infliximab45 51 1111.4MMP1, TNFRSF11B, BGLAP, SPP1, TNFSF11
13hydroxyapatite459.2IBSP, TNFSF11, BGLAP, SPP1, TNFRSF11B, PTHLH
14hydroxyproline45 24 1111.2ACP5, MMP9, PTHLH, TNFRSF11B, IBSP, MMP1
15zoledronic acid459.2BGLAP, IBSP, TNFSF11, TNFRSF11B, PTHLH, MMP9
16azathioprine45 3 51 1112.1PCNA, PTHLH, TNFRSF11B, BGLAP, TNFSF11, ACP5
17geranylgeranyl pyrophosphate459.1TNFRSF11B, MMP9, MMP1, TNFSF11
18celecoxib45 61 29 51 24 1113.9TNFSF11, TNFRSF11B, MMP9, MMP1, PCNA, CSF1
19prostaglandin458.8PTHLH, MMP9, MMP1, CSF1, TNFRSF11B, TNFSF11
20tgf beta1458.8PTHLH, SPP1, TNFRSF11B, MMP1, CSF1, PDGFRB
21matrigel458.8IBSP, SPP1, PTHLH, MMP9, PDGFRB, MMP1
22rantes458.7MMP1, MMP9, TNFRSF11B, TNFSF11, CSF1, ACP5
23ns 39845 619.7BGLAP, TNFSF11, TNFRSF11B, MMP9, PCNA, MMP1
24pd 98,059458.7CSF1, CSF1R, MMP9, TNFSF11, IBSP, MMP1
25estradiol45 24 1110.7IBSP, ACP5, MMP1, TNFRSF11B, TNFSF11, PTHLH
261,25 dihydroxy vitamin d3458.6CSF1, CSF1R, ACP5, PTHLH, TNFRSF11B, TNFSF11
27nicotine45 29 51 1111.6MMP1, PTHLH, CSF1, SPP1, PCNA, MMP9
28indomethacin45 29 61 1111.6SPP1, TNFSF11, PTHLH, CSF1, TNFRSF11B, PCNA
29vitamin d458.6BGLAP, SPP1, CSF1R, TNFSF11, IBSP, PTHLH
30ascorbic acid45 249.5SPP1, CSF1, BGLAP, IBSP, TNFRSF11B, MMP1
31calcitriol45 61 24 1111.5TNFSF11, BGLAP, SPP1, TNFRSF11B, PTHLH, MMP1
32cyclosporin a45 29 6110.5TNFRSF11B, BGLAP, SPP1, TNFSF11, MMP9, MMP1
33cycloheximide458.4SPP1, MMP9, TNFRSF11B, TNFSF11, MMP1, IBSP
34procollagen458.4SPP1, BGLAP, IBSP, TNFSF11, TNFRSF11B, PCNA
35heparin45 29 24 1111.3PTHLH, BGLAP, TNFSF11, IBSP, SPP1, TNFRSF11B
36genistein45 29 61 3 24 1113.2PCNA, MMP9, SPP1, PDGFRB, TNFSF11, MMP1
37phosphatidylinositol458.2IBSP, SPP1, TNFRSF11B, PTHLH, TNFSF11, CSF1R
38h2o2458.1TNFSF11, PDGFRB, MMP1, BGLAP, SPP1, CSF1
39progesterone45 29 61 24 1112.1SPP1, TNFRSF11B, PTHLH, MMP1, CSF1R, CSF1
40pge2458.0TNFSF11, TNFRSF11B, PTHLH, MMP9, MMP1, ACP5
41creatinine457.8CSF1, PCNA, ACP5, MMP1, MMP9, PTHLH
42testosterone45 61 24 1110.8CSF1, TNFRSF11B, TNFSF11, PTHLH, MMP9, BGLAP
43tyrosine457.7CSF1, CSF1R, ACP5, MMP9, PTHLH, TNFRSF11B
44serine457.7TNFRSF11B, IBSP, BGLAP, SPP1, PTHLH, MMP9
45retinoic acid45 248.7CSF1R, PDGFRB, CSF1, PCNA, TNFRSF11B, MMP1
46dexamethasone45 51 29 1110.5CSF1, CSF1R, PCNA, ACP5, MMP1, MMP9
47vegf457.3IBSP, BGLAP, SPP1, TNFSF11, TNFRSF11B, PTHLH
48cysteine457.3CSF1R, TNFSF11, PDGFRB, TNFRSF11B, IBSP, BGLAP
49estrogen457.1IBSP, PDGFRB, CSF1, PTHLH, SPP1, BGLAP
50paraffin457.0ACP5, MMP1, MMP9, TNFSF11, IBSP, BGLAP

GO Terms for genes affiliated with Pigmented Villonodular Synovitis

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16Gene Ontology
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Cellular components related to Pigmented Villonodular Synovitis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1membrane-bounded vesicleGO:03198810.0BGLAP, SPP1
2extracellular regionGO:0055768.4MMP1, SPP1, BGLAP, IBSP, TNFSF11, TNFRSF11B
3extracellular spaceGO:0056158.3SPP1, BGLAP, TNFSF11, TNFRSF11B, PTHLH, MMP9

Biological processes related to Pigmented Villonodular Synovitis according to GeneCards/GeneDecks:

(show all 18)
idNameGO IDScoreTop Affiliating Genes
1regulation of bone resorptionGO:04512410.2CSF1R, BGLAP
2osteoclast proliferationGO:00215810.1TNFSF11, CSF1
3positive regulation of bone resorptionGO:04578010.1TNFSF11, SPP1
4response to vitamin DGO:03328010.1BGLAP, SPP1
5phosphatidylinositol metabolic processGO:04648810.0PDGFRB, CSF1R
6biomineral tissue developmentGO:03121410.0IBSP, SPP1
7osteoblast developmentGO:0020769.9BGLAP, PTHLH
8osteoclast differentiationGO:0303169.9CSF1, CSF1R, TNFSF11
9positive regulation of osteoclast differentiationGO:0456729.9TNFSF11, CSF1
10extracellular matrix disassemblyGO:0226179.8SPP1, MMP9, MMP1
11macrophage differentiationGO:0302259.8MMP9, CSF1R, CSF1
12skeletal system developmentGO:0015019.7MMP9, PTHLH, TNFRSF11B, BGLAP
13bone resorptionGO:0454539.7ACP5, TNFSF11
14ossificationGO:0015039.7CSF1, MMP9, TNFSF11, IBSP
15positive regulation of cell migrationGO:0303359.6CSF1R, CSF1, PDGFRB
16extracellular matrix organizationGO:0301989.5SPP1, IBSP, TNFRSF11B, MMP9, MMP1
17phosphatidylinositol-mediated signalingGO:0480159.4PDGFRB, CSF1R, PCNA
18positive regulation of cell proliferationGO:0082849.3PTHLH, CSF1R, CSF1, PDGFRB

Molecular functions related to Pigmented Villonodular Synovitis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cytokine activityGO:0051259.4CSF1, TNFRSF11B, TNFSF11, SPP1

Products for genes affiliated with Pigmented Villonodular Synovitis

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  • Antibodies
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Sources for Pigmented Villonodular Synovitis

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4CDC
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
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet