MCID: PGM001
MIFTS: 47

Pigmented Villonodular Synovitis malady

Categories: Rare diseases, Bone diseases, Reproductive diseases, Muscle diseases, Cancer diseases

Aliases & Classifications for Pigmented Villonodular Synovitis

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Sources:
65UMLS, 10Disease Ontology, 45NIH Rare Diseases, 12DISEASES, 51Orphanet, 47Novoseek, 36MeSH, 27ICD10, 42NCIt, 59SNOMED-CT, 37MESH via Orphanet, 66UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Pigmented Villonodular Synovitis:

Name: Pigmented Villonodular Synovitis 10 45 12 51 65
Localized Pigmented Villonodular Synovitis 45
Diffuse Pigmented Villonodular Synovitis 45
Diffuse Giant Cell Tumor of Tenosynovium 10
Fibrous Histiocytoma of Tendon Sheath 65
Synovitis, Pigmented Villonodular 36
Synovitis Pigmented Villonodular 47
Tenosynovial Giant Cell Tumors 45
 
Tenosynovial Giant Cell Tumor 51
Diffuse-Type Giant Cell Tumor 51
Testicular Germ Cell Tumor 65
Villous Tenosynovitis 10
Diffuse-Type Gct 51
Tsgct 51
Tgct 51

Classifications:



External Ids:

Disease Ontology10 DOID:2702
ICD1027 M12.2
MeSH36 D013586
NCIt42 C3401
Orphanet51 66627
MESH via Orphanet37 D013586
UMLS via Orphanet66 C0039106
UMLS65 C0039106

Summaries for Pigmented Villonodular Synovitis

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NIH Rare Diseases:45 Pigmented villonodular synovitis (pvns) is a disease in which the tissue lining the joints and tendons in the body (synovium) grows abnormally. it is characterized by a noncancerous mass or tumor. there are two types of pvns: the local or nodular form (where the tumor involves the tendons that support the joint, or in one area of the joint) and the diffuse form (where the entire lining of the joint is involved). symptoms might include: pain, limitation of movement, and locking of the joint. in some cases, the normal joint structure can be destroyed. the knee is most commonly affected by this condition, though it can occur in other joints such as the hip, shoulder, elbow, ankle, wrist, and rarely the jaw. the average age of diagnosis for this condition is 35 years. the cause of pvns is grossly unknown. treatment involves surgery to remove the tumor and damaged portions of the synovium. last updated: 4/4/2016

MalaCards based summary: Pigmented Villonodular Synovitis, also known as localized pigmented villonodular synovitis, is related to entropion and odontoma. An important gene associated with Pigmented Villonodular Synovitis is CSF1 (Colony Stimulating Factor 1), and among its related pathways are Macrophage Differentiation and Growth Inhibition by METS and FGF signaling pathway. Affiliated tissues include bone, breast and endothelial, and related mouse phenotypes are craniofacial and integument.

Wikipedia:68 Pigmented villonodular synovitis (PVNS) is a joint disease characterized by inflammation and overgrowth... more...

Related Diseases for Pigmented Villonodular Synovitis

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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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Drugs for Pigmented Villonodular Synovitis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 15)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1ImmunoglobulinsPhase 1, Phase 24477
2AntibodiesPhase 1, Phase 24477
3Grapefruit Seed ExtractNutraceuticalPhase 238
4
GemcitabineapprovedPhase 1188195058-81-460750
Synonyms:
103882-84-4
122111-03-9
2',2'-DiF-dC
2',2'-Difluoro-2'-deoxycytidine
2',2'-Difluorodeoxycytidine
2'-Deoxy-.beta.-D-2',2'-difluorocytidine
2'-Deoxy-2',2'-difluorocytidine
4-Amino-1-[(2R,4R,5R)-3,3-difluoro-4-hydroxy-5-(hydroxymethyl)oxolan-2-yl]pyrimidin-2-one
4-Amino-1-[3,3-difluoro-4-hydroxy-5-(hydroxymethyl) tetrahydrofuran-2-yl]-1H-pyrimidin-2-one
4-amino-1-((2R,4R,5R)-3,3-Difluoro-4-hydroxy-5-(hydroxymethyl)-tetrahydrofuran-2-yl)pyrimidin-2(1H)-one
4-amino-1-((2R,4R,5R)-3,3-difluoro-4-hydroxy-5-(hydroxymethyl)-tetrahydrofuran-2-yl)pyrimidin-2(1H)-one
95058-81-4
AB1004842
AC1L1TUQ
C07650
CCRIS 8984
CHEBI:175901
CHEMBL888
CID60750
Cytidine, 2'-deoxy-2',2'-difluoro-2'-Deoxy-.beta.-D-2',2'-difluorocytidine
D02368
DB00441
DDFC
DFDC
DFdC
DFdCyd
Folfugem
GEO
Gamcitabine
GemLip
Gemcel
 
Gemcin
Gemcitabin
Gemcitabina
Gemcitabina [INN-Spanish]
Gemcitabine
Gemcitabine (USAN/INN)
Gemcitabine HCl
Gemcitabine Hydrochloride
Gemcitabine hydrochloride
Gemcitabine stereoisomer
Gemcitabinum
Gemcitabinum [INN-Latin]
Gemtro
Gemzar
Gemzar (hydrochloride)
HMS2089P10
HSDB 7567
Inno-D07001
LS-59139
LY 188011
LY-188011
LY188011
NCGC00168784-01
NChemBio.2007.10-comp25
NSC 613327
NSC613327
TL8005979
UNII-B76N6SBZ8R
ZINC18279854
Zefei
gemcitabine
nchembio.573-comp7
nchembio.90-comp2
5
PaclitaxelapprovedPhase 1262133069-62-436314
Synonyms:
(2AR-(2aalpha,4beta,4abeta,6beta,9alpha(alpha r*,betas*),11alpha,12alpha,12balpha))-beta-(benzoylamino)-alpha-hydroxybenzenepropanoic acid 6,12b-bis(acetyloxy)-12-(benzoyloxy)-2a,3,4,4a,5,6,9,10,11,12,12a,12b-dodecahydro-4,11-dihydroxy-4a,8,13,13-tetramethyl-5-oxo-7,11-methano-1H-cyclodeca(3,4)benz(1,2-b)oxet-9-yl ester
12-benzoate, 9-ester with (2R,3S)-N-benzoyl-3-phenylisoserine
157069-30-2
33069-62-4
5beta,20-Epoxy-1,2-alpha,4,7beta,10beta,13alpha-hexahydroxytax-11-en-9-one 4,10-diacetate 2-benzoate 13-ester with (2R,3S)-N-benzoyl-3-phenylisoserine
7,11-Methano-1H-cyclodeca[3,4]benz[1,2-b]oxete, benzenepropanoic acid deriv.
7,11-Methano-5H-cyclodeca[3,4]benz[1,2-b]oxete,benzenepropanoic acid deriv.
7-Epi-Paclitaxel
7-Epi-Taxol
7-Epipaclitaxel
7-Epitaxol
7-epi-Paclitaxel
7-epi-Taxol
AB00513812
ABI 007
ABI-007
ABI007
AC-675
AC1L1IOG
AC1L1VJI
AC1L9AVF
ACon1_002231
ANX-513
Abraxane
Abraxane (TN)
Abraxane I.V. Suspension
Abraxis BioScience brand of albumin-bound paclitaxel
Ambotz33069-62-4
Anzatax
Asotax
BIDD:PXR0046
BMS 181339-01
BMS-181339
BMS-181339-01
BPBio1_000320
BRD-A23723433-001-01-2
BRD-A28746609-001-04-0
BRD-K62008436-001-03-1
BSPBio_000290
BSPBio_001152
BSPBio_002614
Bio-0076
Bio1_000362
Bio1_000851
Bio1_001340
Bio2_000416
Bio2_000896
Bristaxol
C07394
C466458
C47H51NO14
CCRIS 8143
CHEBI:103439
CHEBI:45863
CHEMBL100910
CHEMBL418410
CHEMBL48
CID36314
CID441276
CID4666
CID6713921
CID6915727
CPD-8718
Capxol
D00491
DB01229
DHP-107
DHP-208
DRG-0190
DTS-301
DivK1c_000441
EU-0101201
Ebetaxel
EmPAC
Epitaxol
Genaxol
Genetaxyl
Genexol
Genexol-PM
HMS1362J13
HMS1568O12
HMS1792J13
HMS1922K08
HMS1990J13
HMS2090D07
HMS2093K15
HMS501G03
HSDB 6839
I06-0014
IDI1_000441
IDI1_002171
Intaxel
KBio1_000441
KBio2_000492
KBio2_002016
KBio2_002509
KBio2_003060
KBio2_004584
KBio2_005077
KBio2_005628
KBio2_007152
KBio2_007645
KBio3_000903
KBio3_000904
KBio3_001834
KBio3_002987
KBioGR_000492
KBioGR_001893
 
KBioGR_002509
KBioSS_000492
KBioSS_002016
KBioSS_002517
LMPR0104390001
LS-31070
LipoPac
Lopac0_001201
MBT 0206
MEGxp0_001940
MLS000863266
MLS001077297
MLS002154218
MLS002172439
MLS002695976
MPI-5018
Micellar Paclitaxel
Mitotax
MolPort-001-742-627
MolPort-003-665-783
MolPort-003-932-365
NCGC00024995-02
NCGC00024995-03
NCGC00024995-04
NCGC00024995-05
NCGC00024995-06
NCGC00024995-07
NCGC00164367-01
NCGC00164367-02
NCGC00164367-03
NCI60_000601
NINDS_000441
NK 105
NP-010981
NSC 125973
NSC-125973
NSC125973
NSC358882
Nanotaxel
Neuro_000060
Nova-12005
OAS-PAC-100
OncoGel
Onxal
Onxol
Onxol, Taxol, Nov-Onxol, Paclitaxel
P1632
Paclical
Pacligel
Paclitaxel
Paclitaxel (JAN/USP)
Paclitaxel (JAN/USP/INN)
Paclitaxel (Taxol)
Paclitaxel [USAN:INN:BAN]
Paxceed
Paxene
Paxoral
Plaxicel
Praxel
Prestwick0_000155
Prestwick1_000155
Prestwick2_000155
Prestwick3_000155
Probes2_000350
QW 8184
S-8184 Paclitaxel Injectable Emulsion
S1150_Selleck
SDCCGMLS-0066823.P001
SDP-013
SMP1_000228
SMR000394086
SMR000857385
SPBio_000943
SPBio_002229
SPECTRUM1503908
ST50306996
Spectrum2_000872
Spectrum3_001057
Spectrum4_001197
Spectrum5_001491
Spectrum_001536
T 7402
T1912_SIGMA
T7191_SIGMA
T7402_SIGMA
TA1
TAXOL (TN)
TAXOL, 10-EPI,
TXL
TaxAlbin
Taxol
Taxol A
Taxol Konzentrat
Taxol.RTM. (Registered Trademark)
UNII-P88XT4IS4D
UPCMLD-DP108:001
UPCMLD-DP108:002
Vascular Wrap
Xorane
Yewtaxan
abi-007
albumin-bound paclitaxel
cMAP_000068
nchembio.188-comp1
nchembio.2007.34-comp9
nchembio.215-comp9
nchembio853-comp6
paclitaxel
weekly paclitaxel
6Tubulin ModulatorsPhase 14279
7Antiviral AgentsPhase 18071
8Immunologic FactorsPhase 118483
9Immunosuppressive AgentsPhase 110422
10Antimitotic AgentsPhase 14296
11Albumin-Bound PaclitaxelPhase 12621
12Anti-Infective AgentsPhase 117220
13AntimetabolitesPhase 19454
14Antimetabolites, AntineoplasticPhase 15770
15Antineoplastic Agents, PhytogenicPhase 14294

Interventional clinical trials:

idNameStatusNCT IDPhase
1PLX3397 Phase 3 Study for Pigmented Villonodular Synovitis (PVNS) or Giant Cell Tumor of the Tendon Sheath (GCT-TS)RecruitingNCT02371369Phase 3
2Study of Nilotinib Efficacy in Pigmented Villo-Nodular Synovitis/ Tenosynovial Giant Cell Tumour (PVNS/TGCT)CompletedNCT01261429Phase 2
3MCS110 in Patients With Pigmented Villonodular Synovitis (PVNS) or Giant Cell Tumor of the Tendon Sheath (GCTTS)RecruitingNCT01643850Phase 2
4Study of FPA008 in Patients With Pigmented Villonodular Synovitis / Diffuse Type Tenosynovial Giant Cell TumorRecruitingNCT02471716Phase 1, Phase 2
5Nilotinib in Patients With Relapsed or Metastatic Pigmented Villonodular Synovitis/Tenosynovial Giant Cell Tumor/Diffuse-Type Giant Cell TumorActive, not recruitingNCT01207492Phase 2
6A Study of PLX73086 in Advanced Solid Tumors and Locally Advanced or Refractory Tenosynovial Giant Cell TumorRecruitingNCT02673736Phase 1
7Phase 1 Study of PLX7486 as Single Agent and With Gemcitabine Plus Nab-Paclitaxel in Patients With Advanced Solid TumorsRecruitingNCT01804530Phase 1
8A Study of RO5509554 as Monotherapy and in Combination With Paclitaxel in Patients With Advanced Solid TumorsRecruitingNCT01494688Phase 1
9Safety Study of PLX108-01 in Patients With Solid TumorsActive, not recruitingNCT01004861Phase 1

Search NIH Clinical Center for Pigmented Villonodular Synovitis


Cochrane evidence based reviews: synovitis, pigmented villonodular

Genetic Tests for Pigmented Villonodular Synovitis

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Anatomical Context for Pigmented Villonodular Synovitis

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MalaCards organs/tissues related to Pigmented Villonodular Synovitis:

33
Bone, Breast, Endothelial, T cells, Lung, Liver, Skin

Animal Models for Pigmented Villonodular Synovitis or affiliated genes

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MGI Mouse Phenotypes related to Pigmented Villonodular Synovitis:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053828.9CSF1, CSF1R, PDGFRB, PTPN11
2MP:00107718.6CSF1, CSF1R, MMP9, PDGFRB, PTPN11
3MP:00053718.6CSF1, CSF1R, MMP9, PDGFRB, PTPN11
4MP:00053898.5CSF1, CSF1R, MMP9, PDGFRB, PTPN11
5MP:00053908.2CSF1, CSF1R, MMP9, PDGFRB, PTPN11
6MP:00053877.6CSF1, CSF1R, MMP1, MMP9, PDGFRB, PTPN11

Publications for Pigmented Villonodular Synovitis

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Articles related to Pigmented Villonodular Synovitis:

(show top 50)    (show all 546)
idTitleAuthorsYear
1
Efficacy and safety of brodalumab in patients with generalized pustular psoriasis and psoriatic erythroderma: results from a 52-week, open-label study. (27106510)
2016
2
POLD1 Germline Mutations in Patients Initially Diagnosed with Werner Syndrome. (26172944)
2015
3
ACVR1 p.Q207E causes classic fibrodysplasia ossificans progressiva and is functionally distinct from the engineered constitutively active ACVR1 p.Q207D variant. (24852373)
2014
4
Incongruities in the AAOMS Position Paper: Medication-Related Osteonecrosis of the Jaw-2014 Update. (25315315)
2014
5
Uteroglobin, a possible ligand of the lipoxin receptor inhibits serum amyloid A-driven inflammation. (24782597)
2014
6
Successful treatment of preadolescents with small cell carcinoma of the ovary hypercalcemic type. (23459374)
2013
7
Pathogenesis of coxsackievirus B3-induced myocarditis: role of macrophage migration inhibitory factor. (22340465)
2012
8
Intraoperative facial electromyography and brainstem auditory evoked potential findings in microvascular decompression for hemifacial spasm: correlation with postoperative delayed facial palsy. (22777492)
2012
9
Leiomyoma of the female urethra-a rare tumor: case report and review of the literature. (22844631)
2012
10
Polycystic ovary syndrome (PCOS)-like phenotypes in the d-galactose-induced aging mouse model. (23022527)
2012
11
Paraneoplastic encephalitis associated with myasthenia gravis and malignant thymoma. (22227397)
2012
12
Rosuvastatin in the prevention of stroke among men and women with elevated levels of C-reactive protein: justification for the Use of Statins in Prevention: an Intervention Trial Evaluating Rosuvastatin (JUPITER). (20026779)
2010
13
Association of TGFB1 -509 C>T polymorphism with breast cancer: evidence from a meta-analysis involving 23,579 subjects. (20232138)
2010
14
Involvement of genetic instability in the downregulation of sFRP1 in Chinese patients with hepatocellular carcinoma. (21046672)
2010
15
Hepatoprotective and antioxidant activity of two iridoids from Mussaenda 'dona aurora'. (19678533)
2009
16
Podoplanin expression in advanced-stage gastric carcinoma and prognostic value of lymphatic microvessel density. (18665758)
2008
17
Leber congenital amaurosis - a model for efficient genetic testing of heterogeneous disorders: LXIV Edward Jackson Memorial Lecture. (17964524)
2007
18
Acute interstitial pneumonia in feedlot cattle: effects of feeding feather meal or vitamin E. (17479779)
2007
19
Mouse Models of Lipodystrophy Key reagents for the understanding of the metabolic syndrome. (18193096)
2007
20
Transcript profiles of dendritic cells of PLOSL patients link demyelinating CNS disorders with abnormalities in pathways of actin bundling and immune response. (17530208)
2007
21
c-Met and hepatocyte growth factor: potential as novel targets in cancer therapy. (17288874)
2007
22
Nuclear expression of thymidylate synthase in colorectal cancer cell lines and clinical samples. (15956025)
2006
23
Anti-oxidant enzyme activities and expression and oxidative damage in patients with non-immediate reactions to drugs. (16879248)
2006
24
PTCH mutations in sporadic and Gorlin-syndrome-related odontogenic keratocysts. (16931872)
2006
25
Evaluation of the degree of clinical rheumatoid arthritis activity based on the concentrations of cytokines TNF-alpha, IL-12, IL-15, and IL-18 in serum and synovial fluid]. (16471244)
2006
26
Differentiation of the human mesenchymal stem cells derived from bone marrow and enhancement of cell attachment by fibronectin. (15901064)
2004
27
Evidence that nuclear factor-kappa B activation is critical in oxidant-induced disruption of the microtubule cytoskeleton and barrier integrity and that its inactivation is essential in epidermal growth factor-mediated protection of the monolayers of intestinal epithelia. (12815011)
2003
28
Drug addiction and HIV infection on rise in Tajikistan. (14570036)
2003
29
Stress and vascular responses: atheroprotective effect of laminar fluid shear stress in endothelial cells: possible role of mitogen-activated protein kinases. (12686737)
2003
30
Differences in leptin production by regional fat mass in postmenopausal women. (12402972)
2002
31
Effect of N,N'-dinitrosopiperazine on in vitro expression of human cytochrome P450 2E1. (12215299)
2002
32
Functional characterization of recombinant FV Hong Kong and FV Cambridge. (12091344)
2002
33
No association or linkage between polymorphisms in the genes encoding cholecystokinin and the cholecystokinin B receptor and panic disorder. (11244486)
2001
34
Interferon-associated retinopathy and cystoid macular edema. (11448335)
2001
35
Seroprevalence of herpes simplex virus type 1 and type 2 in selected German populations-relevance for the incidence of genital herpes. (10797375)
2000
36
Erythromycin use linked to pyloric stenosis. (10738394)
2000
37
In vitro and in vivo responses to short-term recombinant human insulin-like growth factor-1 (IGF-I) in a severely growth-retarded girl with ring chromosome 15 and deletion of a single allele for the type 1 IGF receptor gene. (10594514)
1999
38
Subunit asymmetry in the three-dimensional structure of a human CuZnSOD mutant found in familial amyotrophic lateral sclerosis. (9541385)
1998
39
Hyper insulinism and decreased insulin sensitivity in nonobese healthy offspring of conjugal diabetic parents and individuals with IGT and NIDDM. (9062808)
1996
40
Human aldose reductase: subtle effects revealed by rapid kinetic studies of the C298A mutant enzyme. (7578040)
1995
41
Low levels of hepatic cytochrome P-450 in Mexicans with uncomplicated cholelithiasis. (7839018)
1994
42
Elastin in the human posterior longitudinal ligament and spinal dura. A histologic and biochemical study. (7809748)
1994
43
Delta sleep-inducing peptide administration does not influence growth hormone and prolactin secretion in normal women. (8475226)
1993
44
Interstitial irradiation of a large, low grade ependymoma with stereotactically implanted iodine-125 seeds. Case report. (8333303)
1993
45
Structural characterization of a novel glycosyl-phosphatidylinositol from the protozoan Tetrahymena mimbres. (1659383)
1991
46
Glycoprotein Ib bioassays. Activity levels in Bernard-Soulier syndrome and in stored blood bank platelets. (2021317)
1991
47
Simultaneous presentation of rheumatoid arthritis and acute myeloid leukaemia masquerading as Felty's syndrome. (3465419)
1986
48
The relationship between dapsone dose, serum concentration and disease severity in dermatitis herpetiformis. (3954818)
1986
49
SOME MODIFICATIONS IN METHOD OF TREATING TETANUS NEONATORUM WITH INTERMITTENT POSITIVE-PRESSURE RESPIRATION. (14160093)
1964
50

Variations for Pigmented Villonodular Synovitis

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Expression for genes affiliated with Pigmented Villonodular Synovitis

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

Pathways for genes affiliated with Pigmented Villonodular Synovitis

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GO Terms for genes affiliated with Pigmented Villonodular Synovitis

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Biological processes related to Pigmented Villonodular Synovitis according to GeneCards Suite gene sharing:

(show all 11)
idNameGO IDScoreTop Affiliating Genes
1macrophage colony-stimulating factor signaling pathwayGO:003814510.4CSF1, CSF1R
2macrophage differentiationGO:003022510.3CSF1, CSF1R
3positive regulation of osteoclast differentiationGO:004567210.2CSF1, CSF1R
4phosphatidylinositol metabolic processGO:004648810.1CSF1R, PDGFRB
5regulation of phosphatidylinositol 3-kinase signalingGO:00140669.9PDGFRB, PTPN11
6platelet-derived growth factor receptor signaling pathwayGO:00480089.9PDGFRB, PTPN11
7osteoclast differentiationGO:00303169.9ACP5, CSF1, CSF1R
8ossificationGO:00015039.6ACP5, CSF1, MMP9
9dephosphorylationGO:00163119.2ACP5, PTPN11
10axon guidanceGO:00074119.0CSF1R, MMP9, PDGFRB
11innate immune responseGO:00450878.7CSF1, CSF1R, PDGFRB, PTPN11

Molecular functions related to Pigmented Villonodular Synovitis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1metalloendopeptidase activityGO:00042229.4MMP1, MMP9

Sources for Pigmented Villonodular Synovitis

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet