MCID: PRV003
MIFTS: 52

Perivascular Epithelioid Cell Tumor malady

Cancer diseases category

Summaries for Perivascular Epithelioid Cell Tumor

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63Wikipedia, 32MalaCards
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Wikipedia:63 In oncology, PEComa, also PEC tumour and perivascular epithelioid cell tumour, is a family of... more...

MalaCards: Perivascular Epithelioid Cell Tumor, also known as neoplasm with perivascular epithelioid cell differentiation, is related to lymphangioleiomyomatosis and angiomyolipoma. An important gene associated with Perivascular Epithelioid Cell Tumor is ACTC1 (actin, alpha, cardiac muscle 1), and among its related pathways are Inhibition of TSC complex formation by PKB and Glioblastoma Multiforme. The compounds paclitaxel and monochloroacetic acid have been mentioned in the context of this disorder. Affiliated tissues include uterus, liver and colon, and related mouse phenotypes are pigmentation and muscle.

Aliases & Classifications for Perivascular Epithelioid Cell Tumor

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8Disease Ontology, 10DISEASES, 60UMLS, 39NCIt, 56SNOMED-CT, 34MeSH
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer diseases


Aliases & Descriptions:

perivascular epithelioid cell tumor 8 10
neoplasm with perivascular epithelioid cell differentiation 8
perivascular epithelioid cell neoplasms 60
pecoma 8


External Ids:

Disease Ontology8 DOID:2643
NCIt39 C38150
SNOMED-CT56 388601000
MeSH34 D054973

Related Diseases for Perivascular Epithelioid Cell Tumor

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17GeneCards, 18GeneDecks
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Diseases related to Perivascular Epithelioid Cell Tumor via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 180)
idRelated DiseaseScoreTop Affiliating Genes
1lymphangioleiomyomatosis30.8DES, TSC1, TSC2
2angiomyolipoma30.7TSC2, PMEL, VIM, CCL26, S100A1, MLANA
3pancreatitis30.6KITLG
4neuroblastoma30.4SYP
5angiosarcoma30.4VIM, MUC1, ACTC1
6acute leukemia30.4TSC2, KIT, KITLG, NTRK3
7leukemia30.3TFE3, NTRK3, CDKN2A, KITLG, KIT
8tuberous sclerosis30.3VIM, CCL26, S100B, RHEB, SYP, TSC1
9smooth muscle tumor30.3VIM, CALD1, S100B, S100A1, DES, KIT
10leiomyoma30.3VIM, CALD1, S100B, S100A1, DES, KIT
11gastrointestinal stromal tumor30.3ACTC1, KIT, VIM, S100B, S100A1, DES
12kidney angiomyolipoma30.1TSC1, TSC2
13epithelioid leiomyosarcoma30.1VIM, DES, ACTC1
14leiomyosarcoma30.1CALD1, S100B, S100A1, MLANA, DES, MUC1
15melanoma30.0PMEL, VIM, S100B, S100A1, MLANA, DES
16endometriosis29.9ACTC1
17liposarcoma29.9VIM, DES
18clear cell sarcoma29.9S100B, KIT
19sarcoma29.8VIM, CALD1, S100B, S100A1, TFE3, MLANA
20perivascular tumor11.3
21uterus perivascular epithelioid cell tumor10.9
22hepatitis10.5
23appendicitis10.3
24hepatitis a10.3
25benign perivascular tumor10.3
26cervicitis10.3
27diverticulitis10.3
28lymphoblastic leukemia10.3
29mesenchymal cell neoplasm10.3
30prostatitis10.3
31kidney cancer10.0TFE3
32hemangioma10.0ACTC1
33esophagitis10.0CCL26
34renal oncocytoma10.0S100A1
35von hippel-lindau disease10.0TSC2
36atopic dermatitis10.0CCL26
37fasciitis10.0ACTC1
38pneumothorax10.0TSC1
39uterine carcinosarcoma10.0KIT
40histiocytosis10.0S100B
41infantile myofibromatosis10.0DES
42sertoli-leydig cell tumor10.0MUC1
43pituitary adenoma10.0SYP
44carney triad10.0KIT
45pancreatic islet cell tumors10.0SYP
46melanotic neuroectodermal tumor10.0SYP
47cystadenocarcinoma10.0MUC1
48tuberous sclerosis complex10.0TSC1, TSC2
49oxyphilic adenoma10.0TFE3, S100A1
50adenomatoid tumor10.0VIM

Graphical network of the top 20 diseases related to Perivascular Epithelioid Cell Tumor:



Diseases related to perivascular epithelioid cell tumor

Clinical Features for Perivascular Epithelioid Cell Tumor

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Drugs & Therapeutics for Perivascular Epithelioid Cell Tumor

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Perivascular Epithelioid Cell Tumor

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Anatomical Context for Perivascular Epithelioid Cell Tumor

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32MalaCards
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MalaCards organs/tissues related to Perivascular Epithelioid Cell Tumor:

32
Uterus, Liver, Colon, Cervix, Kidney, Pancreas, Lymph node, Lung, Skin, Smooth muscle, Prostate, Heart, Appendix

Animal Models for Perivascular Epithelioid Cell Tumor or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Perivascular Epithelioid Cell Tumor:

36 (show all 15)
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00011869.6PMEL, TFE3, MLANA
2MP:00053699.6RHEB, CDKN2A, ACTC1, TSC1
3MP:00020069.1MUC1, CDKN2A, KITLG, KIT, TSC1, TSC2
4MP:00053709.0MUC1, CDKN2A, KITLG, KIT, TSC1, TSC2
5MP:00107718.9TSC2, PMEL, S100B, TFE3, CDKN2A, KITLG
6MP:00053768.7PMEL, RHEB, DES, KITLG, ACTC1, TSC1
7MP:00053808.6RHEB, CDKN2A, KITLG, KIT, TSC1, TSC2
8MP:00030128.5PMEL, MLANA, SYP, NTRK3, MUC1, CDKN2A
9MP:00053918.4PMEL, VIM, TFE3, NTRK3, MUC1, CDKN2A
10MP:00053868.2VIM, S100B, S100A1, DES, NTRK3, CDKN2A
11MP:00053858.1VIM, S100A1, RHEB, DES, NTRK3, KITLG
12MP:00028738.1S100B, S100A1, TFE3, RHEB, SYP, NTRK3
13MP:00036317.8VIM, RHEB, SYP, NTRK3, CDKN2A, KITLG
14MP:00053787.5VIM, CALD1, TFE3, RHEB, NTRK3, CDKN2A
15MP:00107687.3DES, RHEB, TFE3, CALD1, VIM, NTRK3

Publications for Perivascular Epithelioid Cell Tumor

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50PubMed
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Articles related to Perivascular Epithelioid Cell Tumor:

(show top 50)    (show all 120)
idTitleAuthorsYear
1
Management of perivascular epithelioid cell tumor of the liver: A case report and review of the literature. (24348838)
2014
2
A resected perivascular epithelioid cell tumor (PEComa) of the pancreas diagnosed using endoscopic ultrasound-guided fine-needle aspiration. (24042513)
2013
3
Perivascular epithelioid cell tumor of the liver: a case report and literature review. (23832736)
2013
4
MRI diagnosis of perivascular epithelioid cell tumor (PEComa) of the liver. (24068418)
2013
5
Pigmented perivascular epithelioid cell tumor of the liver: report of a rare case with brief review of literature. (23771383)
2013
6
A huge malignant perivascular epithelioid cell tumor (PEComa) of the uterine cervix and vagina. (24331571)
2013
7
Transcription factor E3 protein-positive perivascular epithelioid cell tumor of the appendix presenting as acute appendicitis: a case report and review of the literature. (23451753)
2013
8
Unusual myoid, perivascular, and postradiation lesions, with emphasis on atypical vascular lesion, postradiation cutaneous angiosarcoma, myoepithelial tumors, myopericytoma, and perivascular epithelioid cell tumor. (23327731)
2013
9
Perivascular epithelioid cell tumor of the cervix with malignant potential. (23850036)
2013
10
An unusual ileum tumor in a young woman. Perivascular epithelioid cell tumor of gastrointestinal tract. (22546372)
2012
11
Perivascular epithelioid cell tumor of the sigmoid colon with transcription factor E3 expression. (21546294)
2012
12
Malignant perivascular epithelioid cell tumor of the mesentery: a case report and literature review. (22414975)
2012
13
Sinonasal perivascular epithelioid cell tumor: benign or malignant neoplasm? (22643949)
2012
14
Perivascular epithelioid cell tumor (PEComa) of gynecologic origin: a clinicopathological study of three cases. (22439417)
2012
15
Bladder perivascular epithelioid cell tumor: a novel rare neoplasm. (22094379)
2012
16
Rare case of uterine PEC-oma (perivascular epithelioid cell tumor) recurrence. Case report and literature review. (22880484)
2012
17
Ovarian perivascular epithelioid cell tumor not otherwise specified with transcription factor E3 gene rearrangement: a case report and review of the literature. (22305236)
2012
18
Malignant perivascular epithelioid cell tumor in children: description of a case and review of the literature. (22703822)
2012
19
Perivascular epithelioid cell tumor of the ascending colon mesentery in a child: case report and review of the literature. (20385296)
2010
20
A perivascular epithelioid cell tumor of the stomach: an unsuspected diagnosis. (20101783)
2010
21
Molecular pathology of lymphangioleiomyomatosis and other perivascular epithelioid cell tumors. (20073603)
2010
22
Perivascular epithelioid cell tumors of the laryngopharynx: three case reports and literature review. (19409723)
2009
23
Perivascular epithelioid cell tumor (PEComa) of the liver diagnosed by contrast-enhanced ultrasonography. (20009396)
2009
24
Malignant variant of sclerosing perivascular epithelioid cell tumor arising in the adnexa. (19851212)
2009
25
A case of HMB45-negative perivascular epithelioid cell tumor (PEComa) of the uterine corpus: a possible diagnostic application of molecular-cytogenetic analysis. (19480261)
2009
26
Perivascular epithelioid cell tumor with SFPQ/PSF-TFE3 gene fusion in a patient with advanced neuroblastoma. (19606011)
2009
27
Primary perivascular epithelioid cell tumor (PEComa) of the liver: report of a case. (19784736)
2009
28
Growing perivascular epithelioid cell tumor of the liver studied with contrast-enhanced ultrasonography and magnetic resonance imaging. (19023006)
2008
29
Pancreatic perivascular epithelioid cell tumor (PEComa)]. (18675170)
2008
30
Uterine perivascular epithelioid cell tumor coexisting with pulmonary lymphangioleiomyomatosis and renal angiomyolipoma: a case report. (18528274)
2008
31
Perivascular epithelioid cell tumor of the orbit. (18625958)
2008
32
Perivascular epithelioid cell tumor of the rib. (18437415)
2008
33
Malignant perivascular epithelioid cell tumor ('PEComa'): a case report and literature review of cutaneous/subcutaneous presentations. (18005173)
2008
34
Perivascular epithelioid cell tumor (PEComa) of falciform ligament - an extremely rare tumor. (23133059)
2008
35
Perivascular epithelioid cell tumor of the liver: a report of two cases and review of the literature. (17907305)
2007
36
Perivascular epithelioid cell tumor (PEComa) in the transverse colon of an adolescent: a case report. (17455882)
2007
37
Perivascular epithelioid cell tumor (PEComa) of the kidney: MR features. (17066289)
2007
38
Malignant perivascular epithelioid cell tumor (PEComa) of the uterus with late renal and pulmonary metastases: a case report with review of the literature. (18053181)
2007
39
Perivascular epithelioid cell tumor (PEComa) of the liver: a case report and review of the literature]. (17370472)
2007
40
Tumors showing perivascular epithelioid cell differentiation: a clinicopathologic study of 39 cases]. (18261305)
2007
41
Perivascular epithelioid cell tumor of the oral mucosa. (15859645)
2005
42
Perivascular epithelioid cell tumor of the uterus: report of four cases. (16175079)
2005
43
Perivascular epithelioid clear cell tumor of the common bile duct. (15252321)
2004
44
Perivascular epithelioid cell tumor of the uterus: a case report. (15213607)
2004
45
First description of a PEComa (perivascular epithelioid cell tumor) of the colon: report of a case and review of the literature. (15540307)
2004
46
Perivascular epithelioid cell tumor (PEComa) of the uterine cervix associated with intraabdominal "PEComatosis": A clinicopathological study with comparative genomic hybridization analysis. (15494070)
2004
47
Perivascular epithelioid cell tumor of the uterus: immunohistochemical, ultrastructural and molecular study. (14629307)
2003
48
Recurrent perivascular epithelioid cell tumor of the uterus (PEComa): an immunohistochemical study and review of the literature. (13678746)
2003
49
Perivascular epithelioid cell tumor of the jejunum. (12650518)
2003
50
Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres: a novel member of the perivascular epithelioid clear cell family of tumors with a predilection for children and young adults. (10976698)
2000

Genetic Variations for Perivascular Epithelioid Cell Tumor

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Expression for genes affiliated with Perivascular Epithelioid Cell Tumor

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Perivascular Epithelioid Cell Tumor

Search GEO for disease gene expression data for Perivascular Epithelioid Cell Tumor.

Pathways for genes affiliated with Perivascular Epithelioid Cell Tumor

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53Reactome, 51QIAGEN, 12EMD Millipore, 37NCBI BioSystems Database, 52R&D Systems, 4Cell Signaling Technology, 29KEGG
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Pathways related to Perivascular Epithelioid Cell Tumor according to GeneCards/GeneDecks:

(show all 14)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.1TSC2, TSC1
29.8TSC2, TSC1, CDKN2A
3
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9.6TSC2, TSC1, RHEB
4
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9.6TSC2, TSC1, RHEB
59.6TSC2, TSC1, RHEB
6
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9.6S100B, KITLG, KIT, TSC2
7
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9.5KITLG, NTRK3, RHEB
89.4RHEB, NTRK3, TSC1, TSC2
9
Hide members
9.3VIM, CALD1, DES, ACTC1
109.3VIM, CALD1, DES, ACTC1
11
Hide members
9.2CCL26, NTRK3, KITLG, KIT, TSC1, TSC2
129.2RHEB, KITLG, KIT, TSC1, TSC2
13
Hide members
9.1VIM, NTRK3, KITLG, KIT, TSC1, TSC2
14
Hide members
8.7TSC2, TSC1, NTRK3, DES, RHEB, CCL26

Compounds for genes affiliated with Perivascular Epithelioid Cell Tumor

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44Novoseek, 49PharmGKB, 11DrugBank, 28IUPHAR, 24HMDB, 59Tocris Bioscience
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Compounds related to Perivascular Epithelioid Cell Tumor according to GeneCards/GeneDecks:

(show top 50)    (show all 78)
idCompoundScoreTop Affiliating Genes
1paclitaxel44 49 1112.1CDKN2A, S100B
2monochloroacetic acid4410.1KIT, VIM, KITLG
3eosin4410.1MUC1, VIM, S100B
4orcein4410.0S100B, DES, ACTC1, VIM
52-methoxyethanol4410.0ACTC1, S100A1, VIM
6creatinine4410.0CDKN2A, SYP, KITLG, ACTC1
7dexamethasone44 49 28 1112.9S100B, CDKN2A, KITLG
8glycogen44 2410.9SYP, KIT, ACTC1, TSC1, TSC2
9acetylcholine44 49 28 11 2413.9SYP, S100A1
10guanosine44 11 2411.8TSC2, TSC1, CDKN2A, VIM
11s 100449.6VIM, S100B, DES, SYP, MUC1, ACTC1
12reticulin449.6VIM, S100B, SYP, DES, MUC1, ACTC1
13toluidine449.6KIT, KITLG, DES, VIM, S100A1
14agarose449.5S100B, MUC1, CDKN2A, KITLG, KIT
15cytarabine44 49 1111.5KITLG, MUC1, VIM, KIT, CDKN2A
16actomyosin449.5S100A1, CALD1, VIM, ACTC1
17adriamycin449.5VIM, CDKN2A, MUC1, DES
18imatinib44 49 1111.5NTRK3, KITLG, KIT, ACTC1, DES, S100B
19ethanol44 49 11 2412.5MUC1, DES, S100B, KITLG, SYP
20steroid449.4MLANA, DES, CDKN2A, SYP, TSC2, ACTC1
21gold449.4DES, MUC1, CDKN2A, KIT, KITLG, SYP
22bromodeoxyuridine449.4VIM, S100B, DES, KITLG
23methotrexate44 49 1111.4KIT, CDKN2A, MUC1, VIM, KITLG
24silver449.4CDKN2A, PMEL, S100A1, DES, SYP
25formaldehyde44 2410.4ACTC1, KIT, MUC1, SYP, DES, S100B
26estradiol44 11 2411.3SYP, DES, VIM, KITLG, MUC1
27mucicarmine449.3SYP, DES, S100A1, S100B, VIM, MUC1
28b 723449.3VIM, S100B, S100A1, MUC1, ACTC1, DES
29actinomycin d449.3DES, CDKN2A, VIM, KITLG, KIT, MUC1
30cyclic amp44 2410.2S100B, CDKN2A, MUC1, SYP, VIM, DES
31lactate449.1VIM, S100B, KITLG, MLANA, KIT, DES
32biotin44 11 2411.1ACTC1, MUC1, DES, S100A1, VIM, KITLG
33rapamycin449.1KITLG, TSC2, TSC1, KIT, CCL26, VIM
34leucine449.1TSC2, S100B, SYP, VIM, TFE3, CDKN2A
35iron44 2410.1CDKN2A, KITLG, ACTC1, VIM, DES, S100B
36oxygen44 2410.0KITLG, TSC2, MUC1, DES, S100B, CCL26
37threonine448.9MUC1, CDKN2A, KIT, TSC1, TSC2, S100B
38doxorubicin44 49 1110.9DES, CDKN2A, KITLG, VIM, KIT
39nitric oxide44 11 2410.8DES, SYP, MUC1, CDKN2A, KITLG, KIT
40glucose448.8ACTC1, KITLG, CDKN2A, MUC1, RHEB, TFE3
41progesterone44 59 28 11 2412.7CDKN2A, VIM, S100B, S100A1, DES, SYP
42cysteine448.7CDKN2A, MUC1, SYP, S100A1, DES, KIT
43hematoxylin448.4ACTC1, KIT, S100B, S100A1, MLANA, DES
44testosterone44 59 11 2411.3MUC1, S100B, CDKN2A, NTRK3, SYP, DES
45tyrosine448.3TSC1, TSC2, S100A1, ACTC1, SYP, DES
46retinoic acid44 249.2KIT, ACTC1, KITLG, CDKN2A, MUC1, NTRK3
47vegf448.2MUC1, NTRK3, KITLG, SYP, DES, S100B
48serine448.1SYP, MUC1, VIM, TSC2, TSC1, KIT
49calcium44 49 11 2410.9CCL26, SYP, ACTC1, KIT, KITLG, CALD1
50paraffin447.7VIM, S100A1, MLANA, DES, S100B, SYP

GO Terms for genes affiliated with Perivascular Epithelioid Cell Tumor

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16Gene Ontology
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Cellular components related to Perivascular Epithelioid Cell Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1TSC1-TSC2 complexGO:03359610.1TSC2, TSC1
2intermediate filamentGO:0058829.8SYNM, DES, VIM
3protein complexGO:0432349.3S100A1, SYP, CDKN2A, TSC1
4cytoplasmGO:0057377.5VIM, S100B, TFE3, NTRK3, MUC1, CDKN2A

Biological processes related to Perivascular Epithelioid Cell Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1negative regulation of cell sizeGO:04579210.1TSC2, TSC1
2muscle filament slidingGO:0300499.7VIM, DES, ACTC1
3germ cell programmed cell deathGO:0352349.7KIT, KITLG
4cell cycle arrestGO:0070509.3TSC2, TSC1, CDKN2A, RHEB

Molecular functions related to Perivascular Epithelioid Cell Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1structural constituent of cytoskeletonGO:0052009.8SYNM, DES, VIM
2p53 bindingGO:0020399.7CDKN2A, MUC1, NTRK3
3S100 protein bindingGO:0445489.6S100A1, S100B
4identical protein bindingGO:0428029.1VIM, S100B, S100A1, DES, SYP
5protein bindingGO:0055156.7TSC2, PMEL, VIM, S100B, S100A1, MLANA

Products for genes affiliated with Perivascular Epithelioid Cell Tumor

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  • Antibodies
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Sources for Perivascular Epithelioid Cell 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