CCA
MCID: CHL065
MIFTS: 69

Cholangiocarcinoma (CCA) malady

Liver diseases, Cancer diseases, Rare diseases categories
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Summaries for Cholangiocarcinoma

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Wikipedia:65 Cholangiocarcinoma is a form of cancer that is composed of mutated epithelial cells (or cells showing... more...

MalaCards based summary: Cholangiocarcinoma, also known as adult primary cholangiocellular carcinoma, is related to gallbladder cancer and biliary papillomatosis, and has symptoms including hepatitis/icterus/cholestasis, abnormal colour of the urine/cholic/dark urines and extrahepatic biliary tract/gallbladder neoplasm/tumor/carcinoma/cancer. An important gene associated with Cholangiocarcinoma is PTPN3 (protein tyrosine phosphatase, non-receptor type 3), and among its related pathways are IL-9 Signaling Pathways and O-linked glycosylation of mucins. The compounds [alcl] and gemcitabine have been mentioned in the context of this disorder. Affiliated tissues include liver, small intestine and lymph node, and related mouse phenotypes are tumorigenesis and integument.

Disease Ontology:8 A bile duct adenocarcinoma that has material basis in bile duct epithelial cells.

Description from OMIM:46 615619

Aliases & Classifications for Cholangiocarcinoma

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Sources:
30LifeMap Discovery®, 8Disease Ontology, 10DISEASES, 44Novoseek, 48Orphanet, 46OMIM, 62UMLS, 39NCIt, 57SNOMED-CT, 34MeSH, 26ICD10 via Orphanet, 63UMLS via Orphanet, 35MESH via Orphanet
See all sources

Cholangiocarcinoma, Aliases & Descriptions:

Name: Cholangiocarcinoma 30 8 10 44 48 46 62
Adult Primary Cholangiocellular Carcinoma 8 62
Bile Duct Cancer 48 62
 
Adult Primary Cholangiocarcinoma 8
Cholangiosarcoma 8
Cca 48


Classifications:

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


Characteristics (Orphanet epidemiological data):

48
cholangiocarcinoma:
Inheritance: Sporadic; Prevalence: 1-9/100000; Age of onset: Adulthood


External Ids:

Disease Ontology8 DOID:4947
NCIt39 C4436
MeSH34 D018281
ICD10 via Orphanet26 C22.1
SNOMED-CT57 70179006, 312104005
UMLS via Orphanet63 C0206698, C0740277
OMIM46 615619
MESH via Orphanet35 D001650, D018281

Related Diseases for Cholangiocarcinoma

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Diseases related to Cholangiocarcinoma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 239)
idRelated DiseaseScoreTop Affiliating Genes
1gallbladder cancer31.3CDX2, CEACAM3, MUC2
2biliary papillomatosis31.2MUC2
3bile duct carcinoma30.8MAGEA3, CEACAM3
4primary biliary cirrhosis30.7KRT19, KRT7, MCL1
5bile duct adenoma30.6KRT19, KRT7
6colon adenocarcinoma30.6CDKN1B, KRT20, LGALS3, MUC5AC, MUC2
7spindle cell sarcoma30.6KRT7, MUC1
8liver cirrhosis30.5KRT19, MUC5AC, TIMP3, AFP
9lung squamous cell carcinoma30.5KRT19, TIMP3
10cystadenoma30.4KRT7, CEACAM3
11cystadenocarcinoma30.4CEACAM3, MUC5AC, MUC1, MUC2
12neuroendocrine carcinoma30.3CDX2, KRT19, KRT20, KRT7, MUC1
13parathyroid adenoma30.3CDKN1B, LGALS3
14glioblastoma30.1CDKN1B, MAGEA3, LGALS3, MCL1, TIMP3
15pancreatic cancer29.9CDX2, KRT19, KRT20, KRT7, CEACAM3, MUC5AC
16liver cancer29.7CDKN1B, CDX2, KRT19, KRT7, OGG1, MAGEA3
17adenocarcinoma29.5CDKN1B, CDX2, KRT19, KRT20, KRT7, OGG1
18melanoma29.4CDKN1B, CDX2, MAGEA3, LGALS3, CEACAM3, MUC4
19colorectal cancer29.4CDKN1B, CDX2, KRT20, KRT7, OGG1, LGALS3
20breast cancer29.3CDKN1B, CDX2, KRT19, KRT20, KRT7, LGALS3
21adenoma29.1CDKN1B, CDX2, KRT19, KRT20, KRT7, OGG1
22intrahepatic cholangiocarcinoma11.3
23hepatitis10.8
24hepatocellular carcinoma10.8
25cholangitis10.8
26sclerosing cholangitis10.7
27primary sclerosing cholangitis10.7
28ampulla of vater carcinoma10.6MUC2
29kidney cancer10.6KRT7
30inverted papilloma10.5KRT20
31nephrogenic adenoma10.5KRT7
32sertoli cell tumor10.5MUC1
33metanephric adenoma10.5KRT7
34hepatitis b10.5
35papilloma10.5MUC5AC, MUC2
36ovarian brenner tumor10.5KRT20, KRT7
37papillary adenocarcinoma10.5MUC1, MUC2
38opisthorchiasis10.5
39pancreatitis10.5
40acinar cell carcinoma10.5KRT7, AFP
41mammary paget's disease10.5KRT20, KRT7, MUC1
42dry eye syndrome10.5MUC5AC, MUC4
43pancreatic islet cell tumors10.5CEACAM3, AFP
44signet ring cell adenocarcinoma10.5KRT20, KRT7, MUC2
45epithelioid sarcoma10.5KRT7, MUC1
46secretory meningioma10.5KRT7, MUC1
47sertoli-leydig cell tumor10.5KRT7, MUC1, AFP
48abrikosov's tumor10.5KRT20, KRT7, MUC1
49bladder adenocarcinoma10.5CDX2, KRT20, KRT7
50congenital contractural arachnodactyly10.5

Graphical network of the top 20 diseases related to Cholangiocarcinoma:



Diseases related to cholangiocarcinoma

Symptoms for Cholangiocarcinoma

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Clinical features from OMIM:

615619

Symptoms:

48 (show all 9)
  • hepatitis/icterus/cholestasis
  • abnormal colour of the urine/cholic/dark urines
  • extrahepatic biliary tract/gallbladder neoplasm/tumor/carcinoma/cancer
  • pruritus/itching
  • asthenia/fatigue/weakness
  • acute abdominal pain/colic
  • anorexia
  • weight loss/loss of appetite/break in weight curve/general health alteration
  • fever/chilling

HPO human phenotypes related to Cholangiocarcinoma:

(show all 7)
id Description Frequency HPO Source Accession
1 abnormality of urine homeostasis hallmark (90%) HP:0003110
2 biliary tract neoplasm hallmark (90%) HP:0100574
3 pruritus typical (50%) HP:0000989
4 weight loss occasional (7.5%) HP:0001824
5 abdominal pain occasional (7.5%) HP:0002027
6 anorexia occasional (7.5%) HP:0002039
7 abnormality of temperature regulation occasional (7.5%) HP:0004370

Drugs & Therapeutics for Cholangiocarcinoma

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Drug clinical trials:

Search ClinicalTrials for Cholangiocarcinoma

Search NIH Clinical Center for Cholangiocarcinoma

Genetic Tests for Cholangiocarcinoma

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Anatomical Context for Cholangiocarcinoma

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MalaCards organs/tissues related to Cholangiocarcinoma:

32
Liver, Small intestine, Lymph node, Colon, Endothelial, Brain, Skeletal muscle, Thyroid, Ovary, Neutrophil, T cells, Kidney, Lung, Pancreas, Breast, Prostate, Testis, Testes, Myeloid, Monocytes, Fetal liver

Animal Models for Cholangiocarcinoma or affiliated genes

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MGI Mouse Phenotypes related to Cholangiocarcinoma:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020069.4CDKN1B, AFP, MUC2, MUC1, OGG1, KRT19
2MP:00107719.4MCL1, MUC1, LGALS3, KRT19, CDKN1B, TIMP3
3MP:00053819.3MUC2, MUC1, MUC5AC, LGALS3, KRT19, CDX2
4MP:00053799.3AFP, TIMP3, MUC2, MUC1, KRT19, CDX2
5MP:00053879.0PTPN3, TIMP3, MCL1, MUC2, MUC5AC, LGALS3
6MP:00053768.9AFP, TIMP3, MUC2, MUC1, MUC5AC, LGALS3
7MP:00053848.9TIMP3, MCL1, MUC2, LGALS3, OGG1, KRT7
8MP:00107688.6AFP, TIMP3, MCL1, MUC2, LGALS3, OGG1

Publications for Cholangiocarcinoma

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Articles related to Cholangiocarcinoma:

(show top 50)    (show all 1450)
idTitleAuthorsYear
1
Hydroxytyrosol inhibits cholangiocarcinoma tumor growth: An in vivo and in vitro study. (24247752)
2014
2
Diagnosis and management of intrahepatic cholangiocarcinoma: A comprehensive update for the radiologist. (25240565)
2014
3
Integrins I+vI^6, I+6I^4 and I+3I^1 are down-regulated in cholangiolocellular carcinoma but not cholangiocarcinoma. (24552196)
2014
4
Prediction of the Postoperative Prognosis of Intrahepatic Cholangiocarcinoma (ICC): Importance of Preoperatively-Determined Anatomic Invasion Level and Number of Tumors. (24122559)
2013
5
Adjuvant chemotherapy in resectable cholangiocarcinoma patients. (23829232)
2013
6
Clinical significances of cancer stem cells markers in patients with intrahepatic cholangiocarcinoma who underwent hepatectomy. (23645762)
2013
7
Chemotherapy for cholangiocarcinoma: An update. (23919111)
2013
8
Hepatobiliary resection with concomitant resection of the inferior vena cava for advanced intrahepatic cholangiocarcinoma: report of a case. (22961194)
2013
9
Clinical usefulness of (18) F-fluorodeoxyglucose positron emission tomography/computed tomography for patients with primary liver cancer with special reference to rare histological types, hepatocellular carcinoma with sarcomatous change and combined hepatocellular and cholangiocarcinoma. (23145869)
2013
10
Comparison of computed tomography- and positron emission tomography-based radiotherapy planning in cholangiocarcinoma. (24051924)
2013
11
Factors affecting survival time of cholangiocarcinoma patients: a prospective study in Northeast Thailand. (23679246)
2013
12
Intrahepatic cholangiocarcinoma: impact of genetic hemochromatosis on outcome and overall survival after surgical resection. (23183056)
2013
13
Anticancer effect of arsenic trioxide on cholangiocarcinoma: in vitro experiments and in vivo xenograft mouse model. (23467906)
2013
14
Infiltration of peritumoural but tumour-free parenchyma with IgG4-positive plasma cells in hilar cholangiocarcinoma and pancreatic adenocarcinoma. (23602806)
2013
15
A zebrafish model of intrahepatic cholangiocarcinoma by dual expression of hepatitis B virus X and hepatitis C virus core protein in liver. (22729936)
2012
16
Clinicopathological significance of E-cadherin, I^-catenin and epidermal growth factor receptor expression in intrahepatic cholangiocarcinoma. (22281980)
2012
17
Treatment of recurrent intrahepatic cholangiocarcinoma. (23132419)
2012
18
Periductal infiltrating type of intrahepatic cholangiocarcinoma: a rare macroscopic type without any apparent mass. (22350300)
2012
19
Cholangiocarcinoma complicating secondary sclerosing cholangitis from cryptosporidiosis in an adult patient with CD40 ligand deficiency: case report and review of the literature. (22678466)
2012
20
Claudin-18 in biliary neoplasms. Its significance in the classification of intrahepatic cholangiocarcinoma. (21607649)
2011
21
Development of a questionnaire (EORTC module) to measure quality of life in patients with cholangiocarcinoma and gallbladder cancer, the EORTC QLQ-BIL21. (21266979)
2011
22
MicroRNA down-regulated in human cholangiocarcinoma control cell cycle through multiple targets involved in the G1/S checkpoint. (21809359)
2011
23
The value of F-18 FDG PET in patients with primary sclerosing cholangitis and cholangiocarcinoma using visual and semiquantitative analysis. (21892037)
2011
24
Periostin, a matrix protein, has potential as a novel serodiagnostic marker for cholangiocarcinoma. (21347516)
2011
25
A case of intrahepatic cholangiocarcinoma with marked mucus production. (22128317)
2011
26
Mixed hepatocellular cholangiocarcinoma and intrahepatic cholangiocarcinoma in patients undergoing transplantation for hepatocellular carcinoma. (21438129)
2011
27
Ubiquitin-specific protease 14 expression associated with intrahepatic cholangiocarcinoma cell differentiation. (21627382)
2011
28
Different roles of S100P overexpression in intrahepatic cholangiocarcinoma: carcinogenesis of perihilar type and aggressive behavior of peripheral type. (21412073)
2011
29
Radiofrequency ablation of intrahepatic cholangiocarcinoma: preliminary experience. (20411389)
2010
30
Clinical and histopathological study of "follicular cholangitis": Sclerosing cholangitis with prominent lymphocytic infiltration masquerading as hilar cholangiocarcinoma. (20880060)
2010
31
The E-cadherin repressor slug and progression of human extrahepatic hilar cholangiocarcinoma. (20594328)
2010
32
The prognosis and survival outcome of intrahepatic cholangiocarcinoma following surgical resection: association of lymph node metastasis and lymph node dissection with survival. (19626372)
2009
33
Relationship between microvessel count and postoperative survival in patients with intrahepatic cholangiocarcinoma. (19434454)
2009
34
Alterations of gene expression of RB pathway in Opisthorchis viverrini infection-induced cholangiocarcinoma. (19582476)
2009
35
Clinicopathological analysis of colorectal cancer liver metastasis and intrahepatic cholangiocarcinoma: are they just apples and oranges? (18329969)
2008
36
A novel "patient-like" model of cholangiocarcinoma progression based on bile duct inoculation of tumorigenic rat cholangiocyte cell lines. (18081149)
2008
37
Glycine N-methyltransferase is a favorable prognostic marker for human cholangiocarcinoma. (18624901)
2008
38
Serum cytokeratin 19 fragment (CYFRA21-1) as a prognostic factor in intrahepatic cholangiocarcinoma. (17955299)
2008
39
Aberrant expression of alpha-fetoprotein in intrahepatic cholangiocarcinoma: an exceptional occurrence. (18417680)
2008
40
Influence of hepatic resection margin on recurrence and survival in intrahepatic cholangiocarcinoma. (18685896)
2008
41
Indications for surgical treatment of intrahepatic cholangiocarcinoma with lymph node metastases. (18670844)
2008
42
14-3-3sigma negatively regulates the cell cycle, and its down-regulation is associated with poor outcome in intrahepatic cholangiocarcinoma. (17391729)
2007
43
Relation of cholangiocarcinomas to clonorchiasis and bile duct stones. (15185021)
2004
44
Major hepatic resection for hilar cholangiocarcinoma: analysis of 46 patients. (15136352)
2004
45
A case of combined hepatocellular-cholangiocarcinoma with sarcomatous transformation and second primary colon cancer]. (15218348)
2004
46
Comparative immunohistochemical profile of hepatocellular carcinoma, cholangiocarcinoma, and metastatic adenocarcinoma. (12514785)
2002
47
Aberrant CpG island methylation of multiple genes in intrahepatic cholangiocarcinoma. (12213730)
2002
48
Involvement of 85-kd cytosolic phospholipase A(2) and cyclooxygenase-2 in the proliferation of human cholangiocarcinoma cells. (12143044)
2002
49
Biliary carcinoembryonic antigen levels are a marker for cholangiocarcinoma. (8554130)
1996
50
Chronic postcolectomy pericholangitis and cholangiocarcinoma. (2995479)
1985

Variations for Cholangiocarcinoma

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Expression for genes affiliated with Cholangiocarcinoma

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Expression patterns in normal tissues for genes affiliated with Cholangiocarcinoma

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Pathways for genes affiliated with Cholangiocarcinoma

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Pathways related to Cholangiocarcinoma according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
IL-9 Signaling Pathway37
Development Thrombopoietin regulated cell processes60
IL-7 Signaling Pathway37
Immune response IL 9 signaling pathway60
10.0MCL1, MUC1, CDKN1B
2
Show member pathways
9.5MUC2, MUC5AC, MUC4, MUC1, MUC17
3
Show member pathways
9.3MUC17, MUC1, MUC4, MUC5AC, CDX2, MUC2

Compounds for genes affiliated with Cholangiocarcinoma

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Compounds related to Cholangiocarcinoma according to GeneCards/GeneDecks:

(show top 50)    (show all 97)
idCompoundScoreTop Affiliating Genes
1[alcl]4410.2CDKN1B, MUC1, MCL1
2gemcitabine44 50 1111.9MCL1, CDKN1B, CEACAM6, MUC1
312-o-tetradecanoylphorbol 13-acetate449.9CDKN1B, MUC1, MCL1, TIMP3, MUC2
4mucicarmine449.9CEACAM3, MUC1, KRT20, KRT7
5galactose449.9LGALS3, AFP, MUC2, MUC1, CEACAM3
6dmso449.8CDKN1B, MUC5AC, OGG1, MUC1, MUC2, AFP
7actinomycin d449.8MCL1, MUC2, AFP, CDKN1B, MUC1
8wortmannin449.8TIMP3, CDKN1B, PTPN3, MCL1, LGALS3
9pd 98,059449.8MCL1, MUC5AC, MUC2, TIMP3, PTPN3, CDKN1B
10adriamycin449.8OGG1, KRT19, AFP, MUC1, CEACAM3
11paclitaxel44 50 1111.8KRT19, CDKN1B, PTPN3, MCL1, AFP
12polysaccharide449.8KRT7, LGALS3, AFP, MUC5AC, MUC1
13agarose449.8OGG1, MUC1, MUC2, TIMP3
14streptavidin449.7KRT7, CEACAM3, KRT20
15ribonucleic acid449.7TIMP3, MUC1, KRT19, KRT20, MUC5AC
16carbohydrates449.7KRT7, LGALS3, MUC5AC, MUC1, MUC2, KRT19
17cisplatin44 50 61 1112.7KRT19, CDKN1B, AFP, LGALS3, MUC1, MCL1
18matrigel449.6MUC1, KRT19, MUC4, LGALS3, KRT7, TIMP3
19glutamate449.6TIMP3, MCL1, CDKN1B, PTPN3, AFP, LGALS3
20formaldehyde44 2410.6LGALS3, KRT20, CDKN1B, KRT19, MUC1, KRT7
215fluorouracil449.6MUC5AC, MCL1, CDKN1B, CEACAM3, MUC1, MUC2
22b 723449.5KRT19, KRT7, CEACAM3, AFP, MUC1, KRT20
23estradiol44 24 1111.5AFP, TIMP3, CDKN1B, MUC1, KRT19
24glycogen44 2410.5AFP, CDKN1B, MUC1, KRT7, LGALS3, KRT19
25threonine449.5MCL1, PTPN3, MUC2, MUC1, MUC5AC, LGALS3
26methotrexate50 44 1111.5AFP, KRT7, MUC2, MUC1, MUC5AC, CEACAM3
27cysteine449.5TIMP3, MUC2, PTPN3, OGG1, CDX2, MUC1
28nitric oxide44 24 1111.4LGALS3, CEACAM3, MUC5AC, MUC1, OGG1, CDKN1B
29thymidine44 2410.4OGG1, CDKN1B, CEACAM3, AFP, KRT19, CDX2
30forskolin44 50 1111.4CDKN1B, MCL1, PTPN3, MUC2, MUC1
31sialic acid449.4CEACAM3, MUC5AC, MUC2, PTPN3, AFP, MUC1
32butyrate449.4CDX2, KRT19, LGALS3, MUC1, MUC2, MCL1
33dexamethasone44 50 28 1112.4AFP, MUC4, PTPN3, MUC1, MUC5AC, MUC2
34cholesterol44 28 24 1112.4CDKN1B, CEACAM3, MUC5AC, MUC4, MUC1, MUC2
35steroid449.3CDX2, KRT19, CEACAM3, MUC5AC, MUC4, TIMP3
36phosphatidylinositol449.3MUC4, MUC5AC, CEACAM6, LGALS3, CDKN1B, MCL1
37lipid449.3KRT7, AFP, PTPN3, TIMP3, MCL1, MUC1
38testosterone44 61 24 1112.2CDKN1B, AFP, TIMP3, MUC1, MUC5AC, KRT7
39hematoxylin449.2MUC1, MUC5AC, LGALS3, KRT7, KRT20, KRT19
40oligonucleotide449.0LGALS3, MAGEA3, OGG1, CDX2, MUC2, AFP
41glucose448.9TIMP3, KRT7, CDKN1B, KRT19, PTPN3, CEACAM3
42retinoic acid44 249.8CDKN1B, KRT19, MCL1, LGALS3, MUC5AC, TIMP3
43vegf448.8MCL1, CDKN1B, KRT19, KRT20, KRT7, LGALS3
44oxygen44 249.8AFP, CDKN1B, KRT19, OGG1, TIMP3, MCL1
45serine448.8CDKN1B, PTPN3, AFP, KRT19, MUC2, MUC17
46calcium44 50 24 1111.4KRT19, CDX2, MCL1, AFP, PTPN3, TIMP3
47progesterone44 28 61 24 1112.3AFP, TIMP3, KRT19, MUC2, KRT20, KRT7
48estrogen448.2TIMP3, MUC4, PTPN3, MUC2, MUC1, AFP
49tyrosine448.2KRT19, MUC5AC, CEACAM3, LGALS3, KRT7, KRT20
50paraffin447.8CDX2, KRT19, KRT20, CDKN1B, KRT7, MUC1

GO Terms for genes affiliated with Cholangiocarcinoma

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Cellular components related to Cholangiocarcinoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1intermediate filamentGO:0058829.7KRT19, KRT20, KRT7
2Golgi lumenGO:0057969.2MUC5AC, MUC4, MUC1, MUC17, MUC2

Biological processes related to Cholangiocarcinoma according to GeneCards/GeneDecks:

(show all 7)
idNameGO IDScoreTop Affiliating Genes
1negative regulation of membrane protein ectodomain proteolysisGO:05104510.2PTPN3, TIMP3
2negative regulation of mitotic cell cycleGO:04593010.1CDKN1B, PTPN3
3maintenance of gastrointestinal epitheliumGO:03027710.0MUC2, MUC4
4cellular homeostasisGO:0197259.7MCL1, MUC17
5O-glycan processingGO:0162669.6MUC5AC, MUC4, MUC1, MUC17, MUC2
6post-translational protein modificationGO:0436879.6MUC2, MUC17, MUC1, MUC4, MUC5AC
7cellular protein metabolic processGO:0442679.5MUC5AC, MUC4, MUC1, MUC17, MUC2

Molecular functions related to Cholangiocarcinoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular matrix constituent, lubricant activityGO:03019710.0MUC4, MUC17
2protein bindingGO:0055157.3CDKN1B, PTPN3, TIMP3, MCL1, MUC2, MUC17

Products for genes affiliated with Cholangiocarcinoma

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Sources for Cholangiocarcinoma

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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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet