MCID: MMM001
MIFTS: 49

Mammary Paget's Disease malady

Rare diseases, Reproductive diseases, Cancer diseases categories

Aliases & Classifications for Mammary Paget's Disease

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Sources:
61UMLS, 8Disease Ontology, 42NIH Rare Diseases, 48Orphanet, 10DISEASES, 44Novoseek, 39NCIt, 62UMLS via Orphanet, 34MESH via Orphanet, 26ICD10 via Orphanet, 25ICD10
See all sources

Aliases & Descriptions for Mammary Paget's Disease:

Name: Mammary Paget's Disease 8 42
Paget's Disease of the Nipple 42 48 61
Paget Disease of the Nipple 42 48
Paget's Disease, Mammary 42 61
Paget's Disease 8 61
Paget's Disease of the Breast 8
Pagets Disease of the Nipple 42
 
Paget Disease of the Breast 42
Pagets Disease Mammary 44
Mammary Pagets Disease 10
Mammary Paget Disease 8
Paget Cell Neoplasm 8
Osteitis Deformans 61


Classifications:



Characteristics (Orphanet epidemiological data):

48
paget's disease of the nipple:
Age of onset: Adult


External Ids:

Disease Ontology8 DOID:3443
NCIt39 C7073
Orphanet48 180275
UMLS via Orphanet62 C1704323, C0030185
MESH via Orphanet34 D010144
ICD10 via Orphanet26 C50.0
ICD1025 M88.9

Summaries for Mammary Paget's Disease

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MalaCards based summary: Mammary Paget's Disease, also known as paget's disease of the nipple, is related to paget disease, extramammary and melanoma. An important gene associated with Mammary Paget's Disease is KRT14 (keratin 14), and among its related pathways are SHC1 events in ERBB2 signaling and Alzheimers Disease Pathway. The drugs calcitonin and salmon calcitonin and the compounds methyltrienolone and capecitabine have been mentioned in the context of this disorder. Affiliated tissues include breast and bone, and related mouse phenotypes are tumorigenesis and embryogenesis.

Related Diseases for Mammary Paget's Disease

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Diseases related to Mammary Paget's Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 80)
idRelated DiseaseScoreTop Affiliating Genes
1paget disease, extramammary30.4AR, KRT7, ERBB2
2melanoma29.9KRT18, KRT14, KRT8, CFTR
3secretory meningioma10.5KRT7
4cystadenoma10.5KRT7
5breast fibroadenoma10.4PGR
6adenosarcoma10.4PGR
7bowen syndrome10.3KRT7, KRT14
8thymic carcinoma10.3KRT18, KRT7
9pancreatic ductal carcinoma10.3KRT8, KRT7
10epithelioid sarcoma10.3KRT8, KRT7
11liver cirrhosis10.3KRT8, KRT18
12cirrhosis, cryptogenic10.3KRT8, KRT18
13craniopharyngioma10.3KRT8, KRT14
14sertoli-leydig cell tumor10.3PGR, KRT7
15biliary tract neoplasm10.2ERBB2, EGFR
16cervical squamous cell carcinoma10.2KRT8, KRT14
17intestinal obstruction10.2CFTR
18breast disease10.2PGR, ERBB2
19sex cord-gonadal stromal tumor10.2KRT7, PGR
20abrikosov's tumor10.2KRT8, KRT7
21alcoholic hepatitis10.2KRT18, KRT8
22bronchiolo-alveolar adenocarcinoma10.2EGFR, KRT7
23gallbladder cancer10.2KRT7, KRT8
24li-fraumeni syndrome10.2ERBB2, PGR
25superficial spreading melanoma10.2
26sarcoma, synovial10.2KRT7, KRT14, KRT8
27factor vii deficiency10.2ERBB2, EGFR
28angiosarcoma10.1KRT18, KRT14, KRT8
29mucoepidermoid carcinoma10.1KRT14, KRT7, EGFR
30breast cancer10.1PGR, ERBB2, KRT14
31sertoli cell tumor10.1PGR, AR
32gastric adenocarcinoma10.1ERBB2, KRT8, KRT7
33neuroendocrine tumor10.1KRT18, KRT7, KRT8
34bladder cancer, somatic10.1EGFR, ERBB2
35autoimmune hepatitis10.1KRT18, KRT8
36malignant mixed mullerian tumor10.1PGR, ERBB2, KRT7
37tubular adenocarcinoma10.1PGR, ERBB2, KRT7
38large cell carcinoma10.1EGFR, KRT7, ERBB2
39endometrial cancer10.1KRT7, ERBB2, PGR
40fibromatosis10.1AR, PGR
41gynecomastia10.1PGR, AR
42cervical adenocarcinoma10.1PGR, KRT8, KRT7
43inflammatory breast carcinoma10.1EGFR, ERBB2, PGR
44pancreatic cancer10.0EGFR, KRT7, KRT8
45lung cancer10.0EGFR, KRT7, ERBB2
46salivary gland adenoid cystic carcinoma10.0ERBB2, KRT8, KRT14, KRT7
47chordoma10.0KRT8, KRT14, KRT7, KRT18
48ameloblastoma10.0KRT8, KRT14, KRT7, KRT18
49oxyphilic adenoma10.0KRT8, KRT14, KRT7, KRT18
50leiomyosarcoma10.0PGR, KRT8

Graphical network of the top 20 diseases related to Mammary Paget's Disease:



Diseases related to mammary paget's disease

Symptoms for Mammary Paget's Disease

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Drugs & Therapeutics for Mammary Paget's Disease

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FDA approved drugs:

id Drug Name Active Ingredient(s)13 Pharmaceutical Company Approval Date
1
Aredia13 38 PAMIDRONATE DISODIUM Chiron Approved August 1996
FDA Label: Aredia
Malady that Drug Treats: osteolytic bone metastases of breast cancer
Indications and Usage:13 Hypercalcemia of Malignancy; Aredia, in conjunction with adequate hydration, is indicated for the treatment of moderate or severe; hypercalcemia associated with malignancy, with or without bone metastases. Patients who have either; epidermoid or non-epidermoid tumors respond to treatment with Aredia. Vigorous saline hydration, an; integral part of hypercalcemia therapy, should be initiated promptly and an attempt should be made to; restore the urine output to about 2 L/day throughout treatment. Mild or asymptomatic hypercalcemia may; be treated with conservative measures (i.e., saline hydration, with or without loop diuretics). Patients; should be hydrated adequately throughout the treatment, but overhydration, especially in those patients; who have cardiac failure, must be avoided. Diuretic therapy should not be employed prior to correction of; hypovolemia. The safety and efficacy of Aredia in the treatment of hypercalcemia associated with; hyperparathyroidism or with other non-tumor-related conditions has not been established.; Paget s Disease; Aredia is indicated for the treatment of patients with moderate to severe Paget s disease of bone. The; effectiveness of Aredia was demonstrated primarily in patients with serum alkaline phosphatase e"3 times; the upper limit of normal. Aredia therapy in patients with Paget s disease has been effective in reducing; serum alkaline phosphatase and urinary hydroxyproline levels by e"50% in at least 50% of patients, and by; e"30% in at least 80% of patients. Aredia therapy has also been effective in reducing these biochemical; markers in patients with Paget s disease who failed to respond, or no longer responded to other; treatments.; Osteolytic Bone Metastases of Breast Cancer and Osteolytic Lesions of Multiple; Myeloma; Aredia is indicated, in conjunction with standard antineoplastic therapy, for the treatment of osteolytic; bone metastases of breast cancer and osteolytic lesions of multiple myeloma. The Aredia treatment effect; appeared to be smaller in the study of breast cancer patients receiving hormonal therapy than in the study; of those receiving chemotherapy, however, overall evidence of clinical benefit has been demonstrated; (see CLINICAL PHARMACOLOGY, Osteolytic Bone Metastases of Breast Cancer and Osteolytic; Lesions of Multiple Myeloma, Clinical Trials section).
DrugBank Targets:11 1. Farnesyl pyrophosphate synthase; 2. Hydroxylapatite
Mechanism of Action:13 
Target: bone resorption; FPP synthase
Action: inhibitor
FDA: The principal pharmacologic action of Aredia is inhibition of bone resorption. Although the mechanism of; antiresorptive action is not completely understood, several factors are thought to contribute to this action.; Aredia adsorbs to calcium phosphate (hydroxyapatite) crystals in bone and may directly block dissolution; of this mineral component of bone. In vitro studies also suggest that inhibition of osteoclast activity; contributes to inhibition of bone resorption. In animal studies, at doses recommended for the treatment of; hypercalcemia, Aredia inhibits bone resorption apparently without inhibiting bone formation and; mineralization. Of relevance to the treatment of hypercalcemia of malignancy is the finding that Aredia; inhibits the accelerated bone resorption that results from osteoclast hyperactivity induced by various; tumors in animal studies.

Drug clinical trials:

Search ClinicalTrials for Mammary Paget's Disease

Search NIH Clinical Center for Mammary Paget's Disease

Inferred drug relations via UMLS61/NDF-RT40:

Genetic Tests for Mammary Paget's Disease

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Anatomical Context for Mammary Paget's Disease

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MalaCards organs/tissues related to Mammary Paget's Disease:

31
Breast, Bone

Animal Models for Mammary Paget's Disease or affiliated genes

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MGI Mouse Phenotypes related to Mammary Paget's Disease:

35 (show all 13)
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020068.7KRT14, AR, EGFR, ERBB2, PGR
2MP:00053808.4KRT18, ERBB2, KRT8, AR, PGR, EGFR
3MP:00053818.4ERBB2, CFTR, KRT8, KRT14, EGFR, AR
4MP:00053708.3CFTR, AR, EGFR, KRT18, KRT8
5MP:00053878.1AR, PGR, KRT8, KRT14, EGFR, CFTR
6MP:00053898.0EGFR, KRT14, KRT8, ERBB2, PGR, CFTR
7MP:00053787.8KRT14, CFTR, EGFR, AR, ERBB2, KRT18
8MP:00053977.8CFTR, EGFR, AR, KRT14, KRT8, PGR
9MP:00053847.7PGR, CFTR, ERBB2, KRT7, EGFR, AR
10MP:00107687.7CFTR, ERBB2, KRT8, KRT18, EGFR, KRT14
11MP:00028737.7AR, CFTR, PGR, LALBA, ERBB2, EGFR
12MP:00107717.4AR, EGFR, KRT14, ERBB2, CSN3, PGR
13MP:00053797.2LALBA, AR, EGFR, ERBB2, CSN3, PGR

Publications for Mammary Paget's Disease

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Articles related to Mammary Paget's Disease:

(show top 50)    (show all 54)
idTitleAuthorsYear
1
Extra Mammary Paget's Disease: A Rare Case Report. (25657432)
2015
2
Urothelial bladder carcinoma metastasising to the scrotum mimicking primary extra-mammary Paget's disease. (24614713)
2014
3
Minimally invasive mammary Paget's disease without an underlying breast carcinoma. (23851623)
2013
4
Pigmented mammary Paget's disease presenting with dermoscopic features of multiple dots. (23865386)
2013
5
A rare presentation of mammary Paget's disease involving the entire breast in the absence of any underlying ductal malignancy. (23760322)
2013
6
An Unusual Case of Mammary Paget's Disease Diagnosed Using Dynamic Contrast-Enhanced MRI. (23607031)
2013
7
Superficial sebaceous carcinoma on the areola clinically mimicking mammary Paget's disease. (23078184)
2013
8
Extra mammary Paget's disease of the vulva. (23372329)
2012
9
Pigmented mammary Paget's disease mimicking melanoma: report of three cases. (22064040)
2012
10
Characteristics of mammary Paget's disease in China: a national-wide multicenter retrospective study during 1999-2008. (22901142)
2012
11
A hyperpigmented plaque on the right nipple: a quiz. Pigmented mammary Paget's disease. (21369688)
2011
12
Cystic fibrosis transmembrane conductance regulator is helpful in the distinction of extra-mammary Paget's disease from squamous cell carcinoma in situ (Bowen's disease). (21352263)
2011
13
Extra-mammary Paget's disease of the perianal region: a review of the literature emphasizing the operative management technique. (20884199)
2011
14
A case report of pigmented mammary Paget's disease mimicking nevus of the nipple. (21213079)
2011
15
Effectiveness of photodynamic therapy for mammary and extra-mammary Paget's disease: a state of the science review. (21676258)
2011
16
Mammary Paget's disease and extra-mammary Paget's disease: two morphologically similar but biologically different diseases. (19719834)
2010
17
Different panels of markers should be used to predict mammary Paget's disease associated with in situ or invasive ductal carcinoma of the breast. (19201736)
2009
18
Pigmented mammary Paget's disease presenting as an enlarged areola. (19601948)
2009
19
Extra-mammary Paget's disease of the scrotum and penis: a case report. (20184077)
2009
20
Pigmented mammary Paget's disease: not a melanoma. (19413640)
2009
21
Pigmented mammary Paget's disease mimicking melanoma a further case in a man. (19470138)
2009
22
Clinicopathologic evaluation of mammary Paget's disease. (19967013)
2008
23
Immunohistochemical tracking of an immune response in mammary Paget's disease. (18842336)
2008
24
Allergic contact dermatitis mimicking mammary paget's disease. (19882019)
2008
25
Acinar pattern of mammary Paget's disease: a case report. (17760678)
2007
26
Quadruple extra-mammary Paget's disease. (17225023)
2007
27
Extensive mammary Paget's disease. (17397359)
2007
28
Pigmented mammary Paget's disease. (17567331)
2007
29
Mammary Paget's disease occurring after mastectomy. (16899115)
2006
30
Cytokeratin 7 staining was useful in a case of pigmented mammary Paget's disease resembling malignant melanoma. (17040462)
2006
31
Pigmented mammary Paget's disease mimicking melanoma. (15057051)
2004
32
Pigmented mammary Paget's disease in a man. (15032027)
2003
33
The ectopic expression of gastric mucin in extramammary and mammary Paget's disease. (11979091)
2002
34
Morphological evidence for field effect as a mechanism for tumour spread in mammary Paget's disease. (10583581)
1999
35
Mammary Paget's disease: evidence of diverse origin of the disease with a subgroup of Paget's disease developing from the superficial portion of lactiferous duct and a discontinuous pattern of tumor spread. (10594841)
1999
36
Mammary Paget's disease with acantholytic features and without any detectable breast tumor. (10089077)
1998
37
Underlying pathology in mammary Paget's disease. (9181226)
1997
38
Human papilloma virus DNA: a factor in the pathogenesis of mammary Paget's disease? (8932876)
1996
39
Mammary Paget's disease confined to the areola and associated with multifocal Toker cell hyperplasia. (8599455)
1995
40
Mammary Paget's disease and associated carcinoma. An immunohistochemical study. (7680194)
1993
41
Stump the experts. Extra-mammary Paget's disease in the perianal region. (2157742)
1990
42
Study of neu-protein expression in mammary Paget's disease with and without underlying breast carcinoma and in extramammary Paget's disease. (1701961)
1990
43
Extra-mammary Paget's disease of the penis. (2537673)
1989
44
Mammary Paget's disease and intraductal carcinoma. Histologic, histochemical, and immunocytochemical comparison. (2466419)
1988
45
Mammary Paget's disease without underlying carcinoma. (3026196)
1985
46
Immunohistochemical study of mammary and extra-mammary Paget's disease. (6098546)
1984
47
Unusual pulmonary metastasis of mammary cancer: unilateral pulmonary fibrosis after mammary Paget's disease. (4376192)
1974
48
MAMMARY PAGET'S DISEASE. (14179983)
1964
49
Extra-mammary Paget's disease; discussion of pathogenesis. (14892969)
1952
50
Findings in a case of extra-mammary Paget's disease. (14783970)
1950

Variations for Mammary Paget's Disease

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Expression for genes affiliated with Mammary Paget's Disease

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Search GEO for disease gene expression data for Mammary Paget's Disease.

Pathways for genes affiliated with Mammary Paget's Disease

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Pathways related to Mammary Paget's Disease according to GeneCards Suite gene sharing:

(show all 13)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.7EGFR, ERBB2
29.7EGFR, ERBB2
39.7ERBB2, EGFR
49.3KRT18, KRT7, KRT8
5
Show member pathways
9.3PGR, ERBB2, EGFR
6
Show member pathways
Signaling Pathways in Glioblastoma36
9.2AR, EGFR, ERBB2
7
Show member pathways
Translation Non genomic rapid action of Androgen Receptor59
9.2AR, EGFR, ERBB2
89.2AR, EGFR, ERBB2
99.2ERBB2, EGFR, AR
10
Show member pathways
9.2ERBB2, EGFR, AR
11
Show member pathways
9.2EGFR, ERBB2, CFTR
129.0KRT18, KRT7, KRT14, KRT8
138.8EGFR, KRT18, KRT7, KRT8

Compounds for genes affiliated with Mammary Paget's Disease

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Compounds related to Mammary Paget's Disease according to GeneCards Suite gene sharing:

(show top 50)    (show all 138)
idCompoundScoreTop Affiliating Genes
1methyltrienolone28 1110.9PGR, AR
2capecitabine44 50 1111.6PGR, ERBB2, EGFR
3anastrozole44 50 1111.6PGR, EGFR, ERBB2
4taxane449.6PGR, ERBB2, EGFR
5hematoxylin449.4KRT14, KRT7, KRT8, KRT18
6leucovorin44 50 1111.4ERBB2, PGR, EGFR
7exemestane44 50 60 1112.3ERBB2, EGFR, AR, PGR
8lapatinib44 50 1111.3AR, EGFR, ERBB2, PGR
9letrozole44 50 60 1112.3AR, EGFR, ERBB2, PGR
10progestin449.2PGR, AR, EGFR, ERBB2
11geldanamycin44 50 60 1112.2EGFR, AR, PGR, ERBB2
12formaldehyde44 2410.2KRT7, CFTR, ERBB2, KRT18
13trastuzumab44 50 1111.2EGFR, ERBB2, PGR
145fluorouracil449.2EGFR, PGR, KRT18, ERBB2
15gnrh449.2ERBB2, PGR, CFTR, AR
16vitamin a44 24 1111.1KRT14, KRT7, AR, PGR
17mg 13244 6010.1EGFR, AR, CFTR, KRT18
18butyrate449.1CFTR, KRT8, KRT14, KRT18
19curcumin449.1CFTR, ERBB2, AR, EGFR
20matrigel449.0AR, KRT18, KRT7, KRT8
21fulvestrant44 50 28 1112.0AR, EGFR, KRT18, PGR, ERBB2
22docetaxel44 50 60 1112.0PGR, KRT18, ERBB2, EGFR, AR
23cisplatin44 50 60 1111.9EGFR, CFTR, ERBB2, KRT8, KRT14
24n acetylcysteine448.9EGFR, KRT18, CFTR, AR, ERBB2
25n-acetylglucosamine448.8KRT7, KRT18, LALBA, KRT8
26cycloheximide448.8EGFR, PGR, AR, KRT8, ERBB2
27vitamin d448.7KRT14, CFTR, ERBB2, AR
28dexamethasone44 50 28 1111.7CFTR, ERBB2, KRT18, AR, PGR
29tamoxifen44 50 28 1111.7EGFR, AR, ERBB2, PGR, KRT14, KRT8
30agar448.6PGR, AR, EGFR, KRT18, ERBB2, KRT8
31sodium dodecylsulfate448.6LALBA, AR, PGR, CSN3
32urea44 24 1110.5LALBA, KRT14, KRT18, KRT8, CSN3
33genistein44 28 60 1 24 1113.5CFTR, PGR, ERBB2, AR, KRT8, EGFR
34lactose44 119.4CSN3, LALBA, CFTR, KRT7
35ethanol44 50 24 1111.4KRT18, KRT8, LALBA, CSN3
36steroid448.3AR, KRT18, KRT8, ERBB2, LALBA, PGR
37alanine448.3CFTR, LALBA, KRT18, PGR, ERBB2, KRT14
38aspartate448.2KRT18, AR, PGR, LALBA, CFTR, ERBB2
39doxorubicin44 50 1110.2KRT18, AR, EGFR, KRT8, CFTR, PGR
40serine448.2KRT18, KRT8, KRT14, EGFR, CFTR, AR
41retinoid448.1KRT18, AR, PGR, KRT8, KRT7, KRT14
42vegf448.1KRT18, KRT7, ERBB2, PGR, KRT8, AR
43iron44 249.0LALBA, CSN3, KRT7, KRT14, KRT8, KRT18
44glucose447.8CSN3, KRT8, KRT14, KRT7, KRT18, LALBA
45retinoic acid44 248.7KRT18, AR, EGFR, KRT8, PGR, CFTR
46testosterone44 60 24 1110.7PGR, CFTR, KRT8, AR, EGFR, KRT18
47tyrosine447.7EGFR, KRT18, KRT7, KRT14, ERBB2, LALBA
48paraffin447.4KRT8, PGR, CFTR, ERBB2, KRT14, KRT7
49progesterone44 28 60 24 1111.0EGFR, KRT18, KRT7, AR, KRT8, ERBB2
50estrogen446.6KRT14, ERBB2, KRT18, EGFR, AR, PGR

GO Terms for genes affiliated with Mammary Paget's Disease

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Cellular components related to Mammary Paget's Disease according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1apical plasma membraneGO:00163249.2EGFR, ERBB2, CFTR
2basolateral plasma membraneGO:00163239.1CFTR, ERBB2, EGFR
3keratin filamentGO:00450959.1KRT18, KRT7, KRT14, KRT8
4intermediate filamentGO:00058829.0KRT18, KRT7, KRT14, KRT8
5cytoplasmGO:00057377.5ERBB2, KRT8, KRT14, KRT7, KRT18, EGFR

Biological processes related to Mammary Paget's Disease according to GeneCards Suite gene sharing:

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1hepatocyte apoptotic processGO:009728410.0KRT18, KRT8
2positive regulation of transcription from RNA polymerase III promoterGO:004594510.0AR, ERBB2
3tumor necrosis factor-mediated signaling pathwayGO:00332099.9KRT18, KRT8
4extrinsic apoptotic signaling pathwayGO:00971919.7KRT8, KRT18
5positive regulation of phosphorylationGO:00423279.7AR, EGFR
6positive regulation of vasodilationGO:00459099.6CFTR, EGFR
7positive regulation of MAP kinase activityGO:00434069.4EGFR, ERBB2
8cell proliferationGO:00082839.2AR, EGFR, ERBB2
9cell-cell signalingGO:00072678.9AR, PGR, LALBA
10signal transductionGO:00071658.2LALBA, PGR, ERBB2, EGFR, AR

Molecular functions related to Mammary Paget's Disease according to GeneCards Suite gene sharing:

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1epidermal growth factor-activated receptor activityGO:00050069.8EGFR, ERBB2
2receptor signaling protein tyrosine kinase activityGO:00047169.7EGFR, ERBB2
3scaffold protein bindingGO:00971109.7KRT18, KRT8
4ligand-activated sequence-specific DNA binding RNA polymerase II transcription factor activityGO:00048799.7AR, PGR
5transmembrane receptor protein tyrosine kinase activityGO:00047149.4EGFR, ERBB2
6structural molecule activityGO:00051989.3KRT8, KRT7, KRT18
7enzyme bindingGO:00198998.9CFTR, PGR, EGFR, AR
8protein bindingGO:00055157.1CFTR, PGR, ERBB2, KRT8, KRT14, KRT7

Sources for Mammary Paget's Disease

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2CDC
12ExPASy
13FDA
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
57SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet