MCID: PRN049
MIFTS: 49

Paraneoplastic Pemphigus malady

Skin diseases, Rare diseases categories

Summaries for Paraneoplastic Pemphigus

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66Wikipedia, 34MalaCards
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Wikipedia:66 Paraneoplastic Pemphigus is an autoimmune disorder stemming from an underlying tumor. It is hypothesized... more...

MalaCards: Paraneoplastic Pemphigus is related to pemphigus and follicular dendritic cell sarcoma, and has symptoms including enanthema/aphtosa/aphta/leukoplakiaand vesicles/bullous/exsudative lesions/bullous/cutaneous/mucosal detachment. An important gene associated with Paraneoplastic Pemphigus is DSP (desmoplakin), and among its related pathways are Alpha6-Beta4 Integrin Signaling Pathway and Cell junction organization. The compounds dr-10 and asulam have been mentioned in the context of this disorder. Affiliated tissues include skin, b cells and t cells, and related mouse phenotype integument.

Aliases & Classifications for Paraneoplastic Pemphigus

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50Orphanet, 63UMLS, 27ICD10 via Orphanet, 64UMLS via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Skin diseases
Orphanet: 50 
Rare skin diseases


Characteristics (Orphanet epidemiological data):

50
paraneoplastic pemphigus:
Inheritance: Sporadic; Prevalence: <1/1000000; Age of onset: Variable; Age of death: Any age


Aliases & Descriptions:

paraneoplastic pemphigus 50 63


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ICD10 via Orphanet27 L10.8
UMLS via Orphanet64 C1112570

Related Diseases for Paraneoplastic Pemphigus

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18GeneCards, 19GeneDecks
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Diseases related to Paraneoplastic Pemphigus via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 136)
idRelated DiseaseScoreTop Affiliating Genes
1pemphigus31.8PPL, BLZF1, JUP, DSP, DST, DSG1
2follicular dendritic cell sarcoma31.0PPL
3sarcoma31.0JUP, PPL, DSP, DSG1, EVPL
4lichen planus30.9COL17A1, DST, DSG3
5thymoma30.9PPL, DSP, EVPL
6pemphigus vulgaris30.8DST, PPL, HLA-DRB1, JUP, DSP, DSG1
7hodgkin's lymphoma30.8BLZF1, HLA-B, HLA-A
8b-cell lymphomas30.7HLA-B, C6orf25, BLZF1
9pemphigus vegetans30.7DSG1
10pemphigus foliaceus30.7DSG1, PPL, DST, DSG3, DSG2, HLA-DRB1
11leukemia30.5HLA-A, HLA-C, HLA-B, HLA-DRB1, BLZF1, EVPL
12dermatitis30.4DSG3, DSG1, CD79A
13bullous pemphigoid30.4COL17A1, PPL, DSP, DST, EVPL
14skin disease30.3HLA-DRB1
15systemic lupus erythematosus30.2HLA-A, HLA-DQB1, CD79A, C6orf25, BLZF1, HLA-DRB1
16inflammatory bowel disease30.1HLA-B, HLA-DQB1, DSG2, JUP
17melanoma29.8DSG1, HLA-DRB1, HLA-C, JUP, HLA-B, BLZF1
18castleman's disease10.7
19bronchiolitis10.7
20bronchiolitis obliterans10.7
21dendritic cell sarcoma10.6
22chronic lymphocytic leukemia10.5
23respiratory failure10.4
24follicular lymphoma10.4
25myasthenia gravis10.3
26stomatitis10.3
27lichen planus pemphigoides10.3
28conjunctivitis10.2
29hepatocellular carcinoma10.2
30lupus erythematosus10.2
31mediastinitis10.2
32non-hodgkin lymphoma10.2
33inflammatory myofibroblastic tumor10.2
34toxic epidermal necrolysis10.2
35nonepidermolytic palmoplantar keratoderma10.1EVPL
36cicatricial pemphigoid10.1COL17A1
37bullous impetigo10.1DSG1
38pemphigus erythematosus10.1DSP, DSG3
39junctional epidermolysis bullosa10.1COL17A1
40hailey-hailey disease10.1DSP, JUP
41naxos disease10.1DSP, JUP
42keratosis10.1DSP, JUP
43keratoderma10.1DSP, DSG1
44cardiomyopathy dilated with woolly hair and keratoderma10.1DSP, JUP
45graft versus host disease10.1
46burns10.1
47lymphoid leukemia10.1
48hepatitis c10.1
49interdigitating dendritic cell sarcoma10.1
50epithelioid leiomyosarcoma10.1

Graphical network of the top 20 diseases related to Paraneoplastic Pemphigus:



Diseases related to paraneoplastic pemphigus

Symptoms for Paraneoplastic Pemphigus

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50Orphanet
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Symptoms:

50
  • enanthema/aphtosa/aphta/leukoplakia
  • vesicles/bullous/exsudative lesions/bullous/cutaneous/mucosal detachment

Drugs & Therapeutics for Paraneoplastic Pemphigus

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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Approved drugs:

Search CenterWatch for Paraneoplastic Pemphigus

Drug clinical trials:

Search ClinicalTrials for Paraneoplastic Pemphigus

Search NIH Clinical Center for Paraneoplastic Pemphigus

Search CenterWatch for Paraneoplastic Pemphigus

Genetic Tests for Paraneoplastic Pemphigus

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Anatomical Context for Paraneoplastic Pemphigus

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34MalaCards
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MalaCards organs/tissues related to Paraneoplastic Pemphigus:

34
Skin, B cells, T cells, Lung, Testes, Bone, Bone marrow, Prostate, Tongue, Bronchial epithelium

Animal Models for Paraneoplastic Pemphigus or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Paraneoplastic Pemphigus:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00107718.7EVPL, COL17A1, DSG3, DST, DSP, JUP

Publications for Paraneoplastic Pemphigus

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53PubMed
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Articles related to Paraneoplastic Pemphigus:

(show top 50)    (show all 270)
idTitleAuthorsYear
1
Detection of autoantibodies to periplakin and envoplakin in paraneoplastic pemphigus but not idiopathic pulmonary fibrosis using full-length recombinant proteins. (24275244)
2013
2
IgA pemphigus with paraneoplastic pemphigus-like clinical features showing IgA antibodies to desmoglein 1/3 and desmocollin 3, and IgG and IgA antibodies to the basement membrane zone. (23517469)
2013
3
Laboratory diagnosis of paraneoplastic pemphigus. (23796242)
2013
4
Interdigitating dendritic cell sarcoma complicated with paraneoplastic pemphigus after surgery of resection. (23652075)
2013
5
Paraneoplastic pemphigus: initial manifestation of lung cancer. (23670336)
2013
6
A case report of paraneoplastic pemphigus associated with esophageal squamous cell carcinoma. (23613673)
2013
7
Bronchiolitis obliterans presenting as prolonged ventilator dependence in a patient with paraneoplastic pemphigus. (22813514)
2012
8
Pathogenic anti-desmoglein 3 mAbs cloned from a paraneoplastic pemphigus patient by phage display. (22277944)
2012
9
Paraneoplastic pemphigus and Castleman's disease in the setting of herpes simplex virus infection. (22010956)
2012
10
Paraneoplastic pemphigus (paraneoplastic autoimmune multiorgan syndrome): clinical presentations and pathogenesis. (21605807)
2011
11
Improved outcomes after complete resection of underlying tumors for patients with paraneoplastic pemphigus: a single-center experience of 22 cases. (20390428)
2011
12
Paraneoplastic pemphigus with Castleman's disease and bronchiolitis obliterans. (22181577)
2011
13
Paraneoplastic pemphigus associated with corneal perforation and cutaneous alternariosis: a case report and review of cases treated with rituximab. (21434987)
2011
14
Paraneoplastic pemphigus associated with malignant gastrointestinal stromal tumour. (20107737)
2010
15
The protease inhibitor alpha-2-macroglobulin-like-1 is the p170 antigen recognized by paraneoplastic pemphigus autoantibodies in human. (20805888)
2010
16
Fatal paraneoplastic pemphigus associated with a mediastinal tumor. (19087213)
2009
17
A case of paraneoplastic pemphigus associated with Castleman's disease presenting as erosive lichen planus. (19663833)
2009
18
Detection and characterization of anti-envoplakin linker autoantibodies in paraneoplastic pemphigus using specific bead-based assay. (18790674)
2008
19
Paraneoplastic pemphigus. (17683379)
2007
20
Paraneoplastic pemphigus without an underlying neoplasm. (17300250)
2007
21
Paraneoplastic pemphigus associated with Castleman's disease: usefulness of the laboratory of autoimmunity in the diagnosis of this disease. (17804551)
2007
22
Paraneoplastic Pemphigus and Bronchiolitis Obliterans in a Patient with Splenic B-cell Lymphoma. (17908666)
2007
23
Inflammatory myofibroblastic tumor on intercostal nerve presenting as paraneoplastic pemphigus with fatal pulmonary involvement. (17728520)
2007
24
Paraneoplastic pemphigus in a dog with splenic sarcoma. (15657279)
2005
25
Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma and Castleman disease. (16029358)
2005
26
Paraneoplastic pemphigus with widespread mucosal involvement. (16396806)
2005
27
Autoantibody production from a thymoma and a follicular dendritic cell sarcoma associated with paraneoplastic pemphigus. (16120143)
2005
28
Exacerbation of paraneoplastic pemphigus by cyclophosphamide treatment: detection of novel autoantigens and bronchial autoantibodies. (15149520)
2004
29
Paraneoplastic autoimmune multiorgan syndrome (paraneoplastic pemphigus) in a child: case report and review of the literature. (15466078)
2004
30
No involvement of IgG autoantibodies against extracellular domains of desmoglein 2 in paraneoplastic pemphigus or inflammatory bowel diseases. (12850306)
2003
31
Herpetiformis pemphigus and lung carcinoma: a case of paraneoplastic pemphigus. (12361141)
2002
32
Paraneoplastic pemphigus triggered by Castleman's disease. (11903261)
2002
33
Paraneoplastic pemphigus: a refractory autoimmune mucocutaneous disease. (12196890)
2002
34
Paraneoplastic pemphigus. (11589740)
2001
35
Paraneoplastic pemphigus. A report of two cases associated with chronic B-cell lymphocytic leukaemia. (11453921)
2001
36
Paraneoplastic pemphigus with circulating antibodies directed exclusively against the pemphigus vulgaris antigen desmoglein 3. (11004638)
2000
37
Paraneoplastic pemphigus in two patients with B-cell non-Hodgkin's lymphoma: significant responses to cyclophosphamide and prednisolone. (10629583)
2000
38
Periplakin and envoplakin are target antigens in canine and human paraneoplastic pemphigus. (10321593)
1999
39
Plectin serves as an autoantigen in paraneoplastic pemphigus. (10469345)
1999
40
Graft-versus-host disease-like immunophenotype and apoptotic keratinocyte death in paraneoplastic pemphigus. (10583130)
1999
41
Paraneoplastic pemphigus with fatal pulmonary involvement in a woman with a mesenteric Castleman tumour. (10233229)
1999
42
Antibodies against desmoglein 3 (pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice. (9710446)
1998
43
Novel non-radioisotope immunoprecipitation studies indicate involvement of pemphigus vulgaris antigen in paraneoplastic pemphigus. (9673895)
1998
44
Childhood paraneoplastic pemphigus associated with Castleman's tumour. (9039308)
1997
45
cDNA cloning of the 210-kDa paraneoplastic pemphigus antigen reveals that envoplakin is a component of the antigen complex. (9284106)
1997
46
Paraneoplastic pemphigus: report of a case. (8708152)
1996
47
Lethal paraneoplastic pemphigus following treatment of chronic lymphocytic leukaemia with fludarabine. (8664199)
1995
48
Paraneoplastic pemphigus presenting as a lichen planus pemphigoides-like eruption. (8323308)
1993
49
Indirect immunofluorescence on rat bladder transitional epithelium: a test with high specificity for paraneoplastic pemphigus. (7684408)
1993
50
Human autoantibodies against desmoplakins in paraneoplastic pemphigus. (1601988)
1992

Variations for Paraneoplastic Pemphigus

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Expression for genes affiliated with Paraneoplastic Pemphigus

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Paraneoplastic Pemphigus

Search GEO for disease gene expression data for Paraneoplastic Pemphigus.

Pathways for genes affiliated with Paraneoplastic Pemphigus

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Sources:
51PathCards, 39NCBI BioSystems Database, 56Reactome, 31KEGG, 61Thomson Reuters, 58SinoBiological, 55R&D Systems, 54QIAGEN
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Pathways related to Paraneoplastic Pemphigus according to GeneCards/GeneDecks:

(show all 21)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.8DSP, DST, COL17A1
2
Show member pathways
9.7COL17A1, DST, JUP
39.6DSG1, HLA-DRB1, HLA-DQB1
4
Show member pathways
Arrhythmogenic right ventricular cardiomyopathy39
9.6DSG2, DSC2, DSP, JUP
5
Show member pathways
Immune response T cell receptor signaling pathway61
Immune response CD28 signaling61
Immune response ICOS pathway in T helper cell61
9.5HLA-DQB1, HLA-DRB1, CD79A
6
Show member pathways
9.5HLA-DQB1, HLA-C, HLA-A
7
Show member pathways
9.4HLA-B, HLA-A, HLA-C
8
Show member pathways
9.4HLA-B, HLA-A, HLA-C
99.4HLA-B, HLA-A, HLA-C
109.3EVPL, DST, DSP, PPL
11
Show member pathways
9.2DSP, DSG1, DSG3, DSG2
128.7HLA-DRB1, HLA-C, HLA-DQB1, HLA-A, HLA-B
138.7HLA-DRB1, HLA-C, HLA-DQB1, HLA-A, HLA-B
148.7HLA-B, HLA-A, HLA-DQB1, HLA-C, HLA-DRB1
15
Show member pathways
8.7HLA-B, HLA-A, HLA-DQB1, HLA-C, HLA-DRB1
16
Show member pathways
8.7HLA-B, HLA-A, HLA-DQB1, HLA-C, HLA-DRB1
17
Show member pathways
8.7HLA-B, HLA-A, HLA-DQB1, HLA-C, HLA-DRB1
18
Show member pathways
8.7HLA-B, HLA-A, HLA-DQB1, HLA-C, HLA-DRB1
198.7HLA-B, HLA-A, HLA-DQB1, HLA-C, HLA-DRB1
20
Show member pathways
8.4CD79A, HLA-DRB1, HLA-C, HLA-DQB1, HLA-A, HLA-B
21
Show member pathways
Proteasome Degradation39
Immune response Antigen presentation by MHC class I61
8.1DSG2, DSG3, DSG1, DSP, HLA-C, HLA-A

Compounds for genes affiliated with Paraneoplastic Pemphigus

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Sources:
46Novoseek, 52PharmGKB, 12DrugBank, 3BitterDB, 25HMDB, 30IUPHAR, 62Tocris Bioscience
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Compounds related to Paraneoplastic Pemphigus according to GeneCards/GeneDecks:

(show all 27)
idCompoundScoreTop Affiliating Genes
1dr-104610.1HLA-A, HLA-DRB1
2asulam4610.1PPL, DSP
3pneumococcal4610.1CD79A, HLA-B
4gliadin4610.0CD79A, HLA-DQB1
5toxin a469.9DSG3, DSG1
6ecori469.9HLA-B, HLA-DQB1
7gold469.8HLA-B, DST, COL17A1
8allopurinol46 52 1211.8HLA-A, HLA-B
9carbamazepine46 52 1211.7HLA-B, HLA-A, CD79A
10ivig469.7CD79A, DSG3, DSG1
11acetone469.7BLZF1, DSP
12allergens469.6CD79A, BLZF1, HLA-DRB1
135-aza-2deoxycytidine469.6JUP, HLA-A, HLA-B
14paraffin469.5HLA-B, JUP, DST, CD79A
15dapsone46 3 52 1212.5CD79A, HLA-B
16nacl469.5COL17A1, DST, BLZF1
17sucrose46 25 1211.4BLZF1, JUP, DSP, DSG1, COL17A1
18tacrolimus46 52 1211.4CD79A, HLA-A, HLA-B
19steroid469.1HLA-B, BLZF1, DSP, COL17A1, CD79A
20acetylcholine46 52 30 25 1213.1BLZF1, DSG1, DSG3, CD79A
21cyclosporin a46 30 6211.1CD79A, BLZF1, HLA-A, HLA-B
22retinoic acid46 259.9HLA-B, BLZF1, JUP, DSP, DSG1, CD79A
23oligonucleotide468.9HLA-B, HLA-A, HLA-DQB1, HLA-DRB1, JUP, DST
24arginine468.8HLA-B, HLA-A, HLA-DRB1, BLZF1, CD79A
25serine468.4HLA-B, BLZF1, JUP, DSP, DSG1, DSG3
26tyrosine466.5HLA-DRB1, HLA-DQB1, HLA-A, HLA-B, BLZF1, C6orf25
27calcium46 52 25 129.5COL17A1, HLA-B, HLA-A, BLZF1, C6orf25, JUP

GO Terms for genes affiliated with Paraneoplastic Pemphigus

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17Gene Ontology
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Cellular components related to Paraneoplastic Pemphigus according to GeneCards/GeneDecks:

(show all 17)
idNameGO IDScoreTop Affiliating Genes
1fascia adherensGO:00591610.0JUP, DSP
2hemidesmosomeGO:03005610.0COL17A1, DST, JUP
3lateral plasma membraneGO:0163289.9JUP, DSG1, DSG2
4intermediate filamentGO:0058829.8DST, DSP, JUP
5cornified envelopeGO:0015339.8EVPL, DSP
6cell-cell junctionGO:0059119.7COL17A1, DSG2, DSG1, JUP
7MHC class II protein complexGO:0426139.7HLA-DRB1, HLA-DQB1
8MHC class I protein complexGO:0426129.6HLA-B, HLA-A, HLA-C
9phagocytic vesicle membraneGO:0306709.5HLA-C, HLA-A, HLA-B
10early endosome membraneGO:0319019.5HLA-B, HLA-A, HLA-C
11integral component of lumenal side of endoplasmic reticulum membraneGO:0715569.0HLA-B, HLA-A, HLA-DQB1, HLA-C, HLA-DRB1
12ER to Golgi transport vesicle membraneGO:0125079.0HLA-B, HLA-A, HLA-DQB1, HLA-C, HLA-DRB1
13integral component of plasma membraneGO:0058878.8HLA-A, HLA-C, HLA-DRB1, COL17A1, HLA-B
14Golgi membraneGO:0001398.4HLA-B, HLA-A, HLA-DQB1, HLA-C, HLA-DRB1, BLZF1
15desmosomeGO:0300578.3EVPL, PPL, JUP, DSP, DSG1, DSG3
16extracellular vesicular exosomeGO:0700626.6PPL, HLA-B, HLA-A, HLA-C, HLA-DRB1, JUP
17plasma membraneGO:0058865.4HLA-DRB1, HLA-C, HLA-DQB1, HLA-A, HLA-B, PPL

Biological processes related to Paraneoplastic Pemphigus according to GeneCards/GeneDecks:

(show all 24)
idNameGO IDScoreTop Affiliating Genes
1epidermis developmentGO:00854410.1EVPL, COL17A1, DSP
2immunoglobulin production involved in immunoglobulin mediated immune responseGO:00238110.0HLA-DQB1, HLA-DRB1
3maternal process involved in female pregnancyGO:06013510.0DSG2, DSG1
4humoral immune response mediated by circulating immunoglobulinGO:00245510.0HLA-DRB1, HLA-DQB1
5cell-cell adhesionGO:0163379.9JUP, DSP, DSG1
6bundle of His cell to Purkinje myocyte communicationGO:0860699.9DSG2, DSC2, DSP, JUP
7ventricular cardiac muscle cell action potentialGO:0860059.9JUP, DSP, DSC2, DSG2
8regulation of heart rate by cardiac conductionGO:0860919.9JUP, DSP, DSC2, DSG2
9hemidesmosome assemblyGO:0315819.9COL17A1, DST
10peptide cross-linkingGO:0181499.8EVPL, DSP
11antigen processing and presentation of endogenous peptide antigen via MHC class I via ER pathway, TAP-independentGO:0024869.7HLA-C, HLA-A, HLA-B
12antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-independentGO:0024809.7HLA-C, HLA-A, HLA-B
13positive regulation of T cell mediated cytotoxicityGO:0019169.7HLA-C, HLA-A, HLA-B
14detection of bacteriumGO:0160459.7HLA-B, HLA-A, HLA-DRB1
15type I interferon signaling pathwayGO:0603379.6HLA-C, HLA-A, HLA-B
16antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-dependentGO:0024799.6HLA-C, HLA-A, HLA-B
17antigen processing and presentation of exogenous peptide antigen via MHC class IGO:0425909.6HLA-B, HLA-A, HLA-C
18cellular component disassembly involved in execution phase of apoptosisGO:0069219.5DSP, DSG1, DSG3, DSG2
19regulation of immune responseGO:0507769.5HLA-C, HLA-A, HLA-B
20homophilic cell adhesionGO:0071569.5DSG1, DSG3, DSC2, DSG2
21antigen processing and presentation of peptide antigen via MHC class IGO:0024749.4HLA-B, HLA-A, HLA-C
22interferon-gamma-mediated signaling pathwayGO:0603339.1HLA-B, HLA-A, HLA-DQB1, HLA-C, HLA-DRB1
23cytokine-mediated signaling pathwayGO:0192219.0HLA-B, HLA-A, HLA-DQB1, HLA-C, HLA-DRB1
24immune responseGO:0069559.0HLA-B, HLA-A, HLA-DQB1, HLA-C, HLA-DRB1

Molecular functions related to Paraneoplastic Pemphigus according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1structural molecule activityGO:0051989.8EVPL, DSP, JUP
2MHC class II receptor activityGO:0323959.5HLA-DRB1, HLA-DQB1
3TAP bindingGO:0469779.5HLA-C, HLA-A, HLA-B
4peptide antigen bindingGO:0426059.0HLA-DRB1, HLA-C, HLA-A, HLA-B
5calcium ion bindingGO:0055098.8DST, DSG1, DSG3, DSC2, DSG2

Products for genes affiliated with Paraneoplastic Pemphigus

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Sources for Paraneoplastic Pemphigus

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet