HES
MCID: HYP098
MIFTS: 61

Hypereosinophilic Syndrome (HES) malady

Genetic diseases, Rare diseases, Blood diseases, Cancer diseases categories

Summaries for Hypereosinophilic Syndrome

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44NIH Rare Diseases, 66Wikipedia, 34MalaCards
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NIH Rare Diseases:44 Hypereosinophilic syndrome is a group of rare blood disorders characterized by increased levels of eosinophils (a type of white blood cell that plays a role in the human immune system) persisting for more than six months. the signs and symptoms are due to involvement of several internal organs and there is usually no evidence of parasites, allergy, or other known causes of an elevated eosinophil count. this condition largely occurs in males, typically at middle age. it usually presents with fever, weight loss, fatigue, and rash. an enlarged liver and spleen and liver is often present. the lungs, kidneys, heart, and nervous system can also be affected. last updated: 7/11/2011

MalaCards: Hypereosinophilic Syndrome, also known as hypereosinophilic syndrome, idiopathic, is related to endomyocardial fibrosis and leukemia. An important gene associated with Hypereosinophilic Syndrome is PDGFRA (platelet-derived growth factor receptor, alpha polypeptide), and among its related pathways are Osteoblast Signaling and Cytoskeleton remodeling Role of PDGFs in cell migration. The compounds dmpq dihydrochloride and su 16f have been mentioned in the context of this disorder. Affiliated tissues include liver, heart and lung, and related mouse phenotypes are digestive/alimentary and reproductive system.

Wikipedia:66 The hypereosinophilic syndrome (HES) is a disease characterized by a persistently elevated eosinophil... more...

Aliases & Classifications for Hypereosinophilic Syndrome

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44NIH Rare Diseases, 21GeneTests, 46Novoseek, 50Orphanet, 40NCBI Bookshelf, 23GTR, 63UMLS, 64UMLS via Orphanet, 37MESH via Orphanet, 27ICD10 via Orphanet, 60SNOMED-CT via Orphanet
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Classifications:

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


Characteristics (Orphanet epidemiological data):

50
hypereosinophilic syndrome:
Prevalence: 1-9/100000; Age of onset: Variable


Aliases & Descriptions:

hypereosinophilic syndrome 44 21 46 50
hypereosinophilic syndrome, idiopathic 44 23
idiopathic hypereosinophilic syndrome 46 63
hes 44 40
chronic eosinophilic leukemia 63


External Ids:

UMLS via Orphanet64 C1540912
MESH via Orphanet37 D017681
ICD10 via Orphanet27 D47.5
SNOMED-CT via Orphanet60 128835008

Related Diseases for Hypereosinophilic Syndrome

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18GeneCards, 19GeneDecks
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Diseases in the Hypereosinophilic Syndrome family:

Primary Hypereosinophilic Syndrome

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

(show top 50)    (show all 243)
idRelated DiseaseScoreTop Affiliating Genes
1endomyocardial fibrosis30.9FIP1L1, PDGFRA
2leukemia30.8RNASE3, EPX, FIP1L1, PDGFRA, PDGFRB, KIT
3eosinophilia30.6RNASE3, EPX, FIP1L1, IL5, PDGFRA, PDGFRB
4systemic mastocytosis30.5PDGFRB
5myelofibrosis30.5FIP1L1, PDGFRA, PDGFRB
6churg-strauss syndrome30.4RNASE3, IL5
7asthma30.4IL5, EPX, RNASE3
8myeloproliferative disorder30.3PDGFRA, PDGFRB, KIT
9mastocytosis30.3KIT, PDGFRB, PDGFRA, IL5, FIP1L1
10urticaria30.1RNASE3
11helminthiasis30.0IL5, RNASE3
12bullous pemphigoid30.0RNASE3, IL5
13sarcoma30.0PDGFRA, PDGFRB, KIT
14bronchitis29.9RNASE3, IL5
15endocarditis10.5
16cerebritis10.5
17primary hypereosinophilic syndrome10.4
18eosinophilic gastroenteritis10.4
19gastroenteritis10.4
20hepatitis10.3
21myocardial infarction10.3
22restrictive cardiomyopathy10.3
23vasculitis10.3
24hypereosinophilic syndrome, idiopathic, resistant to imatinib10.3
25eosinophilic colitis10.3
26myocarditis10.3
27lymphomatoid papulosis10.3
28peripheral t-cell lymphoma10.3
29aneurysm10.3
30secondary hypereosinophilic syndrome10.3
31ulcerative colitis10.2
32cystitis10.2
33disseminated intravascular coagulation10.2
34lymphoblastic leukemia10.2
35wells syndrome10.2
36lymphoid hypereosinophilic syndrome10.2
37mini stroke10.1
38cardiac tamponade10.1
39loeffler endocarditis10.1
40portal vein thrombosis10.1
41acute leukemia10.1
42coronary aneurysm10.1
43systemic lupus erythematosus10.1
44sclerosing cholangitis10.1
45acute myocardial infarction10.1
46arthritis10.1
47cholangitis10.1
48encephalitis10.1
49lupus erythematosus10.1
50multiple sclerosis10.1

Graphical network of the top 20 diseases related to Hypereosinophilic Syndrome:



Diseases related to hypereosinophilic syndrome

Symptoms for Hypereosinophilic Syndrome

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Drugs & Therapeutics for Hypereosinophilic Syndrome

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
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Approved drugs:

Search CenterWatch for Hypereosinophilic Syndrome

Drug clinical trials:

Search ClinicalTrials for Hypereosinophilic Syndrome

Search NIH Clinical Center for Hypereosinophilic Syndrome

Search CenterWatch for Hypereosinophilic Syndrome

Genetic Tests for Hypereosinophilic Syndrome

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21GeneTests, 23GTR
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Genetic tests related to Hypereosinophilic Syndrome:

id Genetic test Affiliating Genes
1 Hypereosinophilic Syndrome21 PDGFRA
2 Idiopathic Hypereosinophilic Syndrome23

Anatomical Context for Hypereosinophilic Syndrome

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34MalaCards
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MalaCards organs/tissues related to Hypereosinophilic Syndrome:

34
Liver, Heart, Lung, Spleen, Kidney, T cells, Bone, Bone marrow, Skin, Thymus, Brain, B cells, Endothelial, B lymphoblasts

Animal Models for Hypereosinophilic Syndrome or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Hypereosinophilic Syndrome:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053818.5IL5, PDGFRA, PDGFRB, KIT
2MP:00053898.5KIT, PDGFRB, PDGFRA, IL5
3MP:00053808.2IL5, PDGFRA, PDGFRB, KIT
4MP:00053977.7KIT, PDGFRB, PDGFRA, IL5, EPX
5MP:00053877.6EPX, IL5, PDGFRA, PDGFRB, KIT

Publications for Hypereosinophilic Syndrome

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53PubMed
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Articles related to Hypereosinophilic Syndrome:

(show top 50)    (show all 536)
idTitleAuthorsYear
1
Eosinophilic cystitis presented as a manifestation of hypereosinophilic syndrome: a case report and review of the literature. (23573073)
2013
2
Hypereosinophilic syndrome masquerading as a myocardial infarction causing decompensated heart failure. (24053402)
2013
3
Effect of cyclosporine on lymphocytic variant hypereosinophilic syndrome. (23669338)
2013
4
A case of dermatopathic lymphadenopathy associated with hypereosinophilic syndrome. (22499479)
2012
5
Long-term remission of lymphocytic hypereosinophilic syndrome with imatinib mesylate. (21919038)
2012
6
Gadolinium-enhanced cardiovascular magnetic resonance for the detection and characterization of Loeffler endocarditis in patients with hypereosinophilic syndrome. (21963210)
2011
7
Hypereosinophilic syndrome presenting with large left ventricular apical thrombus and pulmonary embolism. (21854430)
2011
8
An 8-year-old boy with hypereosinophilic syndrome. (21646806)
2011
9
Hemophagocytic syndrome associated NK/T nasal type lymphoma presenting as hypereosinophilic syndrome: a case report and literature review. (21501869)
2011
10
Medical therapy alone produces regression of combined aortic and mitral valve involvement in hypereosinophilic syndrome. (20956234)
2010
11
Hypereosinophilic syndromes: pathogenic and therapeutic up-to-date]. (20303627)
2010
12
The proportion of CD3- CD4+ T-cell population remained unaffected after corticosteroids treatment for lymphocytic variant hypereosinophilic syndrome (L-HES). (20883323)
2010
13
Mepolizumab as a corticosteroid-sparing agent in lymphocytic variant hypereosinophilic syndrome. (20810155)
2010
14
Endomyocardial disease related to idiopathic hypereosinophilic syndrome: a cardiac magnetic resonance evaluation. (20396876)
2010
15
Wells Syndrome with Multiorgan Involvement Mimicking Hypereosinophilic Syndrome. (20652113)
2009
16
A novel FIP1L1-PDGFRA mutant destabilizing the inactive conformation of the kinase domain in chronic eosinophilic leukemia/hypereosinophilic syndrome. (19210352)
2009
17
Successful treatment of refractory idiopathic hypereosinophilic syndrome with etoposide. (19142161)
2009
18
Oral mucosa lesions in hypereosinophilic syndrome--an update. (18194138)
2008
19
Hypereosinophilic syndrome with isolated Loeffler's endocarditis: complete resolution with corticosteroids. (18480530)
2008
20
Hypereosinophilic syndrome and mepolizumab. (18584819)
2008
21
Clinical features of hypereosinophilic syndrome: FIP1L1-PDGFRA fusion gene-positive disease is a distinct clinical entity with myeloproliferative features and a poor response to corticosteroid. (17261495)
2007
22
Bullous pemphigoid associated with hypereosinophilic syndrome: simultaneous response to imatinib. (17097375)
2007
23
Eosinophilic ascites as the first sign of idiopathic hypereosinophilic syndrome in childhood. (17881935)
2007
24
Factor V Leiden mutation and deep venous thrombosis in a patient with hypereosinophilic syndrome. (16951787)
2006
25
Current concepts on the pathogenesis of the hypereosinophilic syndrome/chronic eosinophilic leukemia. (23662039)
2006
26
Hypereosinophilic syndrome: lymphoproliferative and myeloproliferative variants. (16612767)
2006
27
Control of hypereosinophilic syndrome-associated recalcitrant coronary artery spasm by combined treatment with prednisone, imatinib mesylate and hydroxyurea. (18651015)
2006
28
Selective priming of peripheral blood eosinophils in patients with idiopathic hypereosinophilic syndrome. (17078855)
2006
29
Intracardiac echo guided valvuloplasty of a stenotic tricuspid prosthetic valve in a patient with idiopathic hypereosinophilic syndrome. (16640713)
2006
30
Interferon treatment for hypereosinophilic syndromes and systemic mastocytosis. (15995323)
2005
31
Thrombotic microangiopathy associated with the hypereosinophilic syndrome. (15840027)
2005
32
The hypereosinophilic syndrome: idiopathic or not, that is the question. (15921372)
2005
33
Hypereosinophilic syndrome: long-term remission following allogeneic stem cell transplant in spite of transient eosinophilia post-transplant. (15609294)
2005
34
Alemtuzumab therapy for refractory idiopathic hypereosinophilic syndrome. (14984510)
2004
35
Successful treatment of hypereosinophilic syndrome after remission of acute lymphoblastic leukemia in a patient who underwent non-myeloablative stem cell transplantation. (15075099)
2004
36
Rapid reversion of Loeffler's endocarditis by imatinib in early stage clonal hypereosinophilic syndrome. (15621768)
2004
37
Safety and efficacy of the monoclonal anti-interleukin-5 antibody SCH55700 in the treatment of patients with hypereosinophilic syndrome. (15070668)
2004
38
Imatinib-mesylate for all patients with hypereosinophilic syndrome? (15158099)
2004
39
Idiopathic hypereosinophilic syndrome manifesting as pulmonary oedema. (12723663)
2003
40
Hypereosinophilic syndrome with pulmonary and cardiac involvement in a patient with asthma. (12538545)
2003
41
Hypereosinophilic dermatosis: skin lesions as the only manifestation of the idiopathic hypereosinophilic syndrome? (10971369)
2000
42
Hypereosinophilic syndrome. (10681232)
2000
43
Interferon-alpha in the idiopathic hypereosinophilic syndrome: consideration of five cases. (9829847)
1998
44
Wells' syndrome associated with idiopathic hypereosinophilic syndrome. (9470919)
1997
45
Sclerosing cholangitis associated with hypereosinophilic syndrome. (9260773)
1997
46
Hypereosinophilic syndrome or chronic eosinophilic leukemia: report of a case with a lytic bone lesion. (9180305)
1997
47
Idiopathic hypereosinophilic syndrome and pregnancy. (9197455)
1997
48
Allogeneic bone marrow transplantation for hypereosinophilic syndrome with advanced myelofibrosis. (9156253)
1997
49
Idiopathic hypereosinophilic syndrome revealed by central nervous system dysfunction. (8711988)
1996
50
alpha-Interferon and hypereosinophilic syndrome with trisomy 8: karyotypic remission. (7718904)
1995

Variations for Hypereosinophilic Syndrome

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Expression for genes affiliated with Hypereosinophilic Syndrome

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

Search GEO for disease gene expression data for Hypereosinophilic Syndrome.

Pathways for genes affiliated with Hypereosinophilic Syndrome

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Sources:
51PathCards, 39NCBI BioSystems Database, 61Thomson Reuters, 54QIAGEN, 55R&D Systems, 5Cell Signaling Technology, 31KEGG, 62Tocris Bioscience, 56Reactome, 52PharmGKB
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Pathways related to Hypereosinophilic Syndrome according to GeneCards/GeneDecks:

(show all 31)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.6PDGFRB, PDGFRA
2
Show member pathways
PDGF Pathway39
9.6PDGFRB, PDGFRA
39.6PDGFRB, PDGFRA
49.6PDGFRB, PDGFRA
59.6PDGFRB, PDGFRA
69.6PDGFRB, PDGFRA
79.6PDGFRA, PDGFRB
89.5KIT, PDGFRA
99.3KIT, IL5
10
Show member pathways
9.1IL5, PDGFRA, PDGFRB
11
Show member pathways
9.0KIT, PDGFRB, PDGFRA
12
Show member pathways
9.0PDGFRA, PDGFRB, KIT
13
Show member pathways
9.0PDGFRA, PDGFRB, KIT
149.0KIT, PDGFRB, PDGFRA
15
Show member pathways
9.0PDGFRA, PDGFRB, KIT
16
Show member pathways
9.0PDGFRA, PDGFRB, KIT
17
Show member pathways
9.0PDGFRA, PDGFRB, KIT
18
Show member pathways
9.0KIT, PDGFRB, PDGFRA
199.0PDGFRA, PDGFRB, KIT
20
Show member pathways
Development EDNRB signaling61
Development ACM2 and ACM4 activation of ERK61
Cell adhesion Integrin inside out signaling61
Development G Proteins mediated regulation MARK ERK signaling61
Signal transduction IP3 signaling61
Development Angiotensin signaling via PYK261
Development EPO induced MAPK pathway61
9.0KIT, PDGFRB, PDGFRA
219.0KIT, PDGFRB, PDGFRA
22
Show member pathways
9.0PDGFRA, PDGFRB, KIT
23
Show member pathways
9.0KIT, PDGFRB, PDGFRA
24
Show member pathways
9.0KIT, PDGFRB, PDGFRA
25
Show member pathways
9.0KIT, PDGFRB, PDGFRA
26
Show member pathways
8.6RNASE3, EPX, IL5
27
Show member pathways
8.5IL5, PDGFRA, PDGFRB, KIT
28
Show member pathways
8.5IL5, PDGFRA, PDGFRB, KIT
29
Show member pathways
8.5IL5, PDGFRA, PDGFRB, KIT
30
Show member pathways
8.5IL5, PDGFRA, PDGFRB, KIT
31
Show member pathways
8.5IL5, PDGFRA, PDGFRB, KIT

Compounds for genes affiliated with Hypereosinophilic Syndrome

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62Tocris Bioscience, 46Novoseek, 30IUPHAR, 12DrugBank, 52PharmGKB, 25HMDB
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Compounds related to Hypereosinophilic Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 82)
idCompoundScoreTop Affiliating Genes
1dmpq dihydrochloride6210.0PDGFRA, PDGFRB
2su 16f6210.0PDGFRA, PDGFRB
3sunitinib malate6210.0PDGFRB, PDGFRA
4su 66686210.0PDGFRB, PDGFRA
5mometasone furoate46 6210.8IL5, RNASE3
6unicap469.8RNASE3, IL5
7su6668469.8PDGFRB, KIT
8levocabastine46 30 1211.8RNASE3, IL5
9beclomethasone dipropionate469.7IL5, RNASE3
10zd6474469.7KIT, PDGFRB
11toluene diisocyanate469.7RNASE3, IL5
12nedocromil sodium469.7IL5, RNASE3
13su5416469.5KIT, PDGFRB
14pranlukast46 30 1211.5IL5, RNASE3
15pkc 412469.5PDGFRB, KIT, PDGFRA
16capecitabine46 52 1211.5KIT, PDGFRB
17ag-1296469.5PDGFRA, KIT, PDGFRB
18pazopanib52 1210.5PDGFRA, PDGFRB, KIT
19sunitinib46 52 1211.5KIT, PDGFRB, PDGFRA
20regorafenib52 1210.4PDGFRA, PDGFRB, KIT
21praziquantel46 1210.4IL5, RNASE3
22tele-methylhistamine469.4RNASE3, EPX
23ltd4469.4RNASE3, IL5
24sephadex469.3RNASE3, EPX
25azelastine46 1210.1IL5, RNASE3
26lte4469.1RNASE3, EPX, IL5
27sodium cromoglycate469.1RNASE3, IL5, EPX
28cetirizine46 30 25 1212.1EPX, IL5, RNASE3
29ketotifen46 1210.0RNASE3, EPX, IL5
30salmeterol46 52 30 1212.0RNASE3, EPX, IL5
31ltc4469.0EPX, RNASE3, IL5
32budesonide46 52 1211.0IL5, EPX, RNASE3
33salbutamol46 52 30 25 1213.0EPX, RNASE3, IL5
34pgd2469.0IL5, EPX, RNASE3
35allergens469.0EPX, IL5, RNASE3
36theophylline46 30 25 1212.0IL5, EPX, RNASE3
37leukotriene b446 62 2511.0IL5, RNASE3, EPX
38prednisolone46 30 1210.9RNASE3, EPX, IL5
39paraffin468.9PDGFRB, PDGFRA, KIT, IL5
40p002468.8KIT, EPX, RNASE3
41fmlp468.8EPX, IL5, RNASE3
42formaldehyde46 259.8EPX, RNASE3, KIT
43oligonucleotide468.7RNASE3, PDGFRA, PDGFRB, KIT
44imatinib46 52 1210.5PDGFRB, PDGFRA, IL5, FIP1L1, KIT
45h2o2468.4RNASE3, PDGFRB, IL5, EPX
46adp46 30 2510.4KIT, PDGFRB, PDGFRA, RNASE3
47histamine46 30 2510.4KIT, IL5, EPX, RNASE3
48vegf468.4KIT, PDGFRB, PDGFRA, IL5, RNASE3
49retinoic acid46 259.4PDGFRB, KIT, PDGFRA, IL5, RNASE3
50tyrosine467.2KIT, RNASE3, EPX, IL5, PDGFRA, PDGFRB

GO Terms for genes affiliated with Hypereosinophilic Syndrome

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17Gene Ontology
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Cellular components related to Hypereosinophilic Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1intrinsic component of plasma membraneGO:0312269.6PDGFRB, PDGFRA

Biological processes related to Hypereosinophilic Syndrome according to GeneCards/GeneDecks:

(show all 24)
idNameGO IDScoreTop Affiliating Genes
1metanephric glomerular capillary formationGO:07227710.0PDGFRA, PDGFRB
2positive regulation of cell proliferation by VEGF-activated platelet derived growth factor receptor signaling pathwayGO:03809110.0PDGFRA, PDGFRB
3retina vasculature development in camera-type eyeGO:06129810.0PDGFRB, PDGFRA
4cardiac myofibril assemblyGO:0550039.9PDGFRB, PDGFRA
5adrenal gland developmentGO:0303259.8PDGFRB, PDGFRA
6platelet-derived growth factor receptor signaling pathwayGO:0480089.8PDGFRA, PDGFRB
7wound healingGO:0420609.6PDGFRA, PDGFRB
8positive regulation of ERK1 and ERK2 cascadeGO:0703749.6PDGFRB, PDGFRA
9positive regulation of JAK-STAT cascadeGO:0464279.6KIT, IL5
10positive regulation of phospholipase C activityGO:0108639.4KIT, PDGFRB, PDGFRA
11positive regulation of phosphatidylinositol 3-kinase activityGO:0435529.4KIT, PDGFRB, PDGFRA
12cell chemotaxisGO:0603269.4PDGFRA, PDGFRB, KIT
13positive regulation of phosphatidylinositol 3-kinase signalingGO:0140689.4KIT, PDGFRB, PDGFRA
14peptidyl-tyrosine phosphorylationGO:0181089.3KIT, PDGFRB, PDGFRA
15positive regulation of cell migrationGO:0303359.3PDGFRA, PDGFRB, KIT
16phosphatidylinositol-mediated signalingGO:0480159.3KIT, PDGFRB, PDGFRA
17protein autophosphorylationGO:0467779.3PDGFRA, PDGFRB, KIT
18fibroblast growth factor receptor signaling pathwayGO:0085439.3KIT, PDGFRB, PDGFRA
19Fc-epsilon receptor signaling pathwayGO:0380959.2PDGFRA, PDGFRB, KIT
20positive regulation of sequence-specific DNA binding transcription factor activityGO:0510919.2KIT, IL5
21epidermal growth factor receptor signaling pathwayGO:0071739.2KIT, PDGFRB, PDGFRA
22neurotrophin TRK receptor signaling pathwayGO:0480119.1KIT, PDGFRB, PDGFRA
23positive regulation of cell proliferationGO:0082849.1KIT, PDGFRB, PDGFRA
24innate immune responseGO:0450878.7KIT, PDGFRB, PDGFRA

Molecular functions related to Hypereosinophilic Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1vascular endothelial growth factor bindingGO:0380859.7PDGFRB, PDGFRA
2platelet-derived growth factor bindingGO:0484079.6PDGFRB, PDGFRA
3platelet-derived growth factor receptor bindingGO:0051619.5PDGFRA, PDGFRB
4protein tyrosine kinase activityGO:0047139.4KIT, PDGFRB
5transmembrane receptor protein tyrosine kinase activityGO:0047149.2KIT, PDGFRA

Products for genes affiliated with Hypereosinophilic Syndrome

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Hypereosinophilic Syndrome

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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