MCID: PMS001
MIFTS: 67

Poems Syndrome malady

Neuronal diseases, Blood diseases, Cancer diseases, Skin diseases, Bone diseases categories

Summaries for Poems Syndrome

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42NIH Rare Diseases, 63Wikipedia, 32MalaCards
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NIH Rare Diseases:42 Poems syndrome is a rare blood disorder. it is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma proliferative disorder, and skin changes. signs and symptoms may include progressive sensorimotor polyneuropathy, enlarged liver and spleen, swollen lymph nodes, and darkening of the skin. further testing and evaluation may show plasma cell disorder and sclerotic bone lesions. many people with poems syndrome have diabetes (20-50%) and primary gonadal failure (55-70%). other general signs and symptoms may include increased body hair, thickening of the skin, whitening of the nails, fatigue, weakness, swelling of the ankles and legs, enlarged breast tissue, generalized aches and pains, fluid in the lungs, and vision changes. sign and symptoms tend to begin in a person's 40's to 50's. poems syndrome is a rapidly progressive disorder that can become life threatening. currently the exact cause of poems syndrome is unknown. researchers believe the syndrome involves an overgrowth of bone marrow cells (plasma cells), which produce chemicals that damage other parts of the body. last updated: 2/18/2013

MalaCards: Poems Syndrome, also known as osteosclerotic myeloma, is related to polyneuropathy and myeloma, and has symptoms including renal disease/nephropathy, hypothalamic-hypophyseal axis functional anomalies/hypopituitarism and tapered fingers. An important gene associated with Poems Syndrome is VEGFA (vascular endothelial growth factor A), and among its related pathways are Rheumatoid arthritis and Prion diseases. The compounds indomethacin and pge2 have been mentioned in the context of this disorder. Affiliated tissues include skin, bone and liver, and related mouse phenotypes are tumorigenesis and immune system.

Wikipedia:63 POEMS syndrome (also known as Crow–Fukase syndrome, Takatsuki disease, or PEP syndrome) is a rare... more...

Aliases & Classifications for Poems Syndrome

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8Disease Ontology, 42NIH Rare Diseases, 10DISEASES, 44Novoseek, 48Orphanet, 60UMLS, 34MeSH, 39NCIt, 56SNOMED-CT, 35MESH via Orphanet, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet
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Classifications:



Characteristics (Orphanet epidemiological data):

48
poems syndrome:
Prevalence: <1/1000000; Age of onset: Adulthood


Aliases & Descriptions:

poems syndrome 8 42 10 44 48 60
osteosclerotic myeloma 48 60
crow-fukase syndrome 42 48
polyneuropathy, organomegaly, endocrinopathy, m protein, and skin changes syndrome 42
polyneuropathy - endocrinopathy - plasma cell dyscrasia 48
polyneuropathy organomegaly 42
takatsuki syndrome 48
pep syndrome 48


External Ids:

Disease Ontology8 DOID:14039
MeSH34 D016878
NCIt39 C80303
MESH via Orphanet35 D016878
SNOMED-CT56 79268002
ICD10 via Orphanet26 D47.7
SNOMED-CT via Orphanet57 425657001, 79268002
UMLS via Orphanet61 C0085404, C1510415

Related Diseases for Poems Syndrome

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17GeneCards, 18GeneDecks
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Diseases related to Poems Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 260)
idRelated DiseaseScoreTop Affiliating Genes
1polyneuropathy31.1MAG
2myeloma30.8IL1B, IL6, CD36, MYOM2, BGLAP, CCL3
3hypertension30.8CST3
4neuropathy30.7MAG
5multiple myeloma30.5IL6, NCAM1, TNFSF11
6plasmacytoma30.4IL6
7skin disease30.4IL1B, IL6
8vascular disease30.4CST3, TNFSF11, TNFRSF11B, VEGFA
9amyloidosis30.3CST3
10osteosclerosis30.3TNFRSF11B, TNFSF11
11pulmonary fibrosis30.2IL1B, CD36, CCL3, TIMP1
12polycythemia30.1TIMP1
13polycythemia vera30.1TIMP1
14myocardial infarction30.1CCL11, CCL3, CST3
15glomerulonephritis29.9IL1B
16familial mediterranean fever29.9IL1B
17osteonecrosis29.9BGLAP
18macroglobulinemia29.9MAG
19acromegaly29.9BGLAP
20myeloid leukemia29.9CSF3
21kaposi's sarcoma29.9IL6
22thrombocytosis29.9CSF3, IL1B, IL6
23hyperparathyroidism29.9BGLAP, TNFSF11, TNFRSF11B
24thrombocytopenia29.9CSF3, IL6
25arthritis29.9TIMP1, TNFSF11, IL1B, IL6
26diabetes mellitus29.9BGLAP, CST3, VEGFA, CD36, IL1B, IL6
27leukemia29.9CCL11, CCL3, CSF3, NCAM1, IL1B, IL6
28multiple sclerosis29.9CST3, MAG, IL5, IL1B, IL6, CCL3
29melanoma29.9TIMP1, CCL11, CCL3, CST3, TNFSF11, TNFRSF11B
30rheumatoid arthritis29.9TIMP1, BGLAP, CCL11, CCL3, CST3, TNFSF11
31hemangioma10.6
32glomeruloid hemangioma10.5
33osteosclerotic myeloma10.5
34extramedullary plasmacytoma10.5
35demyelinating polyneuropathy10.3
36calciphylaxis10.3
37peripheral neuropathy10.3
38image syndrome10.3
39stroke, ischemic10.2
40cryoglobulinemia10.2
41cerebritis10.2
42chronic polyneuropathy10.2
43cystoid macular edema10.1
44alopecia10.1
45congestive heart failure10.1
46thyroiditis10.1
47vitamin b12 deficiency10.1
48blindness10.0VEGFA
49retinopathy of prematurity10.0VEGFA
50malignant glioma10.0VEGFA

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



Diseases related to poems syndrome

Clinical Features for Poems Syndrome

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48Orphanet
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Symptoms:

48 (show all 23)
  • renal disease/nephropathy
  • hypothalamic-hypophyseal axis functional anomalies/hypopituitarism
  • tapered fingers
  • lymphadenopathy/polyadenopathies
  • decreased body hair/axillar/pubic hairlessness
  • hepatomegaly/liver enlargement (excluding storage disease)
  • splenomegaly
  • insulin-independent/type 2 diabetes
  • nerve conduction abnormality
  • late puberty/hypogonadism/hypogenitalism
  • osteosclerosis/osteopetrosis/bone condensation
  • irregular/in bands/reticular skin hyperpigmentation
  • hirsutism/hypertrichosis/increased body hair
  • thick skin/pachydermia/orange skin
  • lymphedema
  • arteriovenous malformations/vascular malformations (excluding port-wine stains)
  • precocious menopause/secondary amenorrhea
  • agammaglobulinemia/hypogammaglobulinemia/b-cell deficiency
  • cortico-adrenal hypoplasia/insufficiency
  • hypotrichosis/atrichia/atrichiasis/scalp hairlessness
  • gynecomastia/breast/mammary gland enlargement/hyperplasia
  • bone/osseous neoplasm/tumor/carcinoma/cancer
  • hypothyroidy

Drugs & Therapeutics for Poems Syndrome

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Poems Syndrome

Drug clinical trials:

Search ClinicalTrials for Poems Syndrome

Search NIH Clinical Center for Poems Syndrome

Search CenterWatch for Poems Syndrome

Genetic Tests for Poems Syndrome

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Anatomical Context for Poems Syndrome

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32MalaCards
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MalaCards organs/tissues related to Poems Syndrome:

32
Skin, Bone, Liver, Breast, Lymph node, Testes, Lung, Spleen, Endothelial, Bone marrow, B cells, Heart, Thyroid, Myeloid, Kidney, Pancreas, Pituitary, Colon

Animal Models for Poems Syndrome or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Poems Syndrome:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020069.7CST3, IL5, IL1B, IL6, TIMP1
2MP:00053878.3IL6, TIMP1, CCL11, CCL3, CSF3, TNFSF11
3MP:00053978.3CSF3, CCL3, CCL11, TIMP1, TNFSF11, TNFRSF11B

Publications for Poems Syndrome

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50PubMed
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Articles related to Poems Syndrome:

(show top 50)    (show all 429)
idTitleAuthorsYear
1
POEMS syndrome and Castleman disease. (24006526)
2013
2
Does suppression of VEGF alone lead to clinical recovery in POEMS syndrome? (23644502)
2013
3
14q32 translocations and 13q14 deletions are common cytogenetic abnormalities in POEMS syndrome. (23957213)
2013
4
New advances in the diagnosis and treatment of POEMS syndrome. (23398538)
2013
5
Electrophysiological features of POEMS syndrome and chronic inflammatory demyelinating polyneuropathy. (24268501)
2013
6
Pulmonary hypertension in POEMS syndrome. (22983590)
2013
7
Preserved autonomic function in patients with POEMS syndrome. (22507753)
2012
8
Acquired facial atrophy: a neglected clinical sign of POEMS syndrome. (22076924)
2012
9
POEMS Syndrome Diagnosed 10a88Years after Disabling Peripheral Neuropathy. (22013451)
2011
10
Development of POEMS syndrome after an initial manifestation of solitary plasmacytoma. (21553018)
2011
11
POEMS syndrome with calciphylaxis: A case report. (21031277)
2011
12
Intracranial hemangiomas in a patient with POEMS syndrome. (19943169)
2010
13
Primary cutaneous diffuse large B-cell lymphoma, leg type, with features simulating POEMS syndrome. (19558504)
2010
14
POEMS syndrome with an isolated lytic lesion and glomeruloid hemangiomas: report of one case]. (19701559)
2009
15
POEMS syndrome complicated by follicular lymphoma. (20009437)
2009
16
Calciphylaxis as a Catastrophic Complication in a Patient with POEMS Syndrome. (20847836)
2009
17
Poems syndrome--a rare variant of plasma cell dyscrasia. Case report and review of literature. (19670534)
2009
18
Systemic corticosteroids as first-line treatment in pulmonary hypertension associated with POEMS syndrome. (19750334)
2009
19
VEGF and osteosclerosis in POEMS syndrome. (18172647)
2008
20
Multiple capillary hemangiomas: a distinctive lesion of multicentric Castleman's disease and POEMS syndrome. (18797059)
2008
21
Paroxysmal hemicrania and POEMS syndrome: further evidence that neuropeptides are implicated in primary headache? (18644027)
2008
22
Endocrinopathy in POEMS syndrome: the Mayo Clinic experience. (17605964)
2007
23
POEMS syndrome presenting with transient immunoglobulin isotype switching after successful treatment with autologous peripheral blood stem cell transplantation]. (17867301)
2007
24
Optic disc edema, cystoid macular edema, and elevated vascular endothelial growth factor in a patient with POEMS syndrome. (17895817)
2007
25
POEMS syndrome-related pulmonary hypertension is steroid-responsive. (16774822)
2007
26
POEMS syndrome. (17850941)
2007
27
POEMS syndrome: failure of newly suggested diagnostic criteria to anticipate the development of the syndrome. (16044435)
2005
28
A case of POEMS syndrome associated with reactive amyloidosis and WaldenstrAPm's macroglobulinaemia. (12270006)
2002
29
Chronic myeloid leukemia and acromegaly in POEMS syndrome. (12443888)
2002
30
POEMS syndrome: a case report and discussion. (11378655)
2001
31
Proptosis with orbital soft tissue and bone changes and unilateral papilloedema: unusual presentation of POEMS syndrome. (11274880)
2000
32
Kaposi's sarcoma herpesvirus-associated Castleman's disease with POEMS syndrome. (10679723)
2000
33
Human herpesvirus 8 infection in patients with POEMS syndrome-associated multicentric Castleman's disease. (10339470)
1999
34
POEMS syndrome, steroid-dependent diabetes mellitus, erythema elevatum diutinum, and rheumatoid arthritis as extramedullary manifestations of plasma cell dyscrasia. (10074579)
1998
35
POEMS syndrome in a patient with diabetic ketoacidosis: case report and review. (9640554)
1998
36
Multiple myeloma associated with diffuse osteosclerotic bone lesions: a clinical entity distinct from osteosclerotic myeloma (POEMS syndrome). (9395194)
1997
37
Castleman disease in POEMS syndrome with elevated interleukin-6. (8499003)
1993
38
POEMS syndrome in association with Sweet's syndrome--a new variant? (8479179)
1993
39
POEMS syndrome with myocardial infarction: observations concerning pathogenesis and review of the literature. (1295088)
1992
40
The kidney disease of Crow-Fukase (POEMS) syndrome: a clinico-pathological study of four cases. (1934663)
1991
41
Premature vascular calcification in a case of POEMS syndrome. (1756747)
1991
42
POEMS syndrome. (1816211)
1991
43
Castleman disease-POEMS syndrome overlap. (1994802)
1991
44
POEMS syndrome: a rarely diagnosed multisystem disorder. (2163715)
1990
45
POEMS syndrome or Shimpo's disease? (2175226)
1990
46
Multicentric angiofollicular lymph node hyperplasia associated with a solitary osteolytic costal IgG lambda myeloma. POEMS syndrome in a South American (Paraguayan) patient. (2602220)
1989
47
Cutaneous angiomas in POEMS syndrome. An ultrastructural and immunohistochemical study. (3284467)
1988
48
Uncompacted lamellae in three patients with POEMS syndrome. (3040418)
1987
49
Therapeutic trial of plasma exchange in osteosclerotic myeloma associated with the POEMS syndrome. (2993255)
1985
50
The POEMS syndrome: report of three cases with radiographic abnormalities. (6505218)
1984

Genetic Variations for Poems Syndrome

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Expression for genes affiliated with Poems Syndrome

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Poems Syndrome

Search GEO for disease gene expression data for Poems Syndrome.

Pathways for genes affiliated with Poems Syndrome

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29KEGG, 52R&D Systems, 12EMD Millipore, 51QIAGEN, 37NCBI BioSystems Database, 53Reactome
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Pathways related to Poems Syndrome according to GeneCards/GeneDecks:

(show all 22)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.3TNFSF11
210.3NCAM1
310.1IL1B, IL5
4
Transcription Role of VDR in regulation of genes involved in osteoporosis
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10.1TNFRSF11B, TNFSF11
510.0IL5, IL1B, IL6
610.0TIMP1, TNFSF11, IL6
7
Hide members
10.0IL5, CCL11, TIMP1
8
Hide members
9.9CD36, VEGFA, TIMP1
99.9CCL3, IL1B, IL6
109.9CCL3, CD36, IL1B
119.9BGLAP, IL5, IL6
12
Immune response Oncostatin M signaling via JAK-Stat in human cells
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9.8IL6, VEGFA, CCL11, TIMP1
13
Hide members
9.8IL6, IL1B, IL5, CCL11
14
Hide members
9.8CD36, IGLV1-44, IGLV1-40
15
Hide members
9.8IL6, IL1B, IL5, NCAM1
16
Hide members
9.7IL5, TNFSF11, CCL3, TIMP1
179.7CCL11, TNFSF11, IL5, IL1B, IL6
189.5CSF3, CD36, IL5, IL1B, IL6
19
Hide members
9.2TIMP1, CCL11, CCL3, CSF3, TNFSF11, IL5
20
Hide members
9.2NCAM1, IGLV1-40, IGLV1-44, CD36, IL5, IL1B
21
Hide members
9.0IL5, TIMP1, CCL11, CCL3, CSF3, TNFSF11
22
Hide members
8.9IL5, TNFSF11, CSF3, CCL3, CCL11, TIMP1

Compounds for genes affiliated with Poems Syndrome

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Sources:
44Novoseek, 59Tocris Bioscience, 28IUPHAR, 11DrugBank, 2BitterDB, 49PharmGKB, 24HMDB
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Compounds related to Poems Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 155)
idCompoundScoreTop Affiliating Genes
1indomethacin44 59 28 1113.7IL5
2pge24410.6CCL11, VEGFA
3matrigel4410.5CCL11, NCAM1
4hydrocortisone44 2 59 1113.4TNFRSF11B, BGLAP
5prednisolone44 28 1112.1TNFRSF11B, CCL11, IL5, BGLAP
6clodronate44 49 1112.1TNFSF11, IL6, TNFRSF11B, CD36, IL1B
7prostaglandin4410.1TNFRSF11B, IL1B, TNFSF11
8mip 1alpha4410.0TNFSF11, IL6, IL1B, CCL3
9procollagen4410.0TNFSF11
10teriparatide44 1111.0BGLAP, TNFSF11, CD36, TNFRSF11B
11mcp 14410.0CCL3, TIMP1, IL5, IL6
12etanercept44 49 1112.0IL6, IL1B, TNFRSF11B, TNFSF11, TIMP1
13ibandronate44 1111.0BGLAP, CD36, TNFRSF11B, TNFSF11
14poly(i-c)449.9IL5, CCL3, IL1B, IL6, CCL11
15pamidronate44 49 1111.9BGLAP, TNFSF11, IL6, CD36, TNFRSF11B
16risedronate44 59 49 28 1113.9CD36, TNFRSF11B, BGLAP, TNFSF11, IL1B
17vitamin k2449.9TNFRSF11B, TNFSF11, BGLAP, CD36
18ns 39844 5910.9VEGFA, IL1B, TNFRSF11B, BGLAP, TNFSF11
19rantes449.9IL6, CCL11, IL1B, CCL3, CSF3
20thalidomide44 49 59 1112.9TNFRSF11B, VEGFA, IL6, CD36, IL5, IL1B
21budesonide44 49 1111.8CCL11, CD36, IL5, IL1B, BGLAP
22peptidoglycan44 2810.8IL6, IL1B, TNFSF11, CCL3, CCL11
23alendronate44 49 1111.8CD36, TNFRSF11B, TNFSF11, BGLAP, TIMP1
2415-deoxy-delta-12,14-prostaglandin j2449.8IL6, IL1B, CD36, VEGFA, CCL11
25zoledronic acid449.8IL6, TNFSF11, CD36, BGLAP, TNFRSF11B, VEGFA
26titanium449.8TNFSF11, BGLAP, TNFRSF11B, CD36, CCL3
27deoxypyridinoline449.8TNFSF11, CCL3, BGLAP, CD36, TNFRSF11B
28pmma449.7CCL3, IL6, TNFRSF11B, TNFSF11
29methylcellulose449.7TIMP1, CCL3, CSF3, IL5, IL6
30tacrolimus44 49 1111.7TIMP1, CCL3, IL5, TNFSF11
31n acetylcysteine449.7TIMP1, CCL11, CST3, TNFSF11, VEGFA, IL1B
32infliximab44 49 1111.7TNFRSF11B, VEGFA, IL1B, IL6, TIMP1, BGLAP
33herbimycin a44 5910.6IL6, TIMP1, CCL11, IL1B, IL5, CD36
34hyaluronic acid44 2410.6TIMP1, IL1B, VEGFA, MYOM2, CD36, IL6
35tgf beta1449.6TNFRSF11B, CCL3, IL6, CD36, IL1B, TIMP1
36ccl344 2810.6CCL3, IL6, IL1B, CSF3, CCL11
37calcitriol44 59 11 2412.5BGLAP, TNFSF11, TNFRSF11B, CD36, IL1B, IL6
38celecoxib44 59 28 49 11 2414.5TNFRSF11B, IL1B, CCL3, TNFSF11, VEGFA
39dexamethasone44 49 28 1112.5TIMP1, BGLAP, CCL11, TNFSF11, TNFRSF11B, VEGFA
40methotrexate44 49 1111.4IL5, TNFSF11, CSF3, CST3, TIMP1, IL6
41genistein44 28 59 2 11 2414.4TIMP1, CCL11, VEGFA, TNFRSF11B, IL1B, IL6
42cyclosporin a44 28 5911.4TNFRSF11B, CSF3, CST3, CCL3, TNFSF11, CCL11
43vitamin d449.3BGLAP, TIMP1, TNFSF11, IL6, TNFRSF11B, CD36
44histamine44 28 2411.3IL6, IL1B, IL5, NCAM1, CCL3, BGLAP
45ly294002449.2VEGFA, TNFSF11, TIMP1, CCL11, CCL3, IL6
46heparin44 28 11 2412.1TNFSF11, TIMP1, BGLAP, CCL3, TNFRSF11B, NCAM1
47sb 20358044 599.9IL6, TIMP1, CCL3, IL1B, IL5, CD36
48cysteine448.7IL5, TIMP1, BGLAP, CCL3, CST3, TNFSF11
49retinoic acid44 249.6TIMP1, IL6, BGLAP, CSF3, CCL3, CCL11
50vegf448.2CCL11, CCL3, CSF3, TNFSF11, TNFRSF11B, NCAM1

GO Terms for genes affiliated with Poems Syndrome

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16Gene Ontology
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Cellular components related to Poems Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular regionGO:0055768.3CST3, CCL3, CCL11, BGLAP, TNFSF11, TNFRSF11B
2extracellular spaceGO:0056158.0IL6, BGLAP, CCL11, CCL3, CST3, CSF3

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

(show all 18)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of intracellular protein kinase cascadeGO:01074010.2VEGFA, TNFSF11
2positive regulation of interleukin-6 productionGO:03275510.2IL6, IL1B, CD36
3monocyte chemotaxisGO:00254810.2TNFSF11, CCL3, IL6
4negative regulation of neuron deathGO:190121510.2CSF3, IL6
5positive regulation of immunoglobulin secretionGO:05102410.2IL5, IL6
6positive regulation of sequence-specific DNA binding transcription factor activityGO:05109110.1TNFSF11, IL1B, IL5, IL6
7positive regulation of peptidyl-serine phosphorylationGO:03313810.1IL6, VEGFA, CSF3
8regulation of cell shapeGO:00836010.1CCL11, CCL3, VEGFA, IL6
9positive regulation of angiogenesisGO:04576610.0VEGFA, CCL11, IL1B
10eosinophil chemotaxisGO:04824510.0CCL11, CCL3
11platelet activationGO:0301689.9VEGFA, CD36, IL6, TIMP1
12positive regulation of protein kinase B signaling cascadeGO:0518979.9TNFSF11, CCL3, IL6, CSF3
13inflammatory responseGO:0069549.8IL1B, CCL3, IL5, CCL11, IL6
14positive regulation of peptidyl-tyrosine phosphorylationGO:0507319.8CSF3, VEGFA, CD36, IL5, IL6
15immune responseGO:0069559.7CCL11, TNFSF11, IL5, IL1B, CSF3
16platelet degranulationGO:0025769.6CD36, VEGFA, TIMP1
17cytokine-mediated signaling pathwayGO:0192219.6TNFSF11, NCAM1, IL5, IL1B, IL6, CSF3
18cell adhesionGO:0071559.5CCL11, MAG, BGLAP, CD36, NCAM1

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

idNameGO IDScoreTop Affiliating Genes
1growth factor activityGO:0080839.7IL6, IL5, VEGFA, CSF3
2cytokine activityGO:0051258.9IL6, CSF3, TNFSF11, TNFRSF11B, VEGFA, IL5

Products for genes affiliated with Poems Syndrome

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Poems Syndrome

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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