MCID: PLM036
MIFTS: 69

Pulmonary Fibrosis malady

Genetic diseases (common), Respiratory diseases categories
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Summaries for Pulmonary Fibrosis

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MedlinePlus:33 Pulmonary fibrosis is a condition in which the tissue deep in your lungs becomes scarred over time. this tissue gets thick and stiff. that makes it hard for you to catch your breath, and your blood may not get enough oxygen. causes of pulmonary fibrosis include environmental pollutants, some medicines, some connective tissue diseases, and interstitial lung disease. interstitial lung disease is the name for a large group of diseases that inflame or scar the lungs. in most cases, the cause cannot be found. this is called idiopathic pulmonary fibrosis. symptoms include shortness of breath a dry, hacking cough that doesn't get better fatigue weight loss for no known reason aching muscles and joints clubbing, which is the widening and rounding of the tips of the fingers or toes your doctor may use your medical history, imaging tests, a biopsy, and lung function tests to diagnose pulmonary fibrosis. there is no cure. treatments can help with symptoms and improve your quality of life. they include medicines, oxygen therapy, pulmonary rehabilitation, or a lung transplant. nih: national heart, lung, and blood institute

MalaCards based summary: Pulmonary Fibrosis, also known as fibrosis of lung, is related to idiopathic pulmonary fibrosis and interstitial lung disease. An important gene associated with Pulmonary Fibrosis is SFTPA2 (surfactant protein A2), and among its related pathways are FOXA1 transcription factor network and Phagosome. The drug oxygen and the compounds sivelestat sodium hydrate and curosurf have been mentioned in the context of this disorder. Affiliated tissues include lung, testes and heart, and related mouse phenotypes are tumorigenesis and growth/size/body.

Wikipedia:65 Pulmonary fibrosis (literally \"scarring of the lungs\") is a respiratory disease in which scars are... more...

Aliases & Classifications for Pulmonary Fibrosis

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Pulmonary Fibrosis, Aliases & Descriptions:

Name: Pulmonary Fibrosis 30 8 10 44 33 62
Fibrosis of Lung 8 62
Post-Inflammatory Pulmonary Fibrosis 62
 
Postinflammatory Pulmonary Fibrosis 8
Lung: Cirrhosis of 8


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Genetic diseases (common)
Anatomical: Respiratory diseases


External Ids:

Disease Ontology8 DOID:3770
NCIt39 C26869
MeSH34 D011658

Related Diseases for Pulmonary Fibrosis

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Diseases in the Pulmonary Fibrosis family:

Localized Pulmonary Fibrosis Pulmonary Fibrosis, Familial
Terc-Related Familial Pulmonary Fibrosis Tert-Related Familial Pulmonary Fibrosis
Sftpc-Related Familial Pulmonary Fibrosis

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

(show top 50)    (show all 199)
idRelated DiseaseScoreTop Affiliating Genes
1idiopathic pulmonary fibrosis32.8MUC5B, TERC
2interstitial lung disease31.6SFTPD
3pulmonary fibrosis, familial31.3TERT, TERC
4pulmonary alveolar proteinosis31.1SFTPD, SFTPC
5chronic obstructive pulmonary disease31.0MUC5B, SFTPD
6dyskeratosis congenita30.9TERT, TERC
7lung cancer30.8MMP7, SFTPC, TERC, GRP, TERT, PDGFB
8bronchitis30.8MUC5B
9pulmonary emphysema30.8SFTPD, ELANE
10bronchiolitis30.8CCL3
11extrinsic allergic alveolitis30.7CCL3, SFTPD
12bronchiolitis obliterans30.4PDGFB, CXCR3
13adult respiratory distress syndrome30.3ELANE, SFTPD, SFTPC
14contact dermatitis30.3CXCR3, CCR4
15pulmonary eosinophilia30.3CCL3, SFTPD
16vascular disease30.3SOD3, ELANE
17pneumonia30.3SFTPD, SFTPC, SOD3, ELANE, CCL3, CCL17
18respiratory failure30.2CCL17, SFTPD, ELANE
19systemic scleroderma30.2CTGF
20sarcoidosis30.2CXCR3, PDGFB, CCR4, CCL18, CCL3, SFTPD
21arthritis30.1CXCR3, SOD3, CTGF, CCL3
22pulmonary sarcoidosis30.1CCR4, PDGFB, CCL3, SFTPD, CXCR3
23cystic fibrosis30.0SFTPC, MUC5B, SFTPD, ELANE, CCL17, CCL22
24influenza30.0SFTPD, CXCR3, CCL18
25glomerulonephritis29.9CTGF, ELANE, PDGFB
26allergic asthma29.9SFTPD, CCR4, CCL17, CCL3
27breast cancer29.9GRP, TERT, TERC, CCL3, MMP7
28dermatitis29.8CCL22, CCR4, CCL18, CCL17, CXCR3
29liver cirrhosis29.7PDGFB, MMP7, TERC, CTGF
30rheumatoid arthritis29.0ELANE, CXCR3, SOD3, SFTPD, CCL3, CCL18
31asthma28.5SFTPD, SFTPC, MUC5B, CXCR3, ELANE, CCL3
32diffuse pulmonary fibrosis10.8
33hypertension10.6
34pulmonary hypertension10.6
35usual interstitial pneumonia10.6
36hermansky-pudlak syndrome10.6
37nonspecific interstitial pneumonia10.6
38paragonimiasis10.5CCL17
39dyskeratosis congenita autosomal dominant10.5TERC, TERT
40pustulosis palmaris et plantaris10.5ELANE
41connective tissue disease10.5
42microscopic polyangiitis10.4
43acute interstitial pneumonia10.4
44asbestosis10.4
45scleroderma10.4
46pulmonary function10.4
47gastritis10.4TERC, TERT
48endotheliitis10.4
49eosinophilic pneumonia10.3CCL17, CCL22
50adenosquamous carcinoma10.3GRP, SFTPC, SFTPD

Graphical network of the top 20 diseases related to Pulmonary Fibrosis:



Diseases related to pulmonary fibrosis

Symptoms for Pulmonary Fibrosis

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Drugs & Therapeutics for Pulmonary Fibrosis

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Drug clinical trials:

Search ClinicalTrials for Pulmonary Fibrosis

Search NIH Clinical Center for Pulmonary Fibrosis

Inferred drug relations via UMLS62/NDF-RT40:

Cell-based therapeutics:


LifeMap Discovery
The database of embryonic development, stem cell research and regenerative medicine
Read about Pulmonary Fibrosis cell therapies at LifeMap Discovery.
Stem-cell-based therapeutic approaches for Pulmonary Fibrosis:
Mesenchymal stem cells for lung fibrosis
Embryonic/Adult Cultured Cells Related to Pulmonary Fibrosis:
Mouse bone marrow-derived mesenchymal stem cells (family), PMIDs: 20801416, 17569781, 18625757

Genetic Tests for Pulmonary Fibrosis

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Anatomical Context for Pulmonary Fibrosis

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MalaCards organs/tissues related to Pulmonary Fibrosis:

32
Lung, Testes, Heart, Neutrophil, Bone, Bone marrow, Monocytes, Endothelial, T cells, Smooth muscle, B cells, Skin, Thymus, Skeletal muscle, Liver, Thyroid, Breast, Myeloid, Nk cells

LifeMap Discovery
The database of embryonic development, stem cell research and regenerative medicine

Cells/anatomical compartments in embryo or adult related to Pulmonary Fibrosis:
id TissueAnatomical CompartmentCell Relevance
1 LungAlveoliAlveolar Epithelial Type 1 Cells Potential therapeutic candidate, affected by disease
2 LungAlveoliAlveolar Epithelial Type 2 Cells Potential therapeutic candidate, affected by disease

Animal Models for Pulmonary Fibrosis or affiliated genes

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MGI Mouse Phenotypes related to Pulmonary Fibrosis:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020068.6TERT, TERC, SFTPC, MMP7, CXCR3, ELANE
2MP:00053788.0TERC, SFTPD, SFTPC, MMP7, CCL3, PDGFB
3MP:00053887.9TERC, SFTPD, SFTPC, CXCR3, CCR4, PDGFB
4MP:00053767.2TERT, TERC, SFTPD, SOD3, MMP7, CXCR3
5MP:00107687.1SFTPC, SFTPD, TERC, TERT, SOD3, MMP7
6MP:00053977.0MMP7, SFTPD, TERC, TERT, CXCR3, ELANE
7MP:00053876.5PDGFB, TERT, TERC, SFTPD, SFTPC, SOD3

Publications for Pulmonary Fibrosis

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Articles related to Pulmonary Fibrosis:

(show top 50)    (show all 1331)
idTitleAuthorsYear
1
MMP28 promotes macrophage polarization toward M2 cells and augments pulmonary fibrosis. (23964118)
2014
2
Landmark approvals in idiopathic pulmonary fibrosis. (25380420)
2014
3
Hypoxia-induced Deoxycytidine Kinase Contributes to Epithelial Proliferation in Pulmonary Fibrosis. (25358054)
2014
4
Presence of a prothrombotic state in people with idiopathic pulmonary fibrosis: a population-based case-control study. (24002055)
2013
5
Echocardiographic and hemodynamic predictors of mortality in idiopathic pulmonary fibrosis. (23450321)
2013
6
Procollagen type III amino terminal propeptide concentrations in dogs with idiopathic pulmonary fibrosis compared with chronic bronchitis and eosinophilic bronchopneumopathy. (22909956)
2013
7
Transcriptional regulation of increased CCL2 expression in pulmonary fibrosis involves nuclear factor-I_B and activator protein-1. (23583295)
2013
8
Targeting sphingosine kinase 1 attenuates bleomycin-induced pulmonary fibrosis. (23315259)
2013
9
Pulmonary fibrosis in dyskeratosis congenita with TINF2 gene mutation. (24072216)
2013
10
Mutations in FAM111B cause hereditary fibrosing poikiloderma with tendon contracture, myopathy, and pulmonary fibrosis. (24268661)
2013
11
Detection of autoantibodies to periplakin and envoplakin in paraneoplastic pemphigus but not idiopathic pulmonary fibrosis using full-length recombinant proteins. (24275244)
2013
12
Evaluation of the Effects of Caffeic Acid Phenethyl Ester on Prostaglandin E2 and Two Key Cytokines Involved in Bleomycin-induced Pulmonary Fibrosis. (23997916)
2013
13
Baicalein attenuates bleomycin-induced pulmonary fibrosis in rats through inhibition of miR-21. (23523661)
2013
14
Idiopathic pulmonary fibrosis in 2011: key updates on guidelines and therapeutics. Concluding remarks. (23735046)
2013
15
Oxidative stress and pulmonary fibrosis. (23219955)
2013
16
Pulmonary hypertension as a prognostic factor in idiopathic pulmonary fibrosis. (23485885)
2013
17
Pulmonary fibrosis in sarcoidosis. Clinical features and outcomes. (23952859)
2013
18
Propylthiouracil prevents cutaneous and pulmonary fibrosis in the reactive oxygen species murine model of systemic sclerosis. (24286160)
2013
19
Mitochondrial Rac1 GTPase import and electron transfer from cytochrome c are required for pulmonary fibrosis. (22157762)
2012
20
Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. (22931326)
2012
21
Expressions of caveolin-1 and extracellular matrix in lung tissues of patients with idiopathic pulmonary fibrosis]. (22883991)
2012
22
Secondary prevention of idiopathic pulmonary fibrosis: catching the horse still in the barn. (22467803)
2012
23
Equine multinodular pulmonary fibrosis in association with asinine herpesvirus type 5 and equine herpesvirus type 5: a case report. (23009194)
2012
24
The natural history and diagnosis of idiopathic pulmonary fibrosis: are we all on the same page? (21729885)
2011
25
Safety of postoperative administration of human urinary trypsin inhibitor in lung cancer patients with idiopathic pulmonary fibrosis. (22216165)
2011
26
Serum IL-33 levels are raised in patients with systemic sclerosis: association with extent of skin sclerosis and severity of pulmonary fibrosis. (21246230)
2011
27
Intedanib, a triple kinase inhibitor of VEGFR, FGFR and PDGFR for the treatment of cancer and idiopathic pulmonary fibrosis. (20432191)
2010
28
Familial interstitial pulmonary fibrosis: a large family with atypical clinical features. (21165348)
2010
29
Surfactant protein-D predicts survival in patients with idiopathic pulmonary fibrosis. (20560296)
2009
30
Serum surfactant protein-A is a strong predictor of early mortality in idiopathic pulmonary fibrosis. (19255294)
2009
31
Increased bronchoalveolar lavage fluid CD1c expressing dendritic cells in idiopathic pulmonary fibrosis. (19556741)
2009
32
Overexpression of MMP9 in macrophages attenuates pulmonary fibrosis induced by bleomycin. (17702637)
2007
33
Methionine aminopeptidase-2 as a selective target of myofibroblasts in pulmonary fibrosis. (17446530)
2007
34
RhoA signaling modulates cyclin D1 expression in human lung fibroblasts; implications for idiopathic pulmonary fibrosis. (16776827)
2006
35
Membrane type-matrix metalloproteinases in idiopathic pulmonary fibrosis. (16933466)
2006
36
CD8+ T lymphocytes in lung tissue from patients with idiopathic pulmonary fibrosis. (16042790)
2005
37
CCR5 expression and CC chemokine levels in idiopathic pulmonary fibrosis. (15802346)
2005
38
NOD2/CARD15 gene polymorphisms in idiopathic pulmonary fibrosis. (16315780)
2005
39
Therapeutic targeting of IL-4- and IL-13-responsive cells in pulmonary fibrosis. (15531774)
2004
40
Transforming growth factor-beta1 gene polymorphisms are associated with disease progression in idiopathic pulmonary fibrosis. (12746254)
2003
41
A study of the cough reflex in idiopathic pulmonary fibrosis. (12917229)
2003
42
Involvement of gelatinases (MMP-2 and MMP-9) in the development of airway inflammation and pulmonary fibrosis. (11991086)
2002
43
Interleukin-1 gene cluster polymorphisms in sarcoidosis and idiopathic pulmonary fibrosis. (11790645)
2002
44
Overexpression of matrix metalloproteinase-7 in pulmonary fibrosis. (11893661)
2002
45
Direct measurement of IGF-I and IGFBP-3 in bronchoalveolar lavage fluid from idiopathic pulmonary fibrosis. (11817709)
2001
46
Interleukin-8 expression in bronchoalveolar lavage cells in the evaluation of alveolitis in idiopathic pulmonary fibrosis. (9616535)
1998
47
Relative release of interleukin-1 beta and interleukin-1 receptor antagonist by alveolar macrophages. A study in asbestos-induced lung disease, sarcoidosis, and idiopathic pulmonary fibrosis. (8325116)
1993
48
Expression and localization of tumor necrosis factor-alpha and its mRNA in idiopathic pulmonary fibrosis. (8362967)
1993
49
TNF in autoimmune diseases, graft-versus-host reactions, and pulmonary fibrosis. (1550870)
1992
50
Diffuse pulmonary fibrosis [abstract]. (20919054)
1968

Variations for Pulmonary Fibrosis

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Expression for genes affiliated with Pulmonary Fibrosis

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Expression patterns in normal tissues for genes affiliated with Pulmonary Fibrosis

Search GEO for disease gene expression data for Pulmonary Fibrosis.

Pathways for genes affiliated with Pulmonary Fibrosis

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Pathways related to Pulmonary Fibrosis according to GeneCards/GeneDecks:

(show all 13)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
FOXA transcription factor networks37
9.9SFTPD, SFTPA2, SFTPA1
29.9SFTPA1, SFTPA2, SFTPD
39.1SOD3, ELANE, CCL3, SFTPD
4
Show member pathways
8.0CCL3, CCL17, CCL18, CCL22, CTGF, PDGFB
5
Show member pathways
7.7CCL3, CXCR3, CCL17, CCR4, CCL22, GRP
6
Show member pathways
7.6CCL22, CCL17, CCR4, CCL18, CCL3, CXCR3
7
Show member pathways
7.6CXCR3, CCL3, CCL17, CCL18, CCR4, CCL22
8
Show member pathways
7.4CXCR3, CCR4, CCL18, CCL17, CCL3, MMP7
9
Show member pathways
7.3GRP, CCR4, CCL17, CCL3, CXCR3, PDGFB
10
Show member pathways
7.2CCR4, PDGFB, CCL22, CCL18, CCL17, CCL3
11
Show member pathways
6.8CCL17, CXCR3, CCL3, CCL18, CCR4, CTGF
12
Show member pathways
6.8CCL22, CXCR3, CCL3, CCL17, CCL18, CCR4
13
Show member pathways
6.8CCL18, CXCR3, CCL17, CCR4, CTGF, PDGFB

Compounds for genes affiliated with Pulmonary Fibrosis

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Compounds related to Pulmonary Fibrosis according to GeneCards/GeneDecks:

(show top 50)    (show all 78)
idCompoundScoreTop Affiliating Genes
1sivelestat sodium hydrate4410.3SFTPD, ELANE
2curosurf4410.3SFTPC, ELANE
3survanta4410.2ELANE, SFTPC
4sivelestat4410.2ELANE, SFTPD
5lipofectamine4410.1TERC, TERT
6allergens449.8CCL17, CXCR3, SFTPD
7cerulein449.7ELANE, GRP
8il 10449.6CCL17, CCL3, CXCR3
9peroxynitrite449.5CCL3, SOD3, SFTPC
10pioglitazone28 44 50 1112.5CTGF, MMP7, SOD3
11methacholine44 50 1111.5GRP, CCL22, CCL3
12endotoxin449.4SFTPD, CXCR3, ELANE, CCL3
13bleomycin44 1110.3SFTPC, CCL3, PDGFB, CTGF
14agar449.2TERC, PDGFB, GRP, CTGF
15etoposide44 50 61 1112.2TERT, TERC, CCL3, GRP
16fibrinogen449.2SFTPC, MMP7, ELANE, CCL17, CCL22, CTGF
17hydrocortisone44 2 61 1112.1CXCR3, ELANE, CCL3, GRP
18epinephrine44 24 1111.1GRP, CCL22, CCL3, ELANE
19tacrolimus44 50 1111.1CXCR3, CCL3, CCL17, CCL22, PDGFB
20matrigel449.0MMP7, CXCR3, CCL3, GRP
21pd 98,059449.0CTGF, PDGFB, CCL3, CXCR3, TERT
22histamine44 28 2411.0SFTPD, CXCR3, CCL3, CCL22, GRP
23oligonucleotide448.9TERT, TERC, SFTPD, MMP7, CCL22, PDGFB
24heparin44 28 24 1111.9SOD3, CXCR3, ELANE, CCL3, CTGF
25phosphatidylinositol448.9CTGF, GRP, CXCR3, MMP7, SFTPD, TERT
26h2o2448.9TERT, SOD3, MMP7, ELANE, CCL3, CTGF
27lactate448.8CTGF, GRP, CCL3, ELANE, SFTPD
28cholesterol44 28 24 1111.7SFTPD, SFTPC, SOD3, CXCR3, CCL3
29cyclosporin a44 28 6110.7ELANE, CCL3, CCL17, CCL22, PDGFB, CTGF
30cycloheximide448.7CTGF, GRP, PDGFB, CCL3, SFTPC
31pge2448.5CXCR3, ELANE, CCL3, CCL22, GRP, CTGF
32paraffin448.4TERT, TERC, MMP7, CCR4, PDGFB, GRP
33cysteine448.4TERT, SFTPD, SFTPC, SOD3, MMP7, ELANE
34ribonucleic acid448.1TERT, SFTPC, MMP7, CCL3, CCR4, PDGFB
35larc448.1CCL22, CCR4, CCL18, CCL17, CCL3, CXCR3
36thymidine44 249.1TERT, TERC, SFTPD, CXCR3, CCL3, PDGFB
37lipid448.1TERT, TERC, SFTPD, SFTPC, SOD3, CCR4
38tarc448.1CXCR3, CCL3, CCL17, CCL18, CCR4, CCL22
39ccl344 289.1CXCR3, CCL3, CCL17, CCL18, CCR4, CCL22
40ccl444 289.1CXCR3, CCL3, CCL17, CCL18, CCR4, CCL22
41rantes448.0CXCR3, CCL3, CCL17, CCL18, CCR4, CCL22
42nitric oxide44 24 1110.0TERT, SFTPD, SOD3, MMP7, CXCR3, CCL3
43estrogen447.7TERT, TERC, MMP7, CXCR3, ELANE, CCL3
44oxygen44 248.7TERT, SFTPD, SFTPC, SOD3, MMP7, CXCR3
45retinoic acid44 248.4TERT, TERC, SFTPC, MMP7, ELANE, CCL3
46serine447.4TERT, SFTPD, SOD3, MMP7, CXCR3, ELANE
47tyrosine447.2TERT, MMP7, CXCR3, CCR4, PDGFB, GRP
48vegf447.1TERT, SFTPC, SOD3, MMP7, CXCR3, CCL3
49dexamethasone44 50 28 1110.1SFTPD, SFTPC, CXCR3, CCL3, CCL17, CCL18
50calcium44 50 24 118.9MMP7, SFTPA2, SFTPC, SFTPA1, SFTPD, TERT

GO Terms for genes affiliated with Pulmonary Fibrosis

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Cellular components related to Pulmonary Fibrosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1collagenGO:0055819.9SFTPA2, SFTPD, SFTPA1
2extracellular regionGO:0055766.1CTGF, MMP7, CCL17, CCL3, ELANE, PDGFB
3extracellular spaceGO:0056156.0SFTPD, CTGF, GRP, PDGFB, CCL22, CCL18

Biological processes related to Pulmonary Fibrosis according to GeneCards/GeneDecks:

(show all 13)
idNameGO IDScoreTop Affiliating Genes
1respiratory gaseous exchangeGO:0075859.9SFTPD, SFTPA1, SFTPC, SFTPA2
2positive regulation of chemotaxisGO:0509219.8PDGFB, CXCR3
3T cell chemotaxisGO:0108189.8CCL3, CXCR3
4reactive oxygen species metabolic processGO:0725939.7SFTPD, PDGFB, CTGF
5macrophage chemotaxisGO:0482469.6SFTPD, CCL3
6positive chemotaxisGO:0509189.5PDGFB, CCL3
7monocyte chemotaxisGO:0025489.2PDGFB, CCL3
8positive regulation of ERK1 and ERK2 cascadeGO:0703749.2CCL3, PDGFB, CTGF
9cell chemotaxisGO:0603269.1PDGFB, CCL22, CCL18, CCL17
10cell-cell signalingGO:0072678.9CCL3, CCL17, CCL18, CCL22
11immune responseGO:0069558.5CCL22, CCR4, CCL18, CCL17
12chemotaxisGO:0069357.9CCL22, CCR4, CCL18, CCL17, CCL3, CXCR3
13inflammatory responseGO:0069547.9CXCR3, CCL3, CCL17, CCL18, CCR4, CCL22

Molecular functions related to Pulmonary Fibrosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1carbohydrate bindingGO:0302469.6SFTPA2, SFTPA1, SFTPD
2chemoattractant activityGO:0420569.5PDGFB, CCL3
3heparin bindingGO:0082019.4SOD3, ELANE, CTGF
4chemokine receptor activityGO:0049509.3CCR4, CXCR3
5chemokine activityGO:0080098.9CCL22, CCL18, CCL17, CCL3

Products for genes affiliated with Pulmonary Fibrosis

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Sources for Pulmonary Fibrosis

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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