MCID: PLM036
MIFTS: 66

Pulmonary Fibrosis malady

Respiratory diseases category

Summaries for Pulmonary Fibrosis

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Sources:
33MedlinePlus, 63Wikipedia, 32MalaCards
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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: Pulmonary Fibrosis, also known as post-inflammatory pulmonary fibrosis, is related to idiopathic pulmonary fibrosis and pneumonia. An important gene associated with Pulmonary Fibrosis is SFTPA2 (surfactant protein A2), and among its related pathways are PEDF Induced Signaling and PAK Pathway. The drug oxygen and the compounds calcium and vegf have been mentioned in the context of this disorder. Affiliated tissues include lung, testes and heart, and related mouse phenotypes are immune system and mortality/aging.

Wikipedia:63 Pulmonary fibrosis is the formation or development of excess fibrous connective tissue (fibrosis) in the... more...

Aliases & Classifications for Pulmonary Fibrosis

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Sources:
8Disease Ontology, 10DISEASES, 44Novoseek, 33MedlinePlus, 30LifeMap Discovery™, 60UMLS, 34MeSH, 56SNOMED-CT, 39NCIt
See all sources

Classifications:

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


Aliases & Descriptions:

pulmonary fibrosis 8 10 44 33 30 60
post-inflammatory pulmonary fibrosis 60
postinflammatory pulmonary fibrosis 8
lung: cirrhosis of 8
fibrosis of lung 8


External Ids:

Disease Ontology8 DOID:3770
MeSH34 D011658
NCIt39 C26869

Related Diseases for Pulmonary Fibrosis

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Sources:
17GeneCards, 18GeneDecks
See all sources

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 189)
idRelated DiseaseScoreTop Affiliating Genes
1idiopathic pulmonary fibrosis32.0TERC, SFTPA1
2pneumonia31.2ELANE, GRP, SOD3, SFTPD, SFTPC, CCL3
3pulmonary emphysema31.0ELANE, SFTPD
4sarcoidosis31.0CCL3, CCL18, CCR4, GRP, SFTPD, PDGFB
5pulmonary sarcoidosis31.0CCR4, CXCR3, PDGFB, SFTPD, CCL3
6pulmonary fibrosis, familial31.0TERC, TERT
7lung cancer30.9PDGFB, TERT, SFTPC, GRP, TERC, MMP7
8interstitial lung disease30.7CCL17, CCL3, SFTPC, GRP, SFTPD, PDGFB
9arthritis30.5CXCR3, SOD3, CCL3
10rheumatoid arthritis30.4CCL3, SFTPD, SOD3, CXCR3, TERT, CCL18
11pulmonary alveolar proteinosis30.4SFTPC, SFTPD
12eosinophilia30.3CCL17, CCR4
13pulmonary eosinophilia30.3SFTPD, CCL3
14cystic fibrosis30.2SFTPC, CCL22, ELANE, MUC5B, SFTPD, CCL17
15dermatomyositis30.2SFTPD, CCL3
16extrinsic allergic alveolitis30.2SFTPD, CCL3
17asthma30.1MUC5B, CCR4, CCL22, CCL17, CCL3, SFTPC
18influenza30.1SFTPD, CXCR3, CCL18
19hepatitis30.1PDGFB, HGF
20bronchiolitis obliterans30.1PDGFB, CXCR3
21bronchiolitis30.1CCL3
22breast cancer29.9TERC, TERT, CXCR3, HGF, CCL3, GRP
23squamous cell carcinoma29.9MMP7, PDGFB, HGF, TERC, GRP, TERT
24allergic asthma29.9CCR4, CCL17, CCL3, SFTPD
25liver cirrhosis29.9TERC, MMP7, PDGFB, HGF
26vascular disease29.9SOD3, PDGFB, GRP, ELANE
27adult respiratory distress syndrome29.9ELANE, SFTPD, SFTPC
28hepatitis c29.9CXCR3, MMP7
29vasculitis29.9ELANE
30myeloma29.8CCL3, HGF
31respiratory failure29.8SFTPC, SFTPD
32diffuse pulmonary fibrosis10.8
33idiopathic interstitial pneumonia10.7
34pulmonary systemic sclerosis10.7
35hypertension10.6
36hermansky-pudlak syndrome10.5
37nonspecific interstitial pneumonia10.5
38localized pulmonary fibrosis10.4
39acute interstitial pneumonia10.4
40connective tissue disease10.4
41asbestosis10.4
42scleroderma10.3
43postinflammatory pulmonary fibrosis10.2
44collagen disease10.2
45pneumothorax10.2
46combined pulmonary fibrosis-emphysema syndrome10.2
47desquamative interstitial pneumonia10.2
48dyskeratosis congenita10.2
49lymphangioleiomyomatosis10.2
50pneumoconiosis10.2

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



Diseases related to pulmonary fibrosis

Clinical Features for Pulmonary Fibrosis

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Drugs & Therapeutics for Pulmonary Fibrosis

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 30LifeMap Discovery™, 60UMLS, 40NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Pulmonary Fibrosis

Drug clinical trials:

Search ClinicalTrials for Pulmonary Fibrosis

Search NIH Clinical Center for Pulmonary Fibrosis

Search CenterWatch for Pulmonary Fibrosis

Inferred drug relations via UMLS60/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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Sources:
30LifeMap Discovery™, 32MalaCards
See all sources

MalaCards organs/tissues related to Pulmonary Fibrosis:

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

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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Sources:
36MGI
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MGI Mouse Phenotypes related to Pulmonary Fibrosis:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000538711.8ELANE, CCR4, CCL17, CCL3, SFTPC, SFTPD
2MP:001076811.5SFTPD, SFTPC, CCL3, HGF, SOD3, PDGFB
3MP:000539711.3CCR4, CCL3, SFTPD, PDGFB, TERT, TERC
4MP:000200610.8SFTPC, CXCR3, MMP7, TERT, TERC, ELANE

Publications for Pulmonary Fibrosis

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Sources:
50PubMed
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Articles related to Pulmonary Fibrosis:

(show top 50)    (show all 1501)
idTitleAuthorsYear
1
Targeting sphingosine kinase 1 attenuates bleomycin-induced pulmonary fibrosis. (23315259)
2013
2
A 2-year-old girl with co-inherited cystic fibrosis and sickle cell-I^+ thalassemia presenting with recurrent vaso-occlusive events during cystic fibrosis pulmonary exacerbations: a case report. (23890029)
2013
3
Idiopathic pulmonary fibrosis: clinically meaningful primary endpoints in phase 3 clinical trials. (23725617)
2013
4
Pathogenesis of Idiopathic Pulmonary Fibrosis. (24050627)
2013
5
Essential role of stem cell factor-c-Kit signalling pathway in bleomycin-induced pulmonary fibrosis. (23401096)
2013
6
FoxO3a (Forkhead Box O3a) deficiency protects Idiopathic Pulmonary Fibrosis (IPF) fibroblasts from type I polymerized collagen matrix-induced apoptosis via caveolin-1 (cav-1) and Fas. (23580232)
2013
7
Idiopathic pulmonary fibrosis: time to get personal? (24328772)
2013
8
Pulmonary function parameters and use of bronchodilators in patients with cystic fibrosis. (23503485)
2013
9
Endothelin-converting enzyme-1 gene ablation attenuates pulmonary fibrosis via CGRP-cAMP/EPAC1 pathway. (23306833)
2013
10
Association analysis reveals genetic variation altering bleomycin-induced pulmonary fibrosis in mice. (23239499)
2013
11
Towards a functional proteomics approach to the comprehension of idiopathic pulmonary fibrosis, sarcoidosis, systemic sclerosis and pulmonary Langerhans cell histiocytosis. (23528693)
2013
12
Grainyheadlike 2 Distribution Reveals Novel Pathophysiological Differences Between Human Idiopathic Pulmonary Fibrosis and Mouse Models of Pulmonary Fibrosis. (24375798)
2013
13
Nebulized and oral thiol derivatives for pulmonary disease in cystic fibrosis. (23852992)
2013
14
Vitamin D Deficiency Is Associated with Pulmonary Exacerbations in Children with Cystic Fibrosis. (24083951)
2013
15
Into the matrix: targeting fibroblasts in pulmonary fibrosis. (22847104)
2012
16
Allele-specific transactivation of matrix metalloproteinase 7 by FOXA2 and correlation with plasma levels in idiopathic pulmonary fibrosis. (22268124)
2012
17
Exacerbations in idiopathic pulmonary fibrosis triggered by pulmonary and nonpulmonary surgery: a case series and comprehensive review of the literature. (22543997)
2012
18
Improvement in pulmonary function following antibiotics in infants with cystic fibrosis. (22009796)
2012
19
Altered DNA methylation profile in idiopathic pulmonary fibrosis. (22700861)
2012
20
Idiopathic pulmonary fibrosis. (23001798)
2012
21
Risk of acute exacerbation of interstitial pneumonia after pulmonary resection for lung cancer in patients with idiopathic pulmonary fibrosis based on preoperative high-resolution computed tomography. (21748606)
2011
22
An alternative therapy for idiopathic pulmonary fibrosis by doxycycline through matrix metalloproteinase inhibition. (21886950)
2011
23
Lung transplantation for pulmonary fibrosis in dyskeratosis congenita: Case Report and systematic literature review. (21676225)
2011
24
An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. (21471066)
2011
25
Viruses and acute exacerbations of idiopathic pulmonary fibrosis: rest in peace? (21693713)
2011
26
FIZZ2/RELM-I^ induction and role in pulmonary fibrosis. (21602491)
2011
27
Activated MCTC mast cells infiltrate diseased lung areas in cystic fibrosis and idiopathic pulmonary fibrosis. (22014187)
2011
28
Familial pulmonary fibrosis is the strongest risk factor for idiopathic pulmonary fibrosis. (21917441)
2011
29
Elevated CC chemokine level in bronchoalveolar lavage fluid is predictive of a poor outcome of idiopathic pulmonary fibrosis. (19270434)
2009
30
Role of the chemokine receptor CXCR2 in bleomycin-induced pulmonary inflammation and fibrosis. (18836137)
2009
31
Effects of elastase inhibitor on the epithelial cell apoptosis in bleomycin-induced pulmonary fibrosis. (19995276)
2009
32
Identification of annexin 1 as a novel autoantigen in acute exacerbation of idiopathic pulmonary fibrosis. (18566442)
2008
33
The CR1 C5507G polymorphism is not involved in susceptibility to idiopathic pulmonary fibrosis in two European populations. (18937792)
2008
34
Comparative expression profiling in pulmonary fibrosis suggests a role of hypoxia-inducible factor-1alpha in disease pathogenesis. (17761615)
2007
35
Myeloperoxidase promoter polymorphism -463G is associated with more severe clinical expression of cystic fibrosis pulmonary disease. (16883063)
2006
36
Inhibition of leukocyte elastase, polymorphonuclear chemoinvasion, and inflammation-triggered pulmonary fibrosis by a 4-alkyliden-beta-lactam with a galloyl moiety. (16249367)
2006
37
Effects of interferon-gamma 1b on biomarker expression in patients with idiopathic pulmonary fibrosis. (15044205)
2004
38
Interleukin-7 and transforming growth factor-beta play counter-regulatory roles in protein kinase C-delta-dependent control of fibroblast collagen synthesis in pulmonary fibrosis. (15133032)
2004
39
Involvement of mast cell chymase in bleomycin-induced pulmonary fibrosis in mice. (14575803)
2003
40
Asbestosis and idiopathic pulmonary fibrosis: comparison of thin-section CT features. (14576443)
2003
41
Inhibition of bleomycin-induced pulmonary fibrosis in mice by the matrix metalloproteinase inhibitor batimastat. (11276015)
2001
42
Upregulation of Fas-signalling molecules in lung epithelial cells from patients with idiopathic pulmonary fibrosis. (11334117)
2001
43
Respiratory disease in cystic fibrosis: from physiopathology to therapy. Kinesitherapy and pulmonary transplantation excluded]. (10549060)
1999
44
The CXC chemokines, IL-8 and IP-10, regulate angiogenic activity in idiopathic pulmonary fibrosis. (9233641)
1997
45
Increased procoagulant and antifibrinolytic activities in the lungs with idiopathic pulmonary fibrosis. (7624837)
1995
46
In vitro production of B cell growth factor and B cell differentiation factor by peripheral blood mononuclear cells and bronchoalveolar lavage T lymphocytes from patients with idiopathic pulmonary fibrosis. (2208788)
1990
47
Acquired tracheomegaly in adults as a complication of diffuse pulmonary fibrosis. (2784256)
1989
48
Enzyme histochemical observations of diffuse pulmonary fibrosis of rats experimentally induced by ionizing irradiation. (1274581)
1976
49
Diffuse pulmonary fibrosis [abstract]. (20919054)
1968
50
THE REGULATION OF VENTILATION IN DIFFUSE PULMONARY FIBROSIS. (14256716)
1965

Genetic Variations for Pulmonary Fibrosis

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Expression for genes affiliated with Pulmonary Fibrosis

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Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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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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Sources:
51QIAGEN, 29KEGG, 53Reactome, 37NCBI BioSystems Database, 12EMD Millipore
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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
Hide members
11.3HGF, CCL22, CCR4, CCL18, CCL17, CCL3
2
Hide members
11.3HGF, CXCR3, PDGFB, CCL3, CCL17, CCL18
3
Hide members
11.3PDGFB, CCL3, CCL17, CCL18, CCR4, CCL22
4
Hide members
11.3HGF, CCL22, CCR4, CCL18, CCL17, CCL3
5
Hide members
11.2CCL22, CCR4, CCL18, CCL17, CCL3, CXCR3
6
Hide members
11.1HGF, CCL22, CCL18, CCL17, CCL3, PDGFB
7
Hide members
11.1CCL22, CCR4, CCL17, CCL3, GRP, CXCR3
8
Hide members
11.1CCL22, CCR4, CCL18, CCL17, CCL3, CXCR3
911.0CXCR3, CCL3, CCL17, CCR4, CCL22
1010.9CCL3, SFTPD, SOD3, ELANE
1110.8SFTPA1, SFTPA2, SFTPD
1210.8SFTPD, SFTPA2, SFTPA1
1310.7MMP7, HGF

Compounds for genes affiliated with Pulmonary Fibrosis

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

(show top 50)    (show all 69)
idCompoundScoreTop Affiliating Genes
1calcium44 49 11 2415.4GRP, SFTPA2, CCL18, CCL3, CCL17, CCR4
2vegf4412.0SOD3, CCL3, TERT, MMP7, CCL18, CXCR3
3retinoic acid44 2412.9SFTPC, CCL3, ELANE, TERC, TERT, MMP7
4thymidine44 2412.8TERC, TERT, CXCR3, GRP, PDGFB, SFTPD
5nitric oxide44 11 2413.7TERT, SFTPD, CCL3, CXCR3, SOD3, PDGFB
6lipid4411.7PDGFB, HGF, CCR4, SFTPC, SFTPD, SOD3
7ccl444 2812.7HGF, CXCR3, CCL3, CCL17, CCL18, CCL22
8dexamethasone44 49 28 1114.7CCL17, HGF, CCL18, SFTPC, SFTPD, PDGFB
9oxygen44 2412.7HGF, CCL3, TERT, SOD3, SFTPC, SFTPD
10paraffin4411.6HGF, TERC, MMP7, GRP, PDGFB, TERT
11larc4411.6CCR4, CCL18, CCL17, CCL3, CXCR3, CCL22
12tarc4411.6CCL3, CXCR3, CCL18, CCL22, CCL17, CCR4
13ccl344 2812.6CCL17, CCL18, CCL22, CCR4, CCL3, CXCR3
14cysteine4411.5ELANE, TERT, MMP7, SOD3, SFTPC, CCL3
15tacrolimus44 49 1113.5HGF, CCL17, CCL3, CXCR3, PDGFB, CCL22
16ribonucleic acid4411.5CCR4, CCL3, TERT, SFTPC, MMP7, PDGFB
17histamine44 28 2413.5CXCR3, CCL3, HGF, SFTPD, GRP, CCL22
18cyclosporin a44 28 5913.5PDGFB, HGF, CCL17, CCL3, ELANE, CCL22
19h2o24411.5MMP7, SOD3, CCL3, HGF, ELANE, TERT
20oligonucleotide4411.4CCL22, TERC, SFTPD, PDGFB, MMP7, TERT
21matrigel4411.4HGF, CCL3, MMP7, CXCR3, GRP
22etoposide44 49 59 1114.4HGF, CCL3, GRP, TERC, TERT
23fibrinogen4411.4CCL22, CCL17, SFTPC, MMP7, ELANE
24pd 98,0594411.3PDGFB, HGF, CCL3, CXCR3, TERT
25wortmannin4411.3TERT, HGF, CCL3, GRP, CXCR3
26doxorubicin44 49 1113.3TERT, TERC, MMP7, GRP, HGF
27arginine4411.3GRP, SOD3, TERT, CXCR3, SFTPC, HGF
28serine4411.3SOD3, CCL3, CXCR3, PDGFB
29bleomycin44 1112.3SFTPC, PDGFB, HGF, CCL3
30sodium nitroprusside4411.3ELANE, HGF, SOD3, CXCR3
31phosphatidylinositol4411.3HGF, MMP7, SFTPD, CXCR3, GRP, TERT
32prednisolone44 28 1113.3HGF, SFTPD, CCR4, CCL3
33heparan sulfate44 2412.2HGF, SOD3, CXCR3, CCL3
34lactate4411.1SFTPD, ELANE, CCL3, GRP, HGF
35rantes4411.1CCL3, CXCR3
36hydrocortisone44 2 59 1114.1ELANE, GRP
37sivelestat sodium hydrate4411.0SFTPD, ELANE
38il 104411.0CCL3, CCL17, CXCR3
39curosurf4411.0SFTPC, ELANE
40pge24411.0GRP
41methacholine44 1112.0GRP, CCL3, CCL22
42sivelestat4410.9ELANE, SFTPD
43egcg4410.9ELANE
44survanta4410.9SFTPC, ELANE
45aspirin44 49 28 2413.9HGF, CCL22, GRP, ELANE
46dextran sulfate4410.9HGF, MMP7, ELANE
47endotoxin4410.9CXCR3
485fluorouracil4410.8HGF, CCL3, TERC, TERT
49cerulein4410.7ELANE, GRP
50agar4410.6PDGFB, HGF, GRP, TERC

GO Terms for genes affiliated with Pulmonary Fibrosis

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Sources:
16Gene Ontology
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Cellular components related to Pulmonary Fibrosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:00561511.8MMP7, HGF, CCL22, CCL18, CCL17, CCL3
2extracellular regionGO:00557611.5SFTPA1, CCL3, CCL17, CCL22, HGF, SFTPA2
3collagenGO:00558110.8SFTPD, SFTPA2, SFTPA1

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

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1chemotaxisGO:00693511.3CXCR3, CCL3, CCL17, CCL18, CCR4, CCL22
2inflammatory responseGO:00695411.3CXCR3, CCL3, CCL17, CCL18, CCR4, CCL22
3respiratory gaseous exchangeGO:00758511.1SFTPA1, SFTPA2, SFTPC, SFTPD
4positive chemotaxisGO:05091810.9HGF, CCL3, PDGFB
5cell-cell signalingGO:00726710.9CCL22, CCL18, CCL17, CCL3
6T cell chemotaxisGO:01081810.8CXCR3, CCL3
7positive regulation of cell migrationGO:03033510.8PDGFB, CCL3, HGF
8positive regulation of DNA biosynthetic processGO:200057310.8PDGFB, HGF
9macrophage chemotaxisGO:04824610.6SFTPD, CCL3
10monocyte chemotaxisGO:00254810.4PDGFB, CCL3

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

idNameGO IDScoreTop Affiliating Genes
1chemoattractant activityGO:04205610.8PDGFB, CCL3, HGF
2chemokine activityGO:00800910.6CCL3, CCL17, CCL18, CCL22

Products for genes affiliated with Pulmonary Fibrosis

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

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
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet