Idiopathic Pulmonary Fibrosis malady

Disease Ontology: A idiopathic interstitial pneumonia which is a distinctive type of chronic fibrosing interstitial pneumonia with thick scarring in the lung creating a honeycomb appearance. the main symptoms start insidiously as shortness of breath on exertion, cough, and diminished stamina. other common complaints include weight loss and fatigue. the level of oxygen in the blood decreases, and the skin may take on a bluish tinge (called cyanosis) and the ends of the fingers may become thick or club-shape. in most people, symptoms worsen over a period ranging from about 6 months to several years.⁶

MalaCards: Idiopathic Pulmonary Fibrosis, also known as familial idiopathic pulmonary fibrosis, is related to acute interstitial pneumonia and interstitial lung disease. An important gene associated with Idiopathic Pulmonary Fibrosis is ELMOD2 (ELMO/CED-12 domain containing 2), and among its related pathways are Breast Cancer Regulation by Stathmin1 and Actin-Based Motility by Rho Family GTPases. The drug oxygen and the compounds glucose and ionomycin have been mentioned in the context of this disorder. Affiliated tissues include thymus, brain and smooth muscle, and related mouse phenotypes are respiratory system and embryogenesis.

NIH Rare Diseases: Idiopathic pulmonary fibrosis is a condition in which tissue deep in the lungs becomes thick and stiff, or scarred, over time. As a result the lungs lose their ability to move oxygen to the brain and throughout the body.  Common symptoms include shortness of breath, and dry, hacking cough. “Idiopathic” refers to cases that’s cause is unknown.³⁰

Genetics Home Reference: Idiopathic pulmonary fibrosis is a chronic, progressive lung disease. This condition causes scar tissue (fibrosis) to build up in the lungs, which makes the lungs unable to transport oxygen into the bloodstream effectively. The disease usually affects people between the ages of 50 and 70.¹⁷

Wikipedia: Idiopathic pulmonary fibrosis (IPF) (or cryptogenic fibrosing alveolitis (CFA) or idiopathic fibrosing...⁴⁴ more...

OMIM: 178500

Aliases & Descriptions:

idiopathic pulmonary fibrosis 6 30 17 8 familial idiopathic pulmonary fibrosis 30 43 cryptogenic fibrosing alveolitis 6 17 fibrocystic pulmonary dysplasia 6 30 hamman-rich syndrome 30 43 idiopathic pulmonary fibrosis, familial 6 fibrosing alveolitis, cryptogenic 30 usual interstitial pneumonitis 43

pulmonary fibrosis, idiopathic 33 pulmonary fibrosis,idiopathic 33 fibrosis idiopathic pulmonary 32 usual interstitial pneumonia 17 fibrosing alveolitis 30 hamman-rich disease 30 pulmonary fibrosis 43 ipf 17

Diseases related to idiopathic pulmonary fibrosis by text searches and GeneDecks gene sharing:

(show top 50)    (show all 999)

id	Related Disease	Score	Top Affiliating Genes
1	acute interstitial pneumonia	33.3	SFTPC, SFTPD, SCGB1A1, S100A1, MMP7, IL13
2	interstitial lung disease	32.2	SFTPB, SFTPC, SFTPD, BMP6, GRP, IL1A
3	usual interstitial pneumonia	32.1	SFTPC, IL13, IL8, CCL2, IFNG, TGFB1
4	pulmonary sarcoidosis	31.6	SFTPD, IL12B, IL18, IL2, IL2RA, IL2RB
5	nonspecific interstitial pneumonia	30.9	SFTPC, IL13, IL18, IL6, IL8, CCL2
6	rheumatoid arthritis	30.5	IL1R1, IL1RN, HLA-DRB1, CRP, LTA
7	idiopathic interstitial pneumonia	30.3	SFTPC, SFTPD, VEGFA, SCGB1A1, GREM1, IL13
8	chronic obstructive pulmonary disease	30.1	SFTPB, SFTPD, SERPINA1, SERPINB1, SCGB1A1, MPO
9	respiratory failure	29.8	SFTPB, SFTPD, SCGB1A1, IL13, CCL17, ALOX5
10	arthritis	29.8	SERPINA1, IL1A, IL1B, IL6, IL8, TNF
11	diffuse panbronchiolitis	29.6	MPO, IL13, IL1B, IL1RAPL2, IL1RN, IL8
12	panbronchiolitis	29.6	MPO, IL13, IL1B, IL1RAPL2, IL1RN, IL8
13	pulmonary hypertension	29.5	SFTPB, SFTPC, BMP4, FLT1, CAV1, RHOA
14	lymphangioleiomyomatosis	28.5	VIM, VEGFA, S100A1, MMP1, CCL2, IFNG
15	alpha 1-antitrypsin deficiency	27.8	SFTPB, SERPINA1, SERPINB1, VEGFA, BMP6, MPO
16	asbestosis	27.8	SERPINA1, VIM, MPO, IL6, TNF, TIMP1
17	fibrosis	27.8	NCF1, NCF2, SFTPA1, SFTPA2, SFTPB, SFTPC
18	pulmonary fibrosis	27.8	NCF1, NCF2, SFTPA1, SFTPA2, SFTPB, SFTPC
19	pulmonary emphysema	27.6	SFTPD, SERPINA1, SERPINB1, VEGFA, CDKN2A, MMP1
20	bronchiectasis	27.4	SERPINA1, SERPINB1, MPO, CD36, MMP1, MMP9
21	polymyositis	26.8	SFTPD, SELE, VEGFA, CD28, MICA, IL1A
22	collagen disease	26.6	SFTPD, VEGFA, CD36, IL10, IL2RA, IL6
23	type 2 diabetes mellitus	26.5	SELE, IL6, MAPK14, CCL2, IGFBP3, IGF1
24	eosinophilia	26.5	SFTPA1, SFTPA2, SFTPD, SERPINA1, MPO, IL10
25	rheumatoid arthritis, association with	26.5	SELE, VIM, S100A1, MMP9, MIF, IL10
26	immunodeficiency	26.1	BMP6, CFLAR, CD28, CDKN3, MIF, FCGR3B
27	pulmonary function	26.1	SFTPB, SERPINA1, MPO, CD36, IL10, IL13
28	sarcoidosis	25.8	SFTPD, SELE, VIM, VEGFA, CLEC12A, KDR
29	histiocytosis	25.6	SERPINA1, VIM, CLEC12A, S100A1, CFLAR, CD207
30	squamous cell carcinoma	24.7	SFTPB, SERPINB3, VIM, VEGFA, MYCL1, BMP6
31	pneumonia	24.2	SFTPA1, SFTPB, SFTPC, SFTPD, SELE, SERPINA1
32	lung carcinoma	23.9	SFTPB, SFTPD, SERPINB3, SERPINA1, VEGFA, MYCL1
33	gestational diabetes	23.2	SELE, SERPINA1, CDKN2A, MICA, MIF, IL10
34	emphysema	23.0	SFTPB, SFTPC, SFTPD, SERPINA1, SERPINB1, VEGFA
35	pulmonary disease	21.0	NCF2, SFTPA1, SFTPB, SFTPC, SFTPD, SELE
36	copd	20.4	SFTPB, SFTPD, SERPINA1, SERPINB1, VEGFA, BMP6
37	carcinoma	18.9	NCF2, SFTPA1, SFTPB, SFTPC, SFTPD, SELE
38	diabetes mellitus	18.1	SELE, SERPINA1, VEGFA, BMP4, BMP6, XBP1
39	obesity	16.9	SFTPD, SELE, VEGFA, BMP4, BMP6, XBP1
40	pancreatitis	16.9	NCF2, SELE, SERPINA1, SEPP1, SERPINB1, VIM
41	hypoxia	16.7	SFTPA1, SFTPB, SFTPC, SELE, SERPINA1, VEGFA
42	hypertension	15.6	SFTPB, SFTPC, SELE, VEGFA, BMP4, BMP6
43	extrinsic allergic alveolitis	14.1	SFTPD, IL18, IL1A, IL8, CCL3, CXCL5
44	secondary syphilis	14.0	BMP4, CD36, IL11, CCL3, IGFBP5, SPP1
45	idiopathic myopathy	13.9	IL1A, IL1R1, CCL2, HLA-B, AARS
46	refractive error	13.9	MMP1, MMP7, MMP9, ACTC1, TIMP1, TIMP3
47	intermediate uveitis	13.8	IL2RA, CCR4, CCR5, CXCR3
48	cavernous hemangioma of face	13.8	XBP1, TP63, KRT18, KRT19, ACTC1, TGFA
49	burkitt's lymphoma	13.8	BMP6, CFLAR, MPP1, IL10RA, IL4, CCL3
50	pulmonary eosinophilia	13.8	SFTPD, SERPINA1, IL13, IL4, IL8, CCL17

Clinical features from OMIM: 178500

Approved drugs:

Drug clinical trials:

Inferred drug relations via UMLS/NDF-RT:

^43 28 oxygen

Cell-based therapeutics:

	Placenta-derived mesenchymal stem cells, PMIDs: 20801416, 15891110, 12815096
	Mesenchymal stem cell, PMIDs: 20801416, 15891110, 12815096

MalaCards organs/tissues related to idiopathic pulmonary fibrosis:

²²

MGI Mouse Phenotypes related to idiopathic pulmonary fibrosis:

²⁵ (show all 27)

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	respiratory system phenotype	MP:0005388	11.1	SFTPC, SFTPD, SERPINB1, SCGB1A1, GSN, GREM1
2	embryogenesis phenotype	MP:0005380	10.5	VIM, XBP1, CFLAR, IL6ST, TP63, TERC
3	craniofacial phenotype	MP:0005382	10.4	BMP4, ATF4, MMP16, GREM1, IL12B, MAPK8
4	renal/urinary system phenotype	MP:0005367	10.4	BMP4, SCGB1A1, LAMC2, GREM1, CAV1, TP63
5	hematopoietic system phenotype	MP:0005397	9.9	SERPINB1, BMP4, XBP1, CFLAR, ATF4, CD28
6	endocrine/exocrine gland phenotype	MP:0005379	9.9	GSN, IL2, IL2RB, AQP5, IFNG, CAV1
7	no phenotypic analysis	MP:0003012	9.6	SFTPC, VEGFA, BMP4, ATF6, CD207, IL13
8	limbs/digits/tail phenotype	MP:0005371	9.6	SEPP1, VEGFA, BMP4, LAMC2, MMP16, MMP9
9	integument phenotype	MP:0010771	9.0	LAMC2, XBP1, ATF4, MMP24, FHIT, GSN
10	other phenotype	MP:0005395	8.8	KDR, LAMC2, CDKN2A, FN1, IL13, IL2
11	nervous system phenotype	MP:0003631	8.7	BMP4, XBP1, CD28, CD36, MMP24, FLT1
12	digestive/alimentary phenotype	MP:0005381	8.4	VEGFA, BMP4, LAMC2, ATF4, CD36, CDKN2A
13	immune system phenotype	MP:0005387	8.3	SERPINB1, VIM, KDR, LAMC2, CFLAR, MPP1
14	adipose tissue phenotype	MP:0005375	8.2	ATF4, CD36, IL18, IL1R1, IL6, ANXA1
15	liver/biliary system phenotype	MP:0005370	8.2	SEPP1, VEGFA, BMP4, XBP1, ATF6, ATF4
16	normal phenotype	MP:0002873	7.8	SELE, VEGFA, MYCL1, BMP4, KDR, SCGB1A1
17	reproductive system phenotype	MP:0005389	7.7	SFTPC, SEPP1, VEGFA, ATF4, MMP9, FN1
18	mortality/aging	MP:0010768	7.6	SFTPB, SFTPC, SFTPD, SELE, SEPP1, SERPINB1
19	behavior/neurological phenotype	MP:0005386	7.5	SFTPB, SFTPC, SELE, SEPP1, VIM, BMP4
20	skeleton phenotype	MP:0005390	7.5	VEGFA, BMP4, BMP6, KDR, LAMC2, ATF4
21	growth/size phenotype	MP:0005378	7.3	SFTPC, SFTPD, SELE, SEPP1, BMP4, BMP6
22	vision/eye phenotype	MP:0005391	6.8	SFTPC, SELE, VIM, VEGFA, BMP4, KDR
23	tumorigenesis	MP:0002006	5.7	SFTPC, SCGB1A1, CDKN2A, MMP7, MMP9, FN1
24	muscle phenotype	MP:0005369	4.9	VIM, VEGFA, BMP4, KDR, S100A1, XBP1
25	homeostasis/metabolism phenotype	MP:0005376	4.0	SFTPB, SFTPD, SELE, SEPP1, SERPINB1, VIM
26	cellular phenotype	MP:0005384	3.6	SELE, SEPP1, SERPINB1, VIM, VEGFA, BMP4
27	cardiovascular system phenotype	MP:0005385	2.8	SFTPC, SELE, VIM, VEGFA, BMP4, KDR

Articles related to idiopathic pulmonary fibrosis:

(show top 50)    (show all 206)

id	Title	Authors	Year	Affiliating Genes
1	Analysis of macrophage migration inhibitory factor (M IF) in patients with idiopathic pulmonary fibrosis. (19464392)	Bargagli E.... Rottoli P.	2009	MIF
2	Different activity of the biological axis VEGF-Flt-1 (fms-like tyrosine kinase 1) and CXC chemokines between pulmonary sarcoidosis a nd idiopathic pulmonary fibrosis: a bronchoalveolar lavage study. (20169144)	Antoniou K.M.... Siafakas N.M.	2009	FLT1, CXCR4, VEGFA
3	WNT1-inducible signaling protein-1 mediates pulmonary fibrosis in mice and is upregulated in humans with idiopathic pulmonary fibrosis. (19287097)	KAPnigshoff M.... Eickelberg O.	2009	WISP1
4	The role of urokinase in idiopathic pulmonary fibrosis and implication for therapy. (18491991)	Gharaee-Kermani M.... Gyetko M.R.	2008	PLAU
5	Increased expression of NADPH oxidase p47-PHOX and p 67-PHOX factor in idiopathic pulmonary fibrosis (17651608)	Wang C.L.... Li Z.H.	2007	NCF2, CYCS, NCF1
6	Telomerase mutations in families with idiopathic pulmonary fibrosis. (17392301)	Armanios M.Y.... Loyd J.E.	2007	TERT, TERC
7	Impairment of alveolar macrophage transcription in idiopathic pulmonary fibrosis. (17332483)	Ren P.... Gochuico B.R.	2007	GTF2H2
8	Membrane type-matrix metalloproteinases in idiopathic pulmonary fibrosis. (16933466)	Garcia-Alvarez J.... Pardo A.	2006	MAPK14, MMP16, MMP24
9	Caveolin-1: a critical regulator of lung fibrosis in idiopathic pulmonary fibrosis. (17178917)	Wang X.M.... Choi A.M.	2006	CAV1
10	ELMOD2 is a candidate gene for familial idiopathic pulmonary fibrosis. (16773575)	Hodgson U.... Laitinen T.	2006	ELMOD2, LOC152586
11	Discoidin domain receptor 1 contributes to the survival of lung fibroblast in idiopathic pulmonary fibrosis. (16507902)	Matsuyama W.... Arimura K.	2006	DDR1
12	Extravascular sources of lung angiotensin peptide synthesis in idiopathic pulmonary fibrosis. (16844946)	Li X.... Uhal B.D.	2006	AGT
13	Bone morphogenetic protein-4 inhibitor gremlin is overexpressed in idiopathic pulmonary fibrosis. (16816361)	Koli K.... Keski-Oja J.	2006	TGFB1, BMP4, GREM1
14	Induction of epithelial-mesenchymal transition in alveolar epithelial cells by transforming growth factor-beta1: potential role in idiopathic pulmonary fibrosis. (15855634)	Willis B.C.... Borok Z.	2005	AQP5
15	Inflammatory activity is still present in the advance d stages of idiopathic pulmonary fibrosis. (16268914)	Mura M.... Fabbri M.	2005	CRP
16	A comparative study of the role of erythropoietin in the pathogenesis of deficient erythropoiesis in idiopathic pulmonary fibrosis as opposed to chronic obstructive pulmonary disease. (15795697)	Tsantes A.... Vaiopoulos G.	2005	EPO
17	Treatment of idiopathic pulmonary fibrosis: is there anything new? (15955138)	Abdelaziz M.M.... Hamad M.M.	2005	IFNG
18	Increased serum levels of endostatin in patients with idiopathic pulmonary fibrosis. (16025479)	Sumi M.... Sekizawa K.	2005	COL18A1
19	Cultured alveolar macrophages from patients with idiopathic pulmonary fibrosis (IPF) show dysregulation of lipopolysaccharide-induced tumor necrosis factor-alpha (TNF-alpha) and interleukin-10 (IL-10) inductions. (15809201)	Freeburn R.W.... Millar A.B.	2005	IL10RA
20	Effects of interferon-gamma 1b on biomarker expression in patients with idiopathic pulmonary fibrosis. (15044205)	Strieter R.M.... Valentine V.G.	2004	CXCL11
21	Change of prostaglandin E2 and interleukin-12, interleukin-13 in the bronchoalveolar lavage fluid and the serum of the patients with idiopathic pulmonary fibrosis (15256084)	Li J.Z.... Yu R.J.	2004	IL13
22	Interferon-gamma toxicity in idiopathic pulmonary fibrosis. (14739137)	O'Connor T.M.... Bredin C.P.	2004	IFNG
23	Transforming growth factor-beta1 gene polymorphisms are associated with disease progression in idiopathic pulmonary fibrosis. (12746254)	Xaubet A.... Picado C.	2003	TGFB1
24	Fibroblasts isolated from normal lungs and those with idiopathic pulmonary fibrosis differ in interleukin-6/gp130-mediated cell signaling and proliferation. (12819039)	Moodley Y.P.... Knight D.A.	2003	IL6, IL6ST, IL11
25	Systemic immune cell activation in a subgroup of patients with idiopathic pulmonary fibrosis. (12915745)	HOMOLKA J.... MA1ller-Quernheim J.	2003	IL2RB
26	The increase in serum soluble ST2 protein upon acute exacerbation of idiopathic pulmonary fibrosis. (14555548)	Tajima S.... Sugiyama Y.	2003	IL1RL1
27	Complement receptor 1 gene polymorphisms are associated with idiopathic pulmonary fibrosis. (12773320)	Zorzetto M.... Luisetti M.	2003	CR1
28	Surfactant protein A and B genetic variants predispose to idiopathic pulmonary fibrosis. (13680361)	Selman M.... Floros J.	2003	SFTPD, SFTPA1, SFTPB
29	A dark side of interferon-gamma in the treatment of idiopathic pulmonary fibrosis? (12663333)	Selman M.	2003	IFNG
30	Fluorescent in situ hybridization (FISH) of the FHIT gene in idiopathic pulmonary fibrosis (IPF) lesions. (12928708)	Uematsu K.... Kudoh S.	2003	FHIT
31	Does interferon-gamma improve pulmonary function in idiopathic pulmonary fibrosis? (14680077)	Prasse A.... Virchow J.C.	2003	IFNG
32	Interleukin-1 gene cluster polymorphisms in sarcoidosis and idiopathic pulmonary fibrosis. (11790645)	Hutyrova B.... Petrek M.	2002	IL1B, IL1R1, IL1A
33	MYCL1, FHIT, SPARC, p16(INK4) and TP53 genes associated to lung cancer in idiopathic pulmonary fibrosis. (12169206)	Demopoulos K.... Spandidos D.A.	2002	TP53, CDKN2A, SPARC
34	Analysis of tumor necrosis factor-alpha, lymphotoxin-alpha, tumor necrosis factor receptor II, and interleukin-6 polymorphisms in patients with idiopathic pulmonary fibrosis. (11371414)	Pantelidis P.... du Bois R.M.	2001	TNF, IL6, TNFRSF1B
35	Regulation of the effects of TGF-beta 1 by activation of latent TGF-beta 1 and differential expression of TGF-beta receptors (T beta R-I and T beta R-II) in idiopathic pulmonary fibrosis. (11713352)	Khalil N.... Gold L.I.	2001	TGFB1, LTBP1, MSBP1
36	Involvement of Epstein-Barr virus latent membrane protein 1 in disease progression in patients with idiopathic pulmonary fibrosis. (11050267)	Tsukamoto K.... Miura K.	2000	MPP1
37	The number of interleukin 1 receptors on lung fibroblasts in patients with idiopathic pulmonary fibrosis. (10364740)	Takeuchi M.... Izumi T.	1999	IL1R1, IL1RAPL2
38	Elevated expression of endothelin-1 and endothelin-converting enzyme-1 in idiopathic pulmonary fibrosis: possible involvement of proinflammatory cytokines. (9032126)	Saleh D.... Giaid A.	1997	ECE1, EDN1
39	Interleukin-8 in bronchoalveolar lavage fluid of pati ents with diffuse panbronchiolitis or idiopathic pulmonary fibrosis. (9395718)	Ogushi F.... Sone S.	1997	IL8
40	Hepatocyte growth factor and idiopathic pulmonary fibrosis. (8625709)	Fujita J.... Yamadori I.	1996	HGF
41	Constitutive activation of 5-lipoxygenase in the lungs of patients with idiopathic pulmonary fibrosis. (8621765)	Wilborn J.... Peters-Golden M.	1996	ALOX5
42	Progastrin-releasing peptide(31-98) in idiopathic pulmonary fibrosis and sarcoidosis. (8970357)	Shijubo N.... Abe S.	1996	GRP
43	Increased interleukin-1 receptor antagonist in idiopathic pulmonary fibrosis. A compartmental analysis. (7767546)	Smith D.R.... Hampton J.N.	1995	IL1R1, IL1RN
44	Cytoskeletal protein modulation in pulmonary alveolar myofibroblasts during idiopathic pulmonary fibrosis. Possible role of transforming growth factor beta and tumor necrosis factor alpha. (8520791)	Kapanci Y.... Gabbiani G.	1995	TGFB1, DES
45	Expression and localization of tumor necrosis factor-alpha and its mRNA in idiopathic pulmonary fibrosis. (8362967)	Piguet P.F.... Kapanci Y.	1993	TNF
46	Quantitative evaluation of gene expression of IL-1 beta and IL-1 receptor antagonist (IL-1ra) from alveolar macrophages in idiopathic pulmonary fibrosis (IPF) and sarcoidosis (8277611)	Shimoji T.... Izumi T.	1993	IL1B, IL1RN, IL1RAPL2
47	Enhanced IL-1 beta and tumor necrosis factor-alpha release and messenger RNA expression in macrophages from idiopathic pulmonary fibrosis or after asbestos exposure. (8473757)	Zhang Y.... Rom W.N.	1993	TNF, IL1B
48	Neutrophil chemotactic factors in the respiratory tract of patients with chronic airway diseases or idiopathic pulmonary fibrosis. (1309969)	Ozaki T.... Ogura T.	1992	IL8
49	Expression of monocyte chemoattractant protein 1 mRNA in human idiopathic pulmonary fibrosis. (1608944)	Antoniades H.N.... Graves D.T.	1992	CCL2
50	Enhanced expression and immunohistochemical distribution of transforming growth factor-beta in idiopathic pulmonary fibrosis. (1997280)	Khalil N.... Greenberg A.	1991	BMP6

Genes related to idiopathic pulmonary fibrosis according to GeneCards:

(show top 50)    (show all 183)

id	Symbol	Description	Score	GeneCards Section Context
1	ELMOD2	ELMO/CED-12 domain containing 2	11.1	Summaries, Publications
2	SFTPA2	surfactant protein A2	11.1	Summaries, Publications
3	SFTPA1	surfactant protein A1	11.1	Summaries, Publications
4	DKK4	dickkopf homolog 4 (Xenopus laevis)	11.0	Publications
5	PDE6G	phosphodiesterase 6G, cGMP-specific, rod, gamma	11.0	Publications
6	SRXN1	sulfiredoxin 1	11.0	Publications
7	CXCL5	chemokine (C-X-C motif) ligand 5	11.0	Publications
8	MYCL1	v-myc myelocytomatosis viral oncogene homolog 1, lung carcinoma derived (avian)	11.0	Publications
9	GTF2H2	general transcription factor IIH, polypeptide 2, 44kDa	11.0	Publications
10	TIMP4	TIMP metallopeptidase inhibitor 4	11.0	Publications
11	SFTPD	surfactant protein D	11.0	Publications
12	LOC152586	glycosyltransferase 54 domain-containing protein	11.0	Publications
13	MSBP1	minisatellite binding protein 1	11.0	Publications
14	SERPINB1	serpin peptidase inhibitor, clade B (ovalbumin), member 1	11.0	Publications
15	CD207	CD207 molecule, langerin	11.0	Publications
16	SERPINB3	serpin peptidase inhibitor, clade B (ovalbumin), member 3	11.0	Publications
17	SEPP1	selenoprotein P, plasma, 1	11.0	Publications
18	ICAM2	intercellular adhesion molecule 2	11.0	Publications
19	HGFAC	HGF activator	11.0	Publications
20	IL10RA	interleukin 10 receptor, alpha	11.0	Publications
21	GREM1	gremlin 1	11.0	Publications
22	FCGR3B	Fc fragment of IgG, low affinity IIIb, receptor (CD16b)	11.0	Publications
23	NCF1	neutrophil cytosolic factor 1	11.0	Publications
24	CCL22	chemokine (C-C motif) ligand 22	11.0	Publications
25	CCL18	chemokine (C-C motif) ligand 18 (pulmonary and activation-regulated)	11.0	Publications
26	SPINT1	serine peptidase inhibitor, Kunitz type 1	11.0	Publications
27	SUV39H1	suppressor of variegation 3-9 homolog 1 (Drosophila)	11.0	Publications
28	AQP5	aquaporin 5	11.0	Publications
29	TCF7	transcription factor 7 (T-cell specific, HMG-box)	11.0	Publications
30	PITRM1	pitrilysin metallopeptidase 1	11.0	Publications
31	PRDX1	peroxiredoxin 1	11.0	Publications
32	MMP24	matrix metallopeptidase 24 (membrane-inserted)	11.0	Publications
33	GATA6	GATA binding protein 6	11.0	Publications
34	WNT1	wingless-type MMTV integration site family, member 1	11.0	Publications
35	WISP1	WNT1 inducible signaling pathway protein 1	11.0	Publications
36	EHMT2	euchromatic histone-lysine N-methyltransferase 2	11.0	Publications
37	DKK1	dickkopf 1 homolog (Xenopus laevis)	11.0	Publications
38	AARS	alanyl-tRNA synthetase	11.0	Publications
39	ATF4	activating transcription factor 4 (tax-responsive enhancer element B67)	11.0	Publications
40	MPP1	membrane protein, palmitoylated 1, 55kDa	11.0	Publications
41	CXCL11	chemokine (C-X-C motif) ligand 11	11.0	Publications
42	CCL21	chemokine (C-C motif) ligand 21	11.0	Publications
43	CCR4	chemokine (C-C motif) receptor 4	11.0	Publications
44	CCL17	chemokine (C-C motif) ligand 17	11.0	Publications
45	ACTG2	actin, gamma 2, smooth muscle, enteric	11.0	Publications
46	SFTPC	surfactant protein C	11.0	Publications
47	SOD3	superoxide dismutase 3, extracellular	11.0	Publications
48	SPINT2	serine peptidase inhibitor, Kunitz type, 2	11.0	Publications
49	PRDX2	peroxiredoxin 2	11.0	Publications
50	XBP1	X-box binding protein 1	11.0	Publications

Pathways related to idiopathic pulmonary fibrosis according to GeneDecks:

(show top 50)    (show all 182)

id	Pathway	Score	Top Affiliating Genes
1	Breast Cancer Regulation by Stathmin136	10.7	BMP4, PDGFA, PDGFB, LTBP1, TIMP3, TIMP4
2	Actin-Based Motility by Rho Family GTPases36	10.0	BMP4, GSN, IL2, IGF1, CTGF, LTBP1
3	Ras Pathway36	10.0	BMP4, KDR, FLT1, MAPK1, DDR1, CTGF
4	Paxillin Interactions36	9.8	BMP4, KDR, FLT1, MAPK8, MAPK14, IGF1
5	RAR-Gamma-RXR-Alpha Degradation36	9.7	BMP4, GTF2H2, IL11, IL12A, IL2, MAPK14
6	CREB Pathway36	9.7	BMP4, BMP6, KDR, ATF4, IL2, IL2RA
7	Chemokine Signaling36	9.6	IL8, MAPK8, MAPK14, MAPK1, CCL22, CCL2
8	Regulation of eIF4 and p70S6K36	9.3	BMP4, IL11, IL12A, IL12B, IL13, IL18
9	ERK5 Signaling36	9.3	BMP4, BMP6, KDR, ATF4, FLT1, IL2
10	SOCS Pathway36	9.2	BMP4, BMP6, IL10, IL11, IL12A, IL12B
11	Akt Signaling36	9.1	BMP4, ATF4, CD28, IL10, IL10RA, IL11
12	Nuclear Receptor Activation by Vitamin-A36	9.1	BMP4, BMP6, KDR, ATF4, FLT1, GTF2H2
13	Pathways in cancer20	9.0	VEGFA, BMP4, MMP1, MMP9, MAPK8, MAPK1
14	African trypanosomiasis20	9.0	SELE, IL10, IL12A, IL12B, IL18, IL1B
15	PTEN Pathway36	9.0	BMP4, KDR, LAMC2, FN1, FLT1, IL2
16	14-3-3 Induced Intracellular Signaling36	8.9	BMP4, FLT1, MAPK8, MAPK14, MAPK1, IGF1
17	Telomerase Components in Cell Signaling36	8.9	BMP4, BMP6, KDR, FLT1, IL2, IGF1
18	DHA Signaling36	8.8	BMP4, BMP6, KDR, FLT1, IL1B, IL2
19	Activation of PKC through GPCR36	8.7	BMP4, BMP6, KDR, ATF4, FLT1, IL2
20	Malaria20	8.7	SELE, CD36, IL10, IL12A, IL18, IL1B
21	Nanog in Mammalian ESC Pluripotency36	8.7	BMP4, BMP6, KDR, FLT1, IL2, IL6
22	Tec Kinases Signaling36	8.6	BMP4, BMP6, CD28, IL10, IL10RA, IL11
23	Cytokine Network36	8.5	IL10, IL12A, IL12B, IL13, IL18, IL1A
24	Rheumatoid arthritis20	8.5	VEGFA, CD28, MMP1, FLT1, IL11, IL18
25	Apoptotic Pathways in Synovial Fibroblasts36	8.5	BMP4, BMP6, KDR, CFLAR, FLT1, IL2
26	mTOR Pathway36	8.4	BMP4, BMP6, IL2, IL6, MAPK8, MAPK14
27	Influenza A20	8.3	IL12A, IL12B, IL18, IL1A, IL1B, IL6
28	PEDF Induced Signaling36	8.3	BMP4, BMP6, CFLAR, IL10, IL11, IL12A
29	Phospholipase-C Pathway36	8.3	BMP4, BMP6, KDR, LAMC2, ATF4, FN1
30	MIF Mediated Glucocorticoid Regulation36	8.3	BMP4, BMP6, MIF, IL10, IL11, IL12A
31	Leishmaniasis20	8.3	NCF1, NCF2, FCGR3B, IL10, IL12A, IL12B
32	GSK3 Signaling36	8.3	BMP4, BMP6, KDR, FLT1, IL2, IL6
33	Mitochondrial Apoptosis36	8.2	BMP4, BMP6, KDR, CDKN2A, FLT1, IL2
34	GPCR Pathway36	8.2	BMP4, BMP6, KDR, ATF4, FLT1, IL2
35	Molecular Mechanisms of Cancer36	8.2	BMP4, BMP6, KDR, CFLAR, CDKN2A, FN1
36	Glucocorticoid Receptor Signaling36	8.1	BMP4, BMP6, ATF4, GTF2H2, IL10, IL10RA
37	PPAR Pathway36	8.1	BMP4, FLT1, IL2, IL2RA, IL6, MAPK14
38	Tuberculosis20	8.0	FCGR3B, IL10, IL10RA, IL12A, IL12B, IL18
39	PAK Pathway36	8.0	BMP4, BMP6, KDR, FLT1, IL10, IL11
40	Cellular Apoptosis Pathway36	8.0	BMP4, BMP6, KDR, CFLAR, CDKN2A, FLT1
41	MIF Regulation of Innate Immune Cells36	7.9	BMP4, BMP6, MIF, IL10, IL11, IL12A
42	Rho Family GTPases36	7.9	NCF2, VIM, BMP4, BMP6, KDR, LAMC2
43	Renin-Angiotensin Pathway36	7.5	BMP4, BMP6, KDR, FLT1, IL2, IL2RA
44	STAT3 Pathway36	7.5	BMP4, BMP6, CD28, IL10, IL10RA, IL11
45	JAK-STAT Pathway36	7.5	BMP4, BMP6, KDR, FLT1, IL10, IL10RA
46	Cytokine-cytokine receptor interaction20	7.4	VEGFA, KDR, FLT1, IL10, IL10RA, IL11
47	MAPK Signaling36	7.4	BMP4, BMP6, KDR, LAMC2, ATF6, ATF4
48	TGF-Beta Pathway36	7.4	BMP4, BMP6, KDR, FLT1, IL10, IL10RA
49	MAPK Family Pathway36	7.3	BMP4, BMP6, KDR, ATF4, FLT1, IL10
50	p38 Signaling36	7.2	BMP4, BMP6, KDR, CD28, FLT1, IL10

Compounds related to idiopathic pulmonary fibrosis according to GeneDecks:

(show top 50)    (show all 411)

id	Compound	Score	Top Affiliating Genes
1	glucose32	11.0	SEPP1, CLEC12A, XBP1, CFLAR, ATF4, MPP1
2	ionomycin32	10.9	GSN, GRP, ANXA1, CCL3
3	budesonide32 42 9 9	13.7	SFTPD, CD36, ANXA1, PTGS1
4	larc32	10.4	IL1A, CCL22, CCL2, CCR4, CCR5, CCL17
5	sp 60012532 42	11.2	MMP1, IL13, AKT1, TP53, CYCS, CTGF
6	hyaluronic acid32 18	11.2	VIM, BMP6, SCGB1A1, CD36, MMP1, FN1
7	aprotinin32 9 9	12.1	MMP9, FN1, IL1A, IGFBP5, IGF1, PLAU
8	ascorbic acid32 18	11.1	SFTPB, BMP4, CD36, IL1A, IL1R1, IL2RA
9	agar32	9.7	VIM, CDKN2A, FN1, FHIT, GRP, CCND1
10	formaldehyde32 18	10.5	SELE, VIM, MPO, CDKN3, MMP1, MMP9
11	estrogen32	9.4	SERPINA1, BMP4, KDR, SCGB1A1, S100A1, XBP1
12	calcium32 9 18 9	11.9	NCF2, SFTPA1, SFTPA2, SFTPB, SFTPC, SFTPD
13	ns 39832 42	9.9	MMP1, IL1A, IL1B, IL8, MAPK8, MAPK14
14	rottlerin32 42	9.9	CD36, GRP, IL8, MAPK8, MAPK14, MAPK1
15	endotoxin32	8.8	SFTPD, SCGB1A1, MIF, GSN, IL12B, IL18
16	gold sodium thiomalate32	8.7	SELE, IL1A, IL1B, IL1R1, IL1RN, IL2
17	calcitriol32 42 9 18 9	12.6	BMP6, CD36, GRP, IL11, IL13, IL2
18	tgf beta132	8.4	BMP6, KDR, CD36, MMP1, FN1, FLT1
19	oxygen32 18	9.4	NCF2, SFTPB, SFTPC, SFTPD, SERPINA1, SEPP1
20	adriamycin32	8.3	VIM, CFLAR, CD36, CDKN2A, FN1, ANXA1
21	celecoxib32 42 34 9 18 9	13.3	VEGFA, KDR, ATF4, MMP1, MMP9, IL1B
22	alpha tocopherol32	8.2	CD36, CDKN3, MMP1, MMP9, IL13, IL2RA
23	superoxide32 18	9.2	NCF2, SERPINB1, MPO, FLT1, GSN, FCGR3B
24	norepinephrine32 9 18 9	11.2	SELE, BMP6, MMP1, MMP9, MIF, GRP
25	polyinosinic-polycytidylic acid32	8.2	SELE, IL10, IL12B, IL18, IL2, IL4
26	troglitazone32 42 9 9	11.1	SELE, BMP6, CFLAR, CD36, MMP1, MMP9
27	aspirin32 34 18	10.0	SELE, BMP6, MMP1, FN1, GRP, IL13
28	ribavirin32 34 9 9	10.9	MMP1, MMP9, IL10, IL18, IL2, IL4
29	8-isoprostane32	7.9	SELE, MPO, MMP9, IL10, IL6, IL8
30	nitric oxide32 9 18 9	10.9	NCF2, SFTPB, SFTPD, SELE, SERPINA1, BMP4
31	atorvastatin32 34 9 18 9	11.8	SELE, VEGFA, BMP6, MMP1, MMP9, IL10
32	matrigel32	7.7	SELE, VIM, VEGFA, BMP6, KDR, CD36
33	cytochalasin d32 42	8.7	SELE, VIM, CD36, CDKN3, MMP1, MMP9
34	rantes32	7.7	SELE, BMP6, MMP1, MMP9, MIF, IL11
35	lipid32	7.6	SFTPB, SFTPC, SFTPD, SELE, SERPINA1, SEPP1
36	5fluorouracil32	7.6	SELE, VEGFA, KDR, CFLAR, MPO, CD36
37	adenylate32	7.4	SELE, VIM, BMP4, CDKN3, MMP9, GSN
38	neopterin32	7.4	IL10, IL18, IL1A, IL1B, IL1R1, IL1RAPL2
39	cholesterol32 9 18 9	10.3	SFTPB, SFTPC, SFTPD, SELE, SERPINA1, MSBP1
40	paraffin32	7.3	SFTPB, SERPINA1, VIM, VEGFA, KDR, S100A1
41	rosiglitazone32 9 18 9	10.2	SELE, CD36, MMP1, MMP9, IL18, IL1R1
42	curcumin32	6.9	VEGFA, MMP1, MMP9, MIF, IL18, IL1A
43	sb 20358032 42	7.7	SELE, VIM, VEGFA, BMP4, BMP6, CD36
44	prednisolone32 9 9	8.7	SFTPD, BMP6, MPO, CDKN3, MMP1, FCGR3B
45	butyrate32	6.6	NCF2, VIM, BMP6, MPO, CDKN2A, CDKN3
46	indomethacin32 9 9	8.0	VIM, BMP6, KDR, MPO, CDKN3, MMP1
47	retinoic acid32 42 18	7.6	NCF2, SFTPB, SFTPC, BMP4, BMP6, KDR
48	lactate32	5.5	SFTPD, SELE, VIM, BMP6, KDR, MPO
49	genistein32 9 18 9	7.9	SELE, VIM, VEGFA, KDR, CD28, CD36
50	actinomycin d32	4.4	SFTPB, SFTPC, SELE, VIM, VEGFA, BMP4

Cellular components related to idiopathic pulmonary fibrosis according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	proteinaceous extracellular matrix	GO:005578	9.8	SPARC, CTGF, LTBP1, TGFB1, WNT1, WNT3A
2	cell surface	GO:009986	9.4	PLAU, NOD2, PDGFA, PDGFB, ELANE, TGFA
3	integral to plasma membrane	GO:005887	9.3	ICAM2, CAV1, HLA-B, HLA-DRB1, TNF, DDR1
4	platelet alpha granule lumen	GO:031093	9.3	PDGFB, TIMP1, TGFB1, HGF, PDGFA, SPARC
5	external side of plasma membrane	GO:009897	9.1	ECE1, IL6ST, IL4, IL2RB, IL1RL1, IL13
6	plasma membrane	GO:005886	7.6	SMAD3, CR1, PLAU, NOD2, CTGF, SSTR2
7	extracellular region	GO:005576	6.7	CTGF, IGF1, IGFBP3, IGFBP5, CCL21, CCL3
8	extracellular space	GO:005615	4.9	CCL3, IL1B, IL1A, IL18, IL13, IL12B

Biological processes related to idiopathic pulmonary fibrosis according to GeneDecks:

(show top 50)    (show all 91)

id	Name	GO ID	Score	Top Affiliating Genes
1	response to hyperoxia	GO:055093	10.5	SFTPB, SFTPC, MMP9, EPO, ALOX5, NCF2
2	osteoblast differentiation	GO:001649	10.3	WNT3A, AKT1, IGFBP5, CCL3, IL18, BMP4
3	response to organic cyclic compound	GO:014070	10.3	IL1A, TIMP3, IL1RN, SERPINA1, CTGF, PTGS2
4	positive regulation of ERK1 and ERK2 cascade	GO:070374	10.3	PDGFRB, PDGFB, PDGFA, NOD2, CTGF, TNF
5	aging	GO:007568	10.2	CCL2, AGT, TOP2A, IL1B, TIMP1, TIMP3
6	positive regulation of phosphatidylinositol 3-kinase cascade	GO:014068	10.2	KDR, PDGFB, PDGFA, FLT1, HGF, PDGFRB
7	response to lipopolysaccharide	GO:032496	10.2	IL13, IL12A, SERPINA1, SELE, SFTPC, SFTPB
8	positive regulation of NF-kappaB import into nucleus	GO:042346	10.2	IL12B, TLR9, GREM1, TNF, IL1B, RHOA
9	positive regulation of T cell proliferation	GO:042102	10.1	IL6, IL12A, IL4, IL1B, IL12B, CD28
10	positive regulation of MAP kinase activity	GO:043406	10.1	TNF, ELANE, TGFB1, FLT1, VEGFA, EDN1
11	positive regulation of mitosis	GO:045840	10.1	CD28, IL1A, IL1B, IGF1, TNF, PDGFB
12	positive regulation of MAPK cascade	GO:043410	10.0	IGF1, IL6, CD36, KDR, IGFBP3, PDGFA
13	positive regulation of angiogenesis	GO:045766	10.0	VEGFA, IL1A, GATA6, DDAH1, HMOX1, IL1B
14	cell-cell signaling	GO:007267	10.0	CXCL10, CCL21, CCL18, SST, SSTR2, PDGFA
15	negative regulation of growth of symbiont in host	GO:044130	10.0	IL12B, CD36, IL10, IFNG, TNF, LTA
16	positive regulation of peptidyl-serine phosphorylation	GO:033138	10.0	AKT1, CAV1, IL6, IL11, MIF, VEGFA
17	cellular response to lipopolysaccharide	GO:071222	9.9	CD36, IL8, IL10, IL12B, TNFRSF1B, ICAM1
18	positive regulation of reactive oxygen species metabolic process	GO:2000379	9.9	PDGFRB, PDGFB, NFE2L2, TP53, MAPK14, CD36
19	positive regulation of protein phosphorylation	GO:001934	9.9	CCND1, AKT1, TNF, CTGF, TGFB1, WNT1
20	cellular response to organic cyclic compound	GO:071407	9.9	TNF, IGFBP5, CCL3, CCL2, IL1B, IL18
21	positive regulation of peptidyl-tyrosine phosphorylation	GO:050731	9.9	PDGFB, TP53, IGF1, IL6, HGF, VEGFA
22	response to ethanol	GO:045471	9.9	GSN, IL13, SPARC, IL4, MMP9, CCND1
23	calcium-mediated signaling	GO:019722	9.8	CXCR4, CXCR3, EDN1, CCL3, CCR5, IL8
24	response to glucocorticoid stimulus	GO:051384	9.8	SPARC, IL6, IL1RN, IL1B, IL10, SFTPC
25	positive regulation of nitric oxide biosynthetic process	GO:045429	9.8	TNF, HBB, EDN1, DDAH1, PTGS2, AKT1
26	chemotaxis	GO:006935	9.8	CCL2, CXCR3, IL4, MAPK14, MAPK1, CCL22
27	positive regulation of smooth muscle cell proliferation	GO:048661	9.7	PTGS2, HMOX1, IGF1, IL6, FLT1, BMP4
28	cell surface receptor signaling pathway	GO:007166	9.7	CXCL10, CCL2, IL1R1, IL2RA, CD36, AGER
29	embryo implantation	GO:007566	9.6	IL1B, SOD1, SCGB1A1, MMP9, PTGS2, SPP1
30	lipopolysaccharide-mediated signaling pathway	GO:031663	9.6	CCL3, CCL2, TNF, TGFB1, MAPK14, IL1B
31	neutrophil chemotaxis	GO:030593	9.6	EDN1, IFNG, CCL3, CCL2, IL8, IL1B
32	MAPK cascade	GO:000165	9.5	CCL2, IL18, CAV1, CCR5, CCL3, IL1B
33	cellular component movement	GO:006928	9.4	IFNG, IGF1, SPINT2, CXCR3, PTGS2, MAPK14
34	immune response	GO:006955	9.4	HLA-B, HLA-DQB1, HLA-DRB1, SMAD3, TCF7, CXCL10
35	positive regulation of interferon-gamma production	GO:032729	9.3	IL12B, IL2, IL18, LTA, TNF, IL1B
36	platelet degranulation	GO:002576	9.3	FN1, SPARC, ALB, CD36, SOD1, SERPINA1
37	virus-host interaction	GO:019048	9.2	MICA, MMP1, CFLAR, VIM, IL2RB, MAPK1
38	signal transduction	GO:007165	9.2	IL1B, IL1RL1, IL2RB, IL8, CCL22, IL13
39	response to drug	GO:042493	8.9	TOP2A, ACTC1, EDN1, LTA, PDGFB, SST
40	positive regulation of cell proliferation	GO:008284	8.8	PDGFRB, EDN1, TIMP1, TGFB1, TGFBR1, CXCL5
41	response to hypoxia	GO:001666	8.8	PDGFB, CRP, SMAD3, TNF, CAV1, CASP1
42	negative regulation of apoptotic process	GO:043066	8.7	TP53, TNF, WNT1, SMAD3, KRT18, TIMP1
43	negative regulation of cell proliferation	GO:008285	8.6	IL6, TGFB1, CDKN2A, CDKN3, IL1B, IGFBP3
44	positive regulation of transcription, DNA-dependent	GO:045893	8.5	WNT3A, EPO, TP63, WNT1, TGFBR1, IGF1
45	inflammatory response	GO:006954	8.4	AGER, CRP, SPP1, PTGS2, TNFRSF1B, TNF
46	anti-apoptosis	GO:006916	8.3	IGF1, HMOX1, PRDX2, TGFA, TERT, TNF
47	platelet activation	GO:030168	8.1	MAPK1, ALB, IGF1, MAPK14, IL6, IL11
48	apoptotic process	GO:006915	8.0	GREM1, IL1B, IL1A, IL2RA, CXCR3, CXCR4
49	blood coagulation	GO:007596	7.7	GATA6, MMP1, MAPK1, PDGFB, HGF, SOD1
50	positive regulation of transcription from RNA polymerase II promoter	GO:045944	7.6	WNT3A, WNT1, TGFB1, TLR9, TOP2A, NFE2L2

Molecular functions related to idiopathic pulmonary fibrosis according to GeneDecks:

(show all 8)

id	Name	GO ID	Score	Top Affiliating Genes
1	chemokine activity	GO:008009	10.7	CXCL9, CXCL5, CXCL11, CXCL10, CCL18, CCL21
2	collagen binding	GO:005518	10.3	PDGFB, PDGFA, SMAD3, SPARC, DDR1, FN1
3	cell surface binding	GO:043498	10.2	VEGFA, MIF, ICAM1, PDGFA, PDGFB
4	platelet-derived growth factor receptor binding	GO:005161	10.1	PDGFRB, PDGFB, PDGFA, IL1R1, VEGFA
5	protein homodimerization activity	GO:042803	8.4	SMAD3, CRP, PDGFA, PDGFB, TOP2A, TGFB1
6	growth factor activity	GO:008083	8.3	IL11, IL12A, IL12B, IL1B, IL2, IL4
7	cytokine activity	GO:005125	8.1	VEGFA, BMP4, BMP6, MIF, GREM1, IL10
8	protein binding	GO:005515	3.5	RHOA, SOD1, SOD3, WNT1, WNT3A, EHMT2