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 Glioma Invasiveness and Actin-Based Motility by Rho Family GTPases. The drug oxygen and the compounds gp 130 and acth 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	Genetic variability in the IL1RN gene and the balance between interleukin (IL)-1 receptor agonist and IL-1I^ in idiopathic pulmonary fibrosis. (22059992)	Barlo N.P.... Grutters J.C.	2011	IL1B, IL1RN
2	The role of dimethylarginine dimethylaminohydrolase i n idiopathic pulmonary fibrosis. (21677199)	Pullamsetti S.S.... Schermuly R.T.	2011	DDAH1
3	Dickkopf proteins influence lung epithelial cell prol iferation in idiopathic pulmonary fibrosis. (20650998)	Pfaff E.M.... KAPnigshoff M.	2011	DKK1, DKK4
4	Compartmentalized expression of c-FLIP in lung tissue s of patients with idiopathic pulmonary fibrosis. (19372246)	Cha S.I.... Riches D.W.	2010	CFLAR
5	Phosphodiesterase 6 subunits are expressed and altere d in idiopathic pulmonary fibrosis. (20979602)	Nikolova S.... Pullamsetti S.S.	2010	PDE6G
6	FcI^ receptor IIIb (CD16b) polymorphisms are associate d with susceptibility to idiopathic pulmonary fibrosis. (20924590)	Bournazos S.... Hart S.P.	2010	FCGR3B
7	Elevated CC chemokine level in bronchoalveolar lavage fluid is predictive of a poor outcome of idiopathic pulmonary fibrosis. (19270434)	Shinoda H.... Ishizaka A.	2009	CCL2, CCL3
8	Serum CC-chemokine ligand 18 concentration predicts outcome in idiopathic pulmonary fibrosis. (19179488)	Prasse A.... MA1ller-Quernheim J.	2009	CCL18
9	Association of ENA-78, IP-10 and VEGF gene polymorph ism with idiopathic pulmonary fibrosis (20137269)	Liu L.... Xin P.	2009	VEGFA, CXCR3, CXCL10
10	Surfactant protein-D predicts survival in patients wi th idiopathic pulmonary fibrosis. (20560296)	Barlo N.P.... Grutters J.C.	2009	SFTPD
11	Interferon gamma for idiopathic pulmonary fibrosis. (19570574)	Bouros D.	2009	IFNG
12	Concentration of surfactant protein D, Clara cell pro tein CC-16 and IL-10 in bronchoalveolar lavage (BAL) in patients with sarcoidos is, hypersensivity pneumonitis and idiopathic pulmonary fibrosis. (19995708)	Kucejko W.... OssoliA8ska M.	2009	SFTPD, SCGB1A1
13	Is metalloproteinase-7 specific for idiopathic pulmonary fibrosis? (18071010)	Huh J.W.... Kim K.R.	2008	MMP7
14	The CR1 C5507G polymorphism is not involved in susceptibility to idiopathic pulmonary fibrosis in two European populations. (18937792)	Kubistova Z.... Petrek M.	2008	CR1
15	Epithelial endoplasmic reticulum stress and apoptosis in sporadic idiopathic pulmonary fibrosis. (18635891)	Korfei M.... Guenther A.	2008	XBP1, ATF4, ATF6
16	MMP-1 polymorphisms and the risk of idiopathic pulmonary fibrosis. (18853190)	Checa M.... Pardo A.	2008	MMP1
17	Involvement of selenoprotein P in the regulation of redox balance and myofibroblast viability in idiopathic pulmonary fibrosis. (17986007)	Kabuyama Y.... Homma Y.	2007	SEPP1
18	Th1/Th2 cytokine gene polymorphisms in patients with idiopathic pulmonary fibrosis. (16573560)	Vasakova M.... Sulc J.	2006	IL6, IL2, IL1B
19	Tissue inhibitor of metalloproteinase-3 is up-regulated by transforming growth factor-beta1 in vitro and expressed in fibroblastic foci in vivo in idiopathic pulmonary fibrosis. (16908447)	Garcia-Alvarez J.... Pardo A.	2006	TGFBR1, TIMP3
20	Changes of matrix metalloproteinase-9 and tissue inhibitor of metalloproteinase-1 in the bronchoalveolar lavage fluid and the serum of patients with idiopathic pulmonary fibrosis (17045024)	Wei L.Q.... Yu R.J.	2006	MMP9, TIMP1
21	The polymorphism C5507G of complement receptor 1 does not explain idiopathic pulmonary fibrosis among the Finns. (15733500)	Hodgson U.... Laitinen T.	2005	CR1
22	Comparison of BALF concentrations of ENA-78 and IP10 in patients with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. (16085216)	Nakayama S.... Kohno S.	2005	CXCR3, CXCL5
23	Enhanced expression of interleukin-18 and its receptor in idiopathic pulmonary fibrosis. (15308504)	Kitasato Y.... Aizawa H.	2004	IL18
24	Cyclooxygenase-2 is up-regulated in lung parenchyma of chronic obstructive pulmonary disease and down-regulated in idiopathic pulmonary fibrosis. (15127973)	Xaubet A.... Picado C.	2004	PTGS1
25	Diagnostic relevance of Langerin detection in cells from bronchoalveolar lavage of patients with pulmonary Langerhans cell histiocytosis, sarcoidosis and idiopathic pulmonary fibrosis. (14722767)	Smetana K.... Gabius H.J.	2004	CD207, CLEC12A
26	Increased level of soluble E-selectin in the serum fr om patients with idiopathic pulmonary fibrosis. (15072224)	Hayashi S.... Sakatani M.	2004	SELE
27	CCL22 and CCL17 in rat radiation pneumonitis and in human idiopathic pulmonary fibrosis. (15293604)	Inoue T.... Yamaguchi K.	2004	CCL22
28	Cytokine gene polymorphisms in idiopathic pulmonary fibrosis. (15030461)	Riha R.L.... Zimmerman P.V.	2004	TGFB1, TNF, IL6
29	Cytokine profiles in idiopathic pulmonary fibrosis suggest an important role for TGF-beta and IL-10. (12882453)	Bergeron A.... Tazi A.	2003	BMP6
30	Analysis of an IL-10 polymorphism in idiopathic pulmonary fibrosis. (12761561)	Whittington H.A.... Millar A.B.	2003	IL10
31	Serum surfactant proteins-A and -D as biomarkers in idiopathic pulmonary fibrosis. (11936520)	Greene K.E.... Mason R.J.	2002	SFTPD
32	Abnormal re-epithelialization and lung remodeling in idiopathic pulmonary fibrosis: the role of deltaN-p63. (12379768)	Chilosi M.... Doglioni C.	2002	TP53, TP63
33	Aberrations in the fragile histidine triad (FHIT) gene in idiopathic pulmonary fibrosis. (11731438)	Uematsu K.... Kudoh S.	2001	FHIT
34	ENA-78 is an important angiogenic factor in idiopathic pulmonary fibrosis. (11751193)	Keane M.P.... Strieter R.M.	2001	CXCL5
35	Upregulation of Fas-signalling molecules in lung epithelial cells from patients with idiopathic pulmonary fibrosis. (11334117)	Maeyama T.... Hara N.	2001	CASP1
36	Myofibroblasts and S-100 protein positive cells in idiopathic pulmonary fibrosis and rheumatoid arthritis-associated interstitial pneumonia. (10543278)	Yoshinouchi T.... Ueda N.	1999	VIM, S100A1
37	Serum level of interleukin 8 is elevated in idiopathic pulmonary fibrosis and indicates disease activity. (9517588)	Ziegenhagen M.W.... Muller-Quernheim J.	1998	IL8
38	Circulating KL-6 predicts the outcome of rapidly prog ressive idiopathic pulmonary fibrosis. (9817725)	Yokoyama A.... Hiwada K.	1998	ELANE
39	Increased expression of proinflammatory chemokines in bronchoalveolar lavage cells of patients with progressing idiopathic pulmonary fibrosis and sarcoidosis. (9676055)	Ziegenhagen M.W.... MA1ller-Quernheim J.	1998	IL8, CCL3
40	Hepatocyte growth factor and neutrophil elastase in idiopathic pulmonary fibrosis. (9415350)	Hojo S.... Takahara J.	1997	HGF, ELANE
41	Immunohistochemical identification and characterization of smooth muscle-like cells in idiopathic pulmonary fibrosis. (7582311)	Ohta K.... King T.E.	1995	ACTG2
42	Monocyte chemoattractant protein-1 in idiopathic pulmonary fibrosis and other interstitial lung diseases. (8200639)	Iyonaga K.... Takahashi K.	1994	CCL2
43	Acute exacerbation in idiopathic pulmonary fibrosis. Analysis of clinical and pathologic findings in three cases. (8404104)	Kondoh Y.... Takagi K.	1993	ALB
44	Elevated serum superoxide dismutase levels correlate with disease severity and neutrophil degranulation in idiopathic pulmonary fibrosis. (8403808)	BorzA- R.M.... Facchini A.	1993	SOD1, ELANE
45	Neutrophilic alveolitis in idiopathic pulmonary fibrosis. The role of interleukin-8. (1596015)	Lynch J.P.... Strieter R.M.	1992	IL8
46	Increased production and immunohistochemical localization of transforming growth factor-beta in idiopathic pulmonary fibrosis. (1892646)	Khalil N.... Greenberg A.H.	1991	BMP6
47	Increased expression of the interleukin-8 gene by alveolar macrophages in idiopathic pulmonary fibrosis. A potential mechanism for the recruitment and activation of neutrophils in lung fibrosis. (1752942)	CarrAc P.C.... Riches D.W.	1991	IL8
48	Platelet-derived growth factor in idiopathic pulmonary fibrosis. (2170444)	Antoniades H.N.... Selman M.	1990	PDGFB
49	Upregulation of platelet-derived growth factor-A and -B gene expression in alveolar macrophages of individuals with idiopathic pulmonary fibrosis. (2347924)	Nagaoka I.... Crystal R.G.	1990	PDGFA
50	Idiopathic pulmonary fibrosis: can cell mediated immunity markers predict clinical outcome? (2118691)	Meliconi R.... Gasbarrini G.	1990	IL2RB

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	Glioma Invasiveness36	10.5	BMP4, BMP6, PLAU, PDGFA, PDGFB, LTBP1
2	Actin-Based Motility by Rho Family GTPases36	10.0	BMP4, GSN, IL2, IGF1, CTGF, LTBP1
3	Transendothelial Migration of Leukocytes36	10.0	NCF1, NCF2, LAMC2, MMP1, MMP7, MMP9
4	eNOS Signaling36	10.0	BMP6, IL2, AQP5, CAV1, AKT1, DDR1
5	NFAT Signaling and Lymphocyte Interactions36	9.8	BMP4, BMP6, IL11, IL12A, IL12B, IL13
6	RAR-Gamma-RXR-Alpha Degradation36	9.7	BMP4, GTF2H2, IL11, IL12A, IL2, MAPK14
7	CREB Pathway36	9.7	BMP4, BMP6, KDR, ATF4, IL2, IL2RA
8	Chemokine Signaling36	9.6	IL8, MAPK8, MAPK14, MAPK1, CCL22, CCL2
9	ILK Signaling36	9.5	VIM, BMP4, KDR, LAMC2, ATF6, ATF4
10	IL-6 Pathway36	9.5	BMP4, BMP6, IL10, IL11, IL12A, IL12B
11	ERK5 Signaling36	9.3	BMP4, BMP6, KDR, ATF4, FLT1, IL2
12	JNK Pathway36	9.2	BMP4, BMP6, KDR, FLT1, IL1R1, IL2
13	Akt Signaling36	9.1	BMP4, ATF4, CD28, IL10, IL10RA, IL11
14	PGC1Alpha Pathway36	9.1	BMP4, BMP6, ATF4, IL10RA, IL11, IL12A
15	Nuclear Receptor Activation by Vitamin-A36	9.1	BMP4, BMP6, KDR, ATF4, FLT1, GTF2H2
16	eIF2 Pathway36	8.9	BMP4, BMP6, IL2, IL2RA, IL6, MAPK1
17	Telomerase Components in Cell Signaling36	8.9	BMP4, BMP6, KDR, FLT1, IL2, IGF1
18	NF-KappaB (p50-p65) Pathway36	8.8	BMP4, BMP6, KDR, FLT1, IL1A, IL1B
19	NF-KappaB Family Pathway36	8.8	BMP4, BMP6, KDR, FLT1, IL1A, IL1B
20	DHA Signaling36	8.8	BMP4, BMP6, KDR, FLT1, IL1B, IL2
21	all-trans-Retinoic Acid Signaling in Brain36	8.8	BMP4, BMP6, IL10, IL11, IL12A, IL12B
22	Pertussis20	8.7	SFTPA1, IL10, IL12A, IL12B, IL1A, IL1B
23	Rac1 Pathway36	8.7	NCF2, BMP4, BMP6, KDR, IL2, IL2RA
24	Endothelin-1 Signaling Pathway36	8.7	BMP4, BMP6, FLT1, IL10, IL11, IL12A
25	Nanog in Mammalian ESC Pluripotency36	8.7	BMP4, BMP6, KDR, FLT1, IL2, IL6
26	THC Differentiation Pathway36	8.6	CD28, IL10, IL10RA, IL12A, IL12B, IL18
27	Tec Kinases Signaling36	8.6	BMP4, BMP6, CD28, IL10, IL10RA, IL11
28	p53 Mediated Apoptosis36	8.6	BMP4, BMP6, KDR, FLT1, IL2, IL2RA
29	Rheumatoid arthritis20	8.5	VEGFA, CD28, MMP1, FLT1, IL11, IL18
30	ERK Signaling36	8.4	BMP4, BMP6, LAMC2, ATF4, FN1, FLT1
31	mTOR Pathway36	8.4	BMP4, BMP6, IL2, IL6, MAPK8, MAPK14
32	PEDF Induced Signaling36	8.3	BMP4, BMP6, CFLAR, IL10, IL11, IL12A
33	Phospholipase-C Pathway36	8.3	BMP4, BMP6, KDR, LAMC2, ATF4, FN1
34	Leishmaniasis20	8.3	NCF1, NCF2, FCGR3B, IL10, IL12A, IL12B
35	Mitochondrial Apoptosis36	8.2	BMP4, BMP6, KDR, CDKN2A, FLT1, IL2
36	Chagas disease (American trypanosomiasis)20	8.2	CFLAR, IL10, IL12A, IL12B, IL1B, IL6
37	Estrogen Pathway36	8.2	BMP4, BMP6, KDR, ATF4, FLT1, GTF2H2
38	Glucocorticoid Receptor Signaling36	8.1	BMP4, BMP6, ATF4, GTF2H2, IL10, IL10RA
39	Cellular Apoptosis Pathway36	8.0	BMP4, BMP6, KDR, CFLAR, CDKN2A, FLT1
40	NFAT in Immune Response36	7.9	BMP4, BMP6, KDR, CD28, FLT1, FCGR3B
41	Rho Family GTPases36	7.9	NCF2, VIM, BMP4, BMP6, KDR, LAMC2
42	p70S6K Signaling36	7.8	BMP4, BMP6, KDR, FLT1, IL2, IL2RA
43	Pancreatic Adenocarcinoma36	7.6	BMP4, BMP6, KDR, CDKN2A, MMP1, MMP7
44	Renin-Angiotensin Pathway36	7.5	BMP4, BMP6, KDR, FLT1, IL2, IL2RA
45	STAT3 Pathway36	7.5	BMP4, BMP6, CD28, IL10, IL10RA, IL11
46	JAK-STAT Pathway36	7.5	BMP4, BMP6, KDR, FLT1, IL10, IL10RA
47	MAPK Signaling36	7.4	BMP4, BMP6, KDR, LAMC2, ATF6, ATF4
48	TGF-Beta Pathway36	7.4	BMP4, BMP6, KDR, FLT1, IL10, IL10RA
49	Antioxidant Action of Vitamin-C36	7.3	NCF1, NCF2, BMP4, BMP6, KDR, CD28
50	MAPK Family Pathway36	7.3	BMP4, BMP6, KDR, ATF4, FLT1, IL10

Compounds related to idiopathic pulmonary fibrosis according to GeneDecks:

(show top 50)    (show all 411)

id	Compound	Score	Top Affiliating Genes
1	gp 13032	10.3	BMP4, BMP6, SCGB1A1, IL13, IL1R1, IL2RA
2	acth32	10.1	SERPINA1, MIF, GRP, IL11, IL1A, IL1B
3	cuprophan32	9.9	FN1, IL1B, IL1R1, IL1RAPL2, IL1RN, IL2RA
4	epinephrine32 9 18 9	12.8	SELE, BMP6, MMP1, MMP9, GRP, IL11
5	pge232	9.7	SFTPB, SELE, BMP4, MMP1, FN1, GRP
6	etoposide32 42 9 9	12.7	SELE, CFLAR, ATF4, CDKN3, GRP, MAPK8
7	procollagen32	9.6	VIM, BMP6, CD36, MMP1, MMP9, IL1A
8	h2o232	9.6	NCF2, SERPINA1, SEPP1, SERPINB1, CLEC12A, MPO
9	aspartate32	9.5	VIM, BMP6, SCGB1A1, CDKN2A, MMP9, IL11
10	polysaccharide32	9.4	SFTPD, SELE, SERPINA1, BMP6, CLEC12A, KDR
11	bleomycin32 9 9	11.4	SFTPB, SFTPC, CDKN3, IL13, IL2RA, IL4
12	nifedipine32 9 9	11.4	SELE, BMP6, MMP9, GRP, IL1A, IL2RB
13	ribonucleic acid32	9.2	SFTPB, SFTPC, BMP4, BMP6, MMP1, MMP7
14	tarc32	9.2	IL10, IL13, IL18, IL1A, IL4, IL8
15	arginine32	9.1	SFTPB, SFTPC, ATF4, FN1, GRP, IL13
16	dextran sulfate32	9.1	SELE, MPO, CD36, MMP7, FN1, IL6
17	heparin32 9 18 9	12.0	SERPINA1, SEPP1, SERPINB1, VIM, BMP4, CLEC12A
18	rapamycin32 42	10.0	VIM, VEGFA, BMP4, KDR, CFLAR, ATF4
19	groalpha32	8.9	SELE, IL10, IL13, IL18, IL1A, IL1B
20	sodium arsenite32	8.9	CFLAR, IL1A, IL2RA, MAPK8, MAPK14, CCND1
21	parthenolide32	8.8	MMP9, IL6, IL8, MAPK8, MAPK14, CCL2
22	lipoteichoic acid32	8.8	SELE, IL10, IL12B, IL18, IL1B, IL1R1
23	sulfasalazine32 9 9	10.6	SELE, MPO, IL12B, IL1A, IL1B, IL1R1
24	fibrinogen32	8.6	SFTPB, SFTPC, SELE, SERPINA1, CLEC12A, MMP7
25	il-1232	8.6	IL10, IL12B, IL13, IL18, IL1B, IL1R1
26	glutamine32	8.6	SFTPD, VIM, BMP6, CD36, FN1, GRP
27	testosterone32 9 18 9	11.5	SELE, SEPP1, VIM, CFLAR, CDKN2A, MMP1
28	tacrolimus32 34 9 9	11.4	BMP6, CD28, CD36, CDKN3, FN1, IL10
29	ag 147832	8.4	MMP7, MMP9, IL8, MAPK8, MAPK14, MAPK1
30	glutamate32	8.4	VIM, BMP6, KDR, CDKN3, MMP9, GSN
31	doxorubicin32 34 9 9	11.4	SELE, VIM, BMP6, KDR, CFLAR, ATF4
32	cysteine32	8.3	SFTPB, SFTPC, SFTPD, SERPINA1, SEPP1, BMP4
33	vitamin d32	8.2	VIM, BMP6, XBP1, CD36, FN1, GSN
34	pentoxifylline32 9 9	10.1	BMP6, MPO, MMP1, IL10, IL12B, IL13
35	losartan32 34 9 9	11.1	SELE, BMP6, CD36, MMP1, MMP16, CCL2
36	etanercept32 9 9	10.0	MMP1, FCGR3B, IL10, IL11, IL18, IL1B
37	thapsigargin32 42	8.8	XBP1, ATF6, ATF4, CD36, CDKN3, GRP
38	infliximab32 9 9	9.6	SELE, VEGFA, MMP1, IL10, IL11, IL18
39	cyclosporin a32 42	8.6	BMP4, CFLAR, CD28, CDKN3, MMP1, MMP9
40	gm-csf32	7.6	VEGFA, BMP6, IL10, IL11, IL13, IL18
41	azathioprine32 34 9 9	10.5	VIM, BMP4, CDKN2A, GSN, IL10, IL11
42	phosphatidylinositol32	7.4	NCF2, SFTPD, BMP4, BMP6, KDR, SCGB1A1
43	15-deoxy-delta-12,14-prostaglandin j232	7.3	VEGFA, BMP6, CD36, IL10, IL1B, IL4
44	histamine32 18	8.1	SFTPD, SELE, VIM, BMP6, MPO, FHIT
45	prostacyclin32	7.0	SELE, VEGFA, BMP6, KDR, FN1, FLT1
46	triptolide32	6.9	VEGFA, IL1B, IL2, IL6, IL8, MAPK8
47	alanine32	6.6	SELE, VIM, BMP6, SCGB1A1, CDKN2A, CDKN3
48	paclitaxel32 34 9 9	9.5	VIM, VEGFA, BMP6, KDR, CFLAR, CDKN2A
49	cisplatin32 34 9 9	8.0	VEGFA, BMP6, KDR, CFLAR, ATF4, MPO
50	dexamethasone32 42 34 9 9	8.5	SFTPB, SFTPC, SFTPD, 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