Cystic Fibrosis malady

MedlinePlus: Cystic fibrosis (cf) is an inherited disease of the mucus and sweat glands. it affects mostly your lungs, pancreas, liver, intestines, sinuses and sex organs. cf causes your mucus to be thick and sticky. the mucus clogs the lungs, causing breathing problems and making it easy for bacteria to grow. this can lead to problems such as repeated lung infections and lung damage. the symptoms and severity of cf vary widely. some people have serious problems from birth. others have a milder version of the disease that doesn't show up until they are teens or young adults. although there is no cure for cf, treatments have improved greatly in recent years. until the 1980s, most deaths from cf occurred in children and teenagers. today, with improved treatments, some people who have cf are living into their forties, fifties, or older. nih: national heart, lung, and blood institute²³

MalaCards: Cystic Fibrosis, also known as mucoviscidosis, is related to fibrosis and pancreatitis. An important gene associated with Cystic Fibrosis is CFTR (cystic fibrosis transmembrane conductance regulator (ATP-binding cassette sub-family C, member 7)), and among its related pathways are Antioxidant Action of Vitamin-C and Rho Family GTPases. The drugs amiloride hydrochloride and coenzyme q10 and the compounds arginine and glutamate have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, whole blood and brain, and related mouse phenotypes are homeostasis/metabolism and mortality/aging.

Genetics Home Reference: Cystic fibrosis is an inherited disease characterized by the buildup of thick, sticky mucus that can damage many of the body's organs. The disorder's most common signs and symptoms include progressive damage to the respiratory system and chronic digestive system problems. The features of the disorder and their severity varies among affected individuals.¹⁷

NIH Rare Diseases: Cystic fibrosis (CF) is an inherited condition that causes mucus to build up and clog some of the organs in the body, particularly in the lungs and pancreas. When mucus clogs the lungs, it can make breathing very difficult. The thick mucus also causes bacteria to get stuck in the airways, which causes inflammation and infections. These infections can cause chronic coughing, and wheezing. Over time, mucus buildup and infections can lead to permanent lung damage, including the formation of scar tissue (fibrosis) and cysts in the lungs. Mucus can also block the digestive tract and pancreas, leading to digestive problems. CF is caused by mutations in the CFTR gene and is inherited in an autosomal recessive fashion.³⁰

Wikipedia: Cystic fibrosis (also known as CF or mucoviscidosis) is an autosomal recessive genetic disorder that...⁴⁴ more...

OMIM: 219700

Aliases & Descriptions:

cystic fibrosis 6 7 44 30 16 17 8 33 32 23 43 mucoviscidosis 6 44 30 16 17 cf 6 44 30 17 fibrocystic disease of the pancreas 44

fibrocystic disease of pancreas 17 breast fibrocystic disease 43 chronic fatigue syndrome 43 cfs 16

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

(show top 50)    (show all 914)

id	Related Disease	Score	Top Affiliating Genes
1	fibrosis	111.1	PIK3CG, PI3, PDE3A, PDE4A, RECK, REG1A
2	pancreatitis	74.6	PIK3CG, PI3, PDE4A, RECK, REG1A, REG3A
3	carcinoma	74.5	PIK3CG, PI3, PDE3A, PDE4A, RECK, REG1A
4	neuronitis	63.6	PIK3CG, PDE3A, PDE4A, RECK, REG1A, REG3A
5	prostatitis	58.9	PIK3CG, PDE4A, RECK, SGK1, VWF, SFTPD
6	adenocarcinoma	58.2	PIK3CG, RECK, REG1A, REG3A, VWF, SFTPB
7	cholesterol	52.9	PIK3CG, RECK, REG1A, REN, SGK1, WASL
8	rheumatoid arthritis	48.9	PIK3CG, PDE4A, RECK, VWF, SFTPD, PCNA
9	pneumonia	48.4	PI3, PDE4A, SFTPA1, SFTPB, SFTPC, SFTPD
10	hepatocellular carcinoma	48.2	PIK3CG, RECK, REG1A, REG3A, VWF, PCNA
11	pulmonary disease	47.5	PIK3CG, PI3, PDE4A, REN, VWF, SFTPA1
12	diabetes mellitus	47.4	PIK3CG, PI3, PDE4A, RECK, REG1A, REN
13	thyroiditis	47.3	PIK3CG, PDE4A, RECK, REN, VWF, SFTPA1
14	colon cancer	46.9	PIK3CG, PDE4A, RECK, REG1A, VWF, PCNA
15	alzheimer's disease	46.3	PIK3CG, PDE4A, RECK, REG1A, REN, WASL
16	hypertension	46.1	PIK3CG, PDE3A, PDE4A, REN, SGK1, VWF
17	cystic fibrosis lung disease	45.1	RECK, GCLC, SERPINA1, BPI, RASSF6, SCGB1A1
18	hypoxia	44.9	PIK3CG, PDE4A, RECK, REN, VWF, SFTPA1
19	cervicitis	44.6	PIK3CG, PDE4A, RECK, REN, VWF, PCNA
20	influenza	44.4	PIK3CG, RECK, SFTPA1, SFTPD, SELL, NAGA
21	sepsis	41.3	PI3, PDE4A, RECK, SFTPD, RBP4, SELL
22	bronchiectasis	41.2	SERPINA1, SERPINA3, SERPINB1, BPI, SCNN1A, SCNN1B
23	breast carcinoma	40.3	PIK3CG, PI3, RECK, WASL, VWF, SFTPB
24	aspergillosis	40.3	SFTPA1, SFTPD, LMAN1, CFTR, FXYD5, CD79A
25	colon carcinoma	40.2	PIK3CG, SELL, SELP, VIP, BRCA1, VEGFA
26	lupus erythematosus	40.1	PIK3CG, RECK, VWF, SFTPD, PCNA, RBP4
27	infertility	39.8	SGK1, GC, SERPINA1, BRCA1, VEGFA, VDAC1
28	rhinitis	39.7	PIK3CG, PDE4A, SFTPA1, SFTPD, SELL, SERPINB1
29	cholera	39.2	PDE4A, SEC61A1, SEC61B, VIP, VDAC1, VCP
30	chronic obstructive pulmonary disease	39.0	PI3, PDE4A, SFTPB, SFTPD, GC, SERPINA1
31	duodenitis	38.5	PIK3CG, REG3A, SGK1, PCNA, VIP, LPL
32	insulin resistance	38.1	PIK3CG, REN, SGK1, VWF, SFTPA1, RBP4
33	pancreatic diseases	37.9	REG1A, REG3A, NAGLU, NAT1, NAT2, CLPS
34	pertussis	37.8	PIK3CG, PI3, PDE4A, SFTPA1, SELL, VIP
35	cholangitis	37.7	PCNA, BPI, SCT, MUC2, LBR, CFTR
36	allergic bronchopulmonary aspergillosis	37.4	SFTPA1, SFTPD, CFTR, FLNC, IL10, IL4
37	copd	37.1	PDE4A, VWF, SFTPB, SFTPD, GCLC, GC
38	systemic lupus erythematosus	36.9	RECK, VWF, RBP4, SERPINB1, VIP, NAT2
39	sinusitis	36.6	PI3, VWF, SFTPD, PCNA, SELL, SELP
40	blindness	36.6	PIK3CG, PDE4A, REN, VWF, RBP4, SELP
41	sclerosing cholangitis	36.5	BPI, SCT, LBR, CFTR, CEACAM3, MPO
42	alpha 1-antitrypsin deficiency	36.4	SFTPB, GC, SERPINE2, SERPINC1, SERPINA1, SERPINA3
43	bronchitis	36.3	SERPINA1, SERPINB1, VIP, LACTB, S100A8, S100A9
44	primary sclerosing cholangitis	36.0	BPI, CFTR, MPO, GPT, HLA-A, DMBT1
45	pulmonary function	35.7	SFTPB, SERPINA1, LEP, CFTR, CEACAM3, MPO
46	asthma	35.5	MME, IL13, IL4, IL5, IGES, IGHE
47	cervical cancer	35.5	PIK3CG, PCNA, SERPINC1, SERPINA1, SERPINA3, NAT2
48	hereditary pancreatitis	35.5	REG1A, REG3A, SERPINA1, BRCA1, LPL, BCHE
49	hepatitis	35.5	RBP4, SELP, SERPINA1, XDH, GUSB, GSR
50	polyposis	35.4	PIK3CG, PCNA, VIP, NAT1, NAT2, BRCA1

Clinical features from OMIM: 219700

Approved drugs:

Drug clinical trials:

Inferred drug relations via UMLS/NDF-RT:

^43 28 acetylcysteine, amiloride, amiloride hydrochloride, coenzyme q10, danazol, dornase alfa,rdna, fish oil, mct, phenylbutyrate sodium, venlafaxine, venlafaxine hydrochloride

Cell-based therapeutics:

Mesenchymal stem cell, PMIDs: 21658631, 16179642

MalaCards organs/tissues related to cystic fibrosis:

²²

id	Organ / Tissue -> Anatomical Compartment -> Cell	Relevance
1	Lung -> Respiratory Bronchioles -> Basal Cells	Potential therapeutic candidate
2	Lung -> Respiratory Bronchioles -> Cilliated Cells	Potential therapeutic candidate, affected by disease
3	Lung -> Submucosal Glands	Potential therapeutic candidate, affected by disease

MGI Mouse Phenotypes related to cystic fibrosis:

²⁵ (show all 28)

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	homeostasis/metabolism phenotype	MP:0005376	30.6	PIK3CG, REG1A, REG3A, REN, SGK1, VWF
2	mortality/aging	MP:0010768	25.6	RECK, REN, WASL, VWF, SFTPB, SFTPC
3	growth/size phenotype	MP:0005378	20.7	PIK3CG, RECK, REN, SGK1, WASL, SFTPC
4	immune system phenotype	MP:0005387	20.2	PDE4A, REN, WASL, VWF, SELL, SELP
5	behavior/neurological phenotype	MP:0005386	19.5	REN, SFTPB, SFTPC, BTD, SELP, SERPINE2
6	cardiovascular system phenotype	MP:0005385	18.8	PIK3CG, PDE4A, RECK, REN, WASL, VWF
7	cellular phenotype	MP:0005384	18.3	PIK3CG, WASL, VWF, PCNA, GCLC, RBP4
8	nervous system phenotype	MP:0003631	18.3	PDE4A, RECK, REN, WASL, VWF, SELP
9	normal phenotype	MP:0002873	16.8	PDE3A, REN, WASL, GCLC, RBP4, VIP
10	digestive/alimentary phenotype	MP:0005381	16.6	WASL, VWF, BRCA1, VEGFA, VDR, CLCN3
11	renal/urinary system phenotype	MP:0005367	16.4	REN, SGK1, BTD, SELP, NAGLU, BRCA1
12	integument phenotype	MP:0010771	15.7	PIK3CG, RECK, BTD, SELP, BRCA1, RAF1
13	hematopoietic system phenotype	MP:0005397	15.7	PCNA, SERPINB1, BRCA1, LNX1, VAMP3, CLNK
14	liver/biliary system phenotype	MP:0005370	15.1	RECK, GCLC, SEC61A1, SERPINC1, VEGFA, LPL
15	reproductive system phenotype	MP:0005389	15.0	PDE3A, REG1A, REN, SFTPC, RBP4, SERPINE2
16	embryogenesis phenotype	MP:0005380	14.5	RECK, WASL, GCLC, SERPINC1, NAT1, BRCA1
17	respiratory system phenotype	MP:0005388	14.1	SFTPC, SFTPD, SELL, SELP, SERPINB1, BRCA1
18	endocrine/exocrine gland phenotype	MP:0005379	14.1	REG1A, WASL, SEC61A1, SELL, SERPINE2, BRCA1
19	muscle phenotype	MP:0005369	13.5	PIK3CG, REN, BTD, RBP4, SEC61A1, BRCA1
20	craniofacial phenotype	MP:0005382	13.4	NAGLU, MYO6, CLPS, CLCN5, PAH, LBR
21	adipose tissue phenotype	MP:0005375	13.3	SEC61A1, BRCA1, LPL, CLCN3, CLPS, KCNJ11
22	no phenotypic analysis	MP:0003012	13.3	SGK1, SFTPC, PCNA, SERPINE2, VEGFA, CLNK
23	vision/eye phenotype	MP:0005391	13.0	SFTPC, BTD, RBP4, SELP, SERPINC1, NAGLU
24	tumorigenesis	MP:0002006	12.9	SFTPC, SELL, BRCA1, SCGB1A1, LEP, CHUK
25	skeleton phenotype	MP:0005390	12.7	PIK3CG, RECK, GC, NAGLU, BRCA1, VEGFA
26	limbs/digits/tail phenotype	MP:0005371	12.3	RECK, BRCA1, VEGFA, VDR, SCD, LEP
27	hearing/vestibular/ear phenotype	MP:0005377	12.1	NAGLU, VDR, MYO6, KCNQ1, CHUK, BAX
28	other phenotype	MP:0005395	11.7	RECK, SCNN1G, CD40LG, MME, FN1, IL13

Articles related to cystic fibrosis:

(show top 50)    (show all 2026)

id	Title	Authors	Year	Affiliating Genes
1	An association study on contrasting cystic fibrosis e ndophenotypes recognizes KRT8 but not KRT18 as a modifier of cystic fibrosis di sease severity and CFTR mediated residual chloride secretion. (21548936)	Stanke F.... TA1mmler B.	2011	KRT18, KRT8
2	Novel de novo large deletion in cystic fibrosis trans membrane conductance regulator gene results in a severe cystic fibrosis phenoty pe. (21663921)	Norek A.... Jurek M.	2011	CFTR
3	Transient receptor potential canonical channel 6 link s Ca2+ mishandling to cystic fibrosis transmembrane conductance regulator chann el dysfunction in cystic fibrosis. (20203293)	Antigny F.... Vandebrouck C.	2011	CFTR, TRPC6
4	The cystic fibrosis-causing mutation deltaF508 affect s multiple steps in cystic fibrosis transmembrane conductance regulator biogene sis. (20667826)	Thibodeau P.H.... Thomas P.J.	2010	CFTR
5	Cystic fibrosis transmembrane conductance regulator c hannel dysfunction in non-cystic fibrosis bronchiectasis. (20167849)	Bienvenu T.... Fajac I.	2010	CFTR
6	Cystic fibrosis transmembrane conductance regulator m utation spectrum in patients with cystic fibrosis in Tunisia. (19715466)	Fredj S.H.... Claustres M.	2009	CFTR
7	Insulin therapy to improve BMI in cystic fibrosis-rel ated diabetes without fasting hyperglycemia: results of the cystic fibrosis rel ated diabetes therapy trial. (19592632)	Moran A.... Allen H.	2009	INS
8	Unique mutations of the cystic fibrosis transmembrane conductance regulator gene of three cases of cystic fibrosis in Nagasaki, Japa n. (19652440)	Izumikawa K.... Kohno S.	2009	CFTR
9	Genetic analysis of Rwandan patients with cystic fibrosis-like symptoms: identification of novel cystic fibrosis transmembrane conductance regulator and epithelial sodium channel gene variants. (19017867)	Mutesa L.... Bours V.	2009	CFTR, SCNN1A, SCNN1B
10	Tumor necrosis factor-alpha in airway secretions from cystic fibrosis patients upregulate endothelial adhesion molecules and induce airway epithelial cell apoptosis: implications for cystic fibrosis lung disease. (19144271)	Mitola S.... De Rose V.	2008	TNF
11	Relationships between cystic fibrosis transmembrane conductance regulator, extracellular nucleotides and cystic fibrosis. (16828872)	Marcet B.... Boeynaems J.M.	2006	CFTR
12	Up-regulation of AMP-activated kinase by dysfunctional cystic fibrosis transmembrane conductance regulator in cystic fibrosis airway epithelial cells mitigates excessive inflammation. (16361706)	Hallows K.R.... Pilewski J.M.	2006	CFTR, PRKAA2
13	Differential sensitivity of the cystic fibrosis (CF)-associated mutants G551D and G1349D to potentiators of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel. (16311240)	Cai Z.... Sheppard D.N.	2006	CFTR
14	Spliceosome-mediated RNA trans-splicing with recombinant adeno-associated virus partially restores cystic fibrosis transmembrane conductance regulator function to polarized human cystic fibrosis airway epithelial cells. (16149910)	Liu X.... Engelhardt J.F.	2005	CFTR
15	Na+/H+ exchanger regulatory factor isoform 1 overexpression modulates cystic fibrosis transmembrane conductance regulator (CFTR) expression and activity in human airway 16HBE14o- cells and rescues DeltaF508 CFTR functional expression in cystic fibrosis cells. (16203733)	Guerra L.... Casavola V.	2005	CFTR, SLC9A3R1
16	TLR-induced inflammation in cystic fibrosis and non-cystic fibrosis airway epithelial cells. (15661927)	Greene C.M.... McElvaney N.G.	2005	IL8, TLR4, ELANE
17	Reversal of cystic fibrosis phenotype in a cultured Delta508 cystic fibrosis transmembrane conductance regulator cell line by oligonucleotide insertion. (15148387)	Zamecnik P.C.... Cantiello H.F.	2004	CFTR
18	Patients with cystic fibrosis managed at the cystic fibrosis units of Madrid: cross-sectional study of 387 subjects (15171831)	Garcia Hernandez G.... Prados C.	2004	CFTR
19	Free secretory component from cystic fibrosis sputa d isplays the cystic fibrosis glycosylation phenotype. (14597481)	Marshall L.J.... Shute J.K.	2004	IL8
20	Compacted DNA nanoparticles administered to the nasal mucosa of cystic fibrosis subjects are safe and demonstrate partial to complete cystic fibrosis transmembrane regulator reconstitution. (15684701)	Konstan M.W.... Cooper M.J.	2004	CFTR
21	Cystic fibrosis transmembrane conductance regulator (CFTR)-mediated residual chloride secretion does not protect against early chronic Pseudomonas aeruginosa infection in F508del homozygous cystic fibrosis patients. (14605249)	Derichs N.... Ballmann M.	2004	CFTR
22	The cystic fibrosis mutation G551D alters the non-Mic haelis-Menten behavior of the cystic fibrosis transmembrane conductance regulat or (CFTR) channel and abolishes the inhibitory Genistein binding site. (12124395)	Derand R.... Becq F.	2002	CFTR
23	Tyrosine kinase c-Src constitutes a bridge between cystic fibrosis transmembrane regulator channel failure and MUC1 overexpression in cystic fibrosis. (11872746)	Gonzalez-Guerrico A.M.... Santa-Coloma T.A.	2002	SRC, CFTR, MUC1
24	Identification of mutation in the gene cystic fibrosis transmembrane regulator (CFTR) in Chilean patients with cystic fibrosis (11680956)	Repetto G.... Foradori A.	2001	CFTR
25	Early diagnosis of cystic fibrosis through neonatal screening prevents severe malnutrition and improves long-term growth. Wisconsin Cystic Fibrosis Neonatal Screening Study Group. (11134427)	Farrell P.M.... Splaingard M.L.	2001	CFTR
26	Effects of cystic fibrosis and congenital bilateral absence of the vas deferens-associated mutations on cystic fibrosis transmembrane conductance regulator-mediated regulation of separate channels. (10762539)	Mickle J.E.... Cutting G.R.	2000	CFTR
27	Identification of novel mutations in Arabs with cystic fibrosis and their impact on the cystic fibrosis transmembrane regulator mutation detection rate in Arab populations. (10834512)	Kambouris M.... Meyer B.F.	2000	CFTR
28	Cystic fibrosis transmembrane conductance regulator does not affect neutrophil migration across cystic fibrosis airway epithelial monolayers. (10751364)	Pizurki L.... Suter S.	2000	CFTR
29	Expression of cystic fibrosis transmembrane conductance regulator in liver tissue from patients with cystic fibrosis. (10915740)	Kinnman N.... Hultcrantz R.	2000	CFTR
30	Abnormal expression of the cystic fibrosis transmembrane regulator in chronic sinusitis in cystic fibrosis and non-cystic fibrosis patients. (10378526)	Coltrera M.D.... Gown A.M.	1999	CFTR
31	Radiological analysis of children with cystic fibrosi s who are homozygous for cystic fibrosis transmembrane conductance regulator mu tation S549R (T-->G). (10401194)	Frossard P.M.... Dawson K.P.	1999	CFTR
32	Expression of delta F508 cystic fibrosis transmembrane conductance regulator protein and related chloride transport properties in the gallbladder epithelium from cystic fibrosis patients. (10347100)	Dray-Charier N.... Housset C.	1999	CFTR
33	Recombinant DNase in cystic fibrosis: a protocol for targeted introduction through n-of-1 trials. Scottish Cystic Fibrosis Group. (10836333)	BAPllert F.G.... Innes J.A.	1999	APEX1
34	The ROMK-cystic fibrosis transmembrane conductance re gulator connection: new insights into the relationship between ROMK and cystic fibrosis transmembrane conductance regulator channels. (9442363)	Ho K.	1998	KCNJ1
35	A mutation in the cystic fibrosis transmembrane conductance regulator gene associated with elevated sweat chloride concentrations in the absence of cystic fibrosis. (9499426)	Mickle J.E.... Cutting G.R.	1998	CFTR
36	Detection of five novel mutations of the cystic fibrosis transmembrane regulator (CFTR) gene in Pakistani patients with cystic fibrosis: Y569D, Q98X, 296+12(T>C), 1161delC and 621+2(T>C). (9482579)	Malone G.... Super M.	1998	CFTR
37	Cystic-fibrosis-like disease unrelated to the cystic fibrosis transmembrane conductance regulator. (9654209)	Mekus F.... Veeze H.J.	1998	CFTR
38	Identification of common cystic fibrosis mutations in African-Americans with cystic fibrosis increases the detection rate to 75%. (9150159)	Macek M.... Cutting G.R.	1997	CFTR
39	A cystic fibrosis transmembrane conductance regulator splice variant with partial penetrance associated with variable cystic fibrosis presentations. (9196095)	Kerem E.... Kerem B.	1997	CFTR
40	Ursodeoxycholic acid for liver disease associated wit h cystic fibrosis: a double-blind multicenter trial. The Italian Group for the Study of Ursodeoxycholic Acid in Cystic Fibrosis. (8675168)	Colombo C.... Giunta A.	1996	GGT1
41	Repeat administration of an adenovirus vector encodin g cystic fibrosis transmembrane conductance regulator to the nasal epithelium o f patients with cystic fibrosis. (8617884)	Zabner J.... Standaert T.A.	1996	CFTR
42	Normalization of raised sodium absorption and raised calcium-mediated chloride secretion by adenovirus-mediated expression of cystic fibrosis transmembrane conductance regulator in primary human cystic fibrosis airway epithelial cells. (7533790)	Johnson L.G.... Boucher R.C.	1995	CFTR
43	Neonatal screening for cystic fibrosis: result of a pilot study using both immunoreactive trypsinogen and cystic fibrosis gene mutation analyses. (8530001)	FAcrec C.... Roussey M.	1995	CFTR
44	Biochemical characterization of the cystic fibrosis transmembrane conductance regulator in normal and cystic fibrosis epithelial cells. (1370488)	Sarkadi B.... Scarborough G.A.	1992	CFTR
45	Mutations and sequence variations detected in the cystic fibrosis transmembrane conductance regulator (CFTR) gene: a report from the Cystic Fibrosis Genetic Analysis Consortium. (1284534)	Tsui L.-C.	1992	CFTR
46	Abnormal localization of cystic fibrosis transmembrane conductance regulator in primary cultures of cystic fibrosis airway epithelia. (1379244)	Denning G.M.... Welsh M.J.	1992	RECK
47	Expression of the cystic fibrosis transmembrane conductance regulator gene in the respiratory tract of normal individuals and individuals with cystic fibrosis. (1713683)	Trapnell B.C.... Crystal R.G.	1991	CFTR
48	Cystic fibrosis with three mutations in the cystic fibrosis transmembrane conductance regulator gene. (1715308)	DAPrk T.... TA1mmler B.	1991	CFTR
49	Sequence analysis of the cystic fibrosis gene in patients with disseminated bronchiectatic lung disease. Application in the identification of a cystic fibrosis patient with atypical clinical course. (1749205)	Poller W.... MA1ller K.M.	1991	CFTR
50	A cluster of cystic fibrosis mutations in the first nucleotide- binding fold of the cystic fibrosis conductance regulator protein. (1695717)	Cutting G.R.... Kazazian H.H. Jr.	1990	CFTR

Genes related to cystic fibrosis according to GeneCards:

(show top 50)    (show all 654)

id	Symbol	Description	Score	GeneCards Section Context
1	CFTR	cystic fibrosis transmembrane conductance regulator (ATP-binding cassette sub-family C, member 7)	11.1	Publications, Summaries, Databases, Aliases & Descriptions (show all 6 sections) Domains & Families, Orthologs
2	S100A8	S100 calcium binding protein A8	11.1	Publications, Aliases & Descriptions, Summaries
3	CFM1	cystic fibrosis modifier 1	11.1	Publications, Aliases & Descriptions, Orthologs
4	SLC9A3R1	solute carrier family 9, subfamily A (NHE3, cation proton antiporter 3), member 3 regulator 1	11.1	Publications, Summaries
5	DEFB1	defensin, beta 1	11.1	Publications, Summaries
6	DNASE1	deoxyribonuclease I	11.1	Publications, UniProt Related, Summaries
7	SLC9A3R2	solute carrier family 9, subfamily A (NHE3, cation proton antiporter 3), member 3 regulator 2	11.1	Publications, Summaries
8	DERL1	derlin 1	11.1	Publications, Summaries
9	DNAJC5	DnaJ (Hsp40) homolog, subfamily C, member 5	11.1	Publications, Summaries
10	IFRD1	interferon-related developmental regulator 1	11.1	Publications, Summaries
11	S100A9	S100 calcium binding protein A9	11.1	Publications, Summaries
12	CLCA2	chloride channel accessory 2	11.1	Publications, Summaries
13	ATF6	activating transcription factor 6	11.1	Publications, Summaries
14	CFM2	cystic fibrosis modifier 2	11.1	Aliases & Descriptions
15	CFTRP1	cystic fibrosis transmembrane conductance regulator pseudogene 1	11.1	Aliases & Descriptions
16	PDZD3	PDZ domain containing 3	11.0	Summaries
17	ELANE	elastase, neutrophil expressed	11.0	Publications
18	IL8	interleukin 8	11.0	Publications
19	MBL2	mannose-binding lectin (protein C) 2, soluble	11.0	Publications
20	MPO	myeloperoxidase	11.0	Publications
21	MUC5AC	mucin 5AC, oligomeric mucus/gel-forming	11.0	Publications
22	TGFB1	transforming growth factor, beta 1	11.0	Publications
23	APEX1	APEX nuclease (multifunctional DNA repair enzyme) 1	11.0	Publications
24	BPI	bactericidal/permeability-increasing protein	11.0	Publications
25	RNASE3	ribonuclease, RNase A family, 3	11.0	Publications
26	SCGB1A1	secretoglobin, family 1A, member 1 (uteroglobin)	11.0	Publications
27	CXCR1	chemokine (C-X-C motif) receptor 1	11.0	Publications
28	DMBT1	deleted in malignant brain tumors 1	11.0	Publications
29	RECK	reversion-inducing-cysteine-rich protein with kazal motifs	11.0	Publications
30	SERPINA1	serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 1	11.0	Publications
31	ADRB2	adrenoceptor beta 2, surface	11.0	Publications
32	IL17A	interleukin 17A	11.0	Publications
33	IL1R1	interleukin 1 receptor, type I	11.0	Publications
34	PF4V1	platelet factor 4 variant 1	11.0	Publications
35	MUC3B	mucin 3B, cell surface associated	11.0	Publications
36	GOPC	golgi-associated PDZ and coiled-coil motif containing	11.0	Publications, Functions
37	IL9R	interleukin 9 receptor	11.0	Publications
38	REG3A	regenerating islet-derived 3 alpha	11.0	Publications
39	CLCA1	chloride channel accessory 1	11.0	Publications, Functions
40	OMP	olfactory marker protein	11.0	Publications
41	SPINK1	serine peptidase inhibitor, Kazal type 1	11.0	Publications
42	TREH	trehalase (brush-border membrane glycoprotein)	11.0	Publications
43	PNLIP	pancreatic lipase	11.0	Publications
44	PRSS8	protease, serine, 8	11.0	Publications
45	CLC	Charcot-Leyden crystal protein	11.0
46	CLCN2	chloride channel, voltage-sensitive 2	11.0	Publications
47	DEFB103B	defensin, beta 103B	11.0	Publications
48	GUCA2A	guanylate cyclase activator 2A (guanylin)	11.0	Publications
49	GUCA2B	guanylate cyclase activator 2B (uroguanylin)	11.0
50	ABCB6	ATP-binding cassette, sub-family B (MDR/TAP), member 6	11.0

Pathways related to cystic fibrosis according to GeneDecks:

(show top 50)    (show all 147)

id	Pathway	Score	Top Affiliating Genes
1	Antioxidant Action of Vitamin-C36	13.6	MYO6, BMP6, CHUK, BAX, JUN, CD4
2	Rho Family GTPases36	13.2	WASL, LMTK2, BMP6, RAF1, BAX, MSN
3	Tec Kinases Signaling36	12.9	BMP6, BAX, CD4, CD80, ITGAM, ITGAV
4	Chemokine Signaling36	12.8	JUN, ARRB2, IL8, MAPK8, MAPK3, MAPK14
5	cAMP Pathway36	12.8	PDE3A, BMP6, RAF1, CLCA2, CLCA4, CLCN3
6	Toll-like receptor signaling pathway20	12.6	PIK3CG, CHUK, LBP, JUN, CD14, CD80
7	ERK Signaling36	12.6	LMTK2, BMP6, RAF1, CHUK, ATF1, CD80
8	p38 Signaling36	12.5	LMTK2, BMP6, BAX, CD4, CD80, CDC25C
9	Chemokine signaling pathway20	12.5	PIK3CG, WASL, RAF1, CHUK, ARRB2, IL8
10	Phagosome20	12.5	SFTPA1, SFTPD, SEC61A1, SEC61B, VAMP3, RAB5A
11	Activation of cAMP-Dependent PKA36	12.4	PDE3A, LMTK2, BMP6, RAF1, CLCA2, CLCA4
12	Glucocorticoid Receptor Signaling36	12.4	BMP6, BAX, JUN, CD4, CD80, IL10
13	ILK Signaling36	12.3	LMTK2, MYO6, ATF6, ATF1, JUN, CDH8
14	MIF Regulation of Innate Immune Cells36	12.2	BMP6, BAX, JUN, CD14, CD80, MIF
15	Bacterial infections in CF airways41	12.2	SFTPA1, SFTPD, CHUK, MUC1, LBP, CFTR
16	Molecular Mechanisms of Cancer36	12.2	PIK3CG, BRCA1, LMTK2, BMP6, RAF1, BAX
17	all-trans-Retinoic Acid Signaling in Brain36	12.2	BMP6, BAX, CD80, IL10, IL13, IL18
18	TGF-Beta Pathway36	12.1	BMP6, BAX, JUN, CD4, CD80, AREG
19	Pertussis20	12.1	SFTPA1, JUN, CD14, ITGAM, IL10, IL1A
20	STAT3 Pathway36	12.1	BMP6, RAF1, BAX, CD4, CD80, IL10
21	Regulation of CFTR activity (norm and CF)41	12.0	S100A10, CFTR, FLNA, ANXA5, ANXA2, GNAI1
22	Immune response_Bacterial infections in normal airways41	12.0	CHUK, LBP, CFTR, CD14, IL1B, IL1R1
23	Leishmaniasis20	11.9	JUN, ITGAM, FCGR2A, IL10, IL1A, IL1B
24	Akt Signaling36	11.8	PIK3CG, LMTK2, RAF1, CHUK, CD4, CD80
25	NF-KappaB (p50-p65) Pathway36	11.8	BMP6, CHUK, UBC, AREG, FGF2, FGF7
26	MIF Mediated Glucocorticoid Regulation36	11.8	BMP6, BAX, CD14, CD80, MIF, IL10
27	Influenza A20	11.8	PIK3CG, VDAC1, RAF1, FURIN, JUN, IL18
28	Immune response IL-1 signaling pathway10	11.7	CHUK, JUN, IL1A, IL1B, IL1R1, IL6
29	MAPK Family Pathway36	11.7	BMP6, RAF1, CHUK, BAX, JUN, CD4
30	Immune response_IL-1 signaling pathway41	11.7	CHUK, UBC, JUN, IL1A, IL1B, IL1R1
31	PGC1Alpha Pathway36	11.7	BMP6, BAX, CD4, CD80, IL13, IL18
32	Chagas disease (American trypanosomiasis)20	11.7	PIK3CG, CHUK, JUN, IL10, IL1B, IL2
33	RAR-Gamma-RXR-Alpha Degradation36	11.6	UBC, CD80, IL2, IL3, IL9, MAPK14
34	Immune response MIF in innate immunity response10	11.6	CHUK, LBP, JUN, CD14, MIF, IL1B
35	Immune response_MIF in innate immunity response41	11.6	CHUK, LBP, JUN, CD14, MIF, IL1B
36	Osteoclast differentiation20	11.5	PIK3CG, CHUK, JUN, FCGR2A, IL1A, IL1B
37	Cytokine production by Th17 cells in CF41	11.5	CHUK, LBP, CFTR, CD14, IL17A, IL17F
38	NOD-like receptor signaling pathway20	11.4	CHUK, IL18, IL1B, IL6, IL8, MAPK8
39	Rheumatoid arthritis20	11.3	VEGFA, ATP6AP1, JUN, CD80, IL17A, IL18
40	IL-6 Pathway36	11.3	BMP6, RAF1, BAX, CD80, IL10, IL18
41	Endothelin-1 Signaling Pathway36	11.3	LMTK2, BMP6, RAF1, CD80, IL10, IL13
42	JAK-STAT Pathway36	11.2	BRCA1, LMTK2, BMP6, RAF1, UBC, BAX
43	NFAT Signaling and Lymphocyte Interactions36	11.0	BMP6, BAX, CD4, CD80, IL13, IL1A
44	Transcription_Role of VDR in regulation of genes involved in osteoporosis41	11.0	VDR, BGLAP, JUN, IL1A, IL1B, IL6
45	Selected targets of GCR-alpha10	11.0	SCNN1A, LEP, CHAT, INS, IL1B, IL4
46	Transcription Role of VDR in regulation of genes involved in osteoporosis10	10.7	VDR, BGLAP, JUN, IL1A, IL1B, IL6
47	Immune response MIF-mediated glucocorticoid regulation10	10.4	CHUK, JUN, MIF, IL2, IL6, IL8
48	Immune response_MIF-mediated glucocorticoid regulation41	10.4	CHUK, JUN, MIF, IL2, IL6, IL8
49	Pathways in cancer20	10.2	PIK3CG, VEGFA, RAD51, CHUK, BAX, JUN
50	Development_VEGF signaling via VEGFR2 - generic cascades41	9.7	VEGFA, RAF1, JUN, ITGAV, MAPK3, MAPK1

Compounds related to cystic fibrosis according to GeneDecks:

(show top 50)    (show all 603)

id	Compound	Score	Top Affiliating Genes
1	arginine32	20.4	PDE4A, RECK, REN, SGK1, VWF, SFTPB
2	glutamate32	20.1	SGK1, PCNA, GC, VIP, VDAC1, VDR
3	estrogen32	19.2	VWF, PCNA, GC, RBP4, SERPINC1, SERPINA1
4	lactate32	17.4	RECK, VWF, SFTPD, PCNA, BTD, RBP4
5	phospholipid32	17.1	PDE4A, VWF, SFTPB, SFTPC, SFTPD, RBP4
6	polysaccharide32	16.4	RECK, SFTPD, SELL, SELP, SERPINE2, SERPINC1
7	ethanol32 34 9 18 9	20.4	SFTPC, GCLC, SERPINA1, BPI, BCHE, SCT
8	fibrinogen32	16.3	RECK, SFTPB, SFTPC, GC, SELP, SERPINC1
9	fatty acid32	16.0	PI3, RECK, SFTPB, SFTPC, GC, BTD
10	iron32 18	16.5	RECK, RBP4, SERPINA1, SERPINA3, NAGLU, LPO
11	glutamine32	15.4	PIK3CG, PDE4A, VWF, SFTPD, PCNA, VIP
12	nmda32 42	15.5	PDE4A, LNX1, PARK2, BCHE, LEP, CHAT
13	lysine32	14.4	RECK, SFTPC, BTD, SERPINA1, BRCA1, VDAC1
14	urea32 9 18 9	17.4	GC, SERPINA3, NAGLU, BPI, RAD51, PAH
15	actinomycin d32	14.1	PIK3CG, PDE4A, SGK1, SFTPB, SFTPC, SERPINE2
16	isoproterenol32 9 9	15.3	PDE3A, PDE4A, SELL, RAF1, KCNQ1, SCT
17	sialic acid32	13.2	RECK, RBP4, SELL, SERPINA3, NAGLU, LPO
18	cytochalasin d32 42	13.8	PIK3CG, VWF, SELL, LPL, RAF1, CFTR
19	sodium nitroprusside32	12.7	PDE4A, REN, VWF, PCNA, VIP, BMP6
20	salbutamol32 34 9 18 9	16.5	PDE4A, VIP, CD79A, IL10, IL13, IL18
21	ly29400232	12.3	PIK3CG, PI3, SGK1, VEGFA, SCD, LEP
22	acth32	12.2	REN, SERPINA1, VIP, SCT, LEP, MUC1
23	chelerythrine32	12.1	PIK3CG, CDKN3, FN1, MAPK8, MAPK14, MAPK10
24	neomycin32	12.0	GUSB, IL13, IL1A, IL3, MAPK3, CCK
25	rapamycin32 42	12.9	PIK3CG, SGK1, SELL, VEGFA, RAF1, CD80
26	prostaglandin f2alpha32	11.9	PDE4A, SFTPB, VIP, LPL, RAF1, IL1A
27	quercetin32 42 9 18 9	15.9	PIK3CG, LPO, RAF1, CHUK, UGT1A1, BAX
28	2-deoxyglucose32	11.9	PIK3CG, XBP1, INS, ANXA5, MAPK10, IGF2
29	pentoxifylline32 9 9	13.8	PDE4A, SELL, SELP, NAGLU, BMP6, LBR
30	glycosaminoglycan32	11.7	SERPINE2, SERPINC1, NAGLU, CD36, MMP8, GUSB
31	haloperidol32 34 9 9	14.7	REN, VIP, SCD, CHAT, IL1R1, IL2RB
32	carnitine32	11.6	LPL, CHAT, CD79A, INS, IGF2, TXN
33	daunorubicin32 9 9	13.6	LPO, RAF1, MVP, BAX, XDH, MPO
34	5 aminosalicylic acid32	11.5	NAGLU, NAT1, NAT2, XDH, MPO, IL10
35	groalpha32	11.4	IL10, IL13, IL17A, IL18, IL1A, IL1B
36	alpha lipoic acid32	11.3	BTD, SERPINA1, CHAT, BAX, MMP9, INS
37	gsno32	11.1	SELP, XDH, CFTR, JUN, CDKN3, FN1
38	phenytoin32 34 9 9	14.1	BMP6, UGT1A1, CD79A, CD40LG, ALB, ALPP
39	nicotine32 34 9 9	14.1	SELL, UGT1A1, ANXA1, GPT, CYP1A2, CYP2C19
40	diclofenac32 34 9 9	14.0	SELL, SERPINC1, NAGLU, SCN4A, UGT1A1, IL13
41	ursodeoxycholic acid32 9 18 9	13.9	PCNA, BMP6, SCT, LBR, CD40LG, MLN
42	s-nitroso-n-acetylpenicillamine32	10.9	XDH, FN1, IL1B, IL8, ANXA5, MAPK14
43	5fluorouracil32	10.8	PIK3CG, PCNA, BRCA1, VEGFA, VDR, CHUK
44	raloxifene32 9 9	12.7	VWF, SERPINC1, VDR, BGLAP, UGT1A1, JUN
45	telmisartan32 9 9	12.6	REN, LEP, INS, ALB, CYP2C19, PPARG
46	methimazole32 9 9	12.6	VWF, PCNA, SELL, LPO, BGLAP, GSR
47	protamine32	10.3	SELL, SELP, SERPINC1, LPL, INS, FGF2
48	captopril32 42 9 9	13.3	REN, VIP, CD79A, MMP2, IL1A, F3
49	pioglitazone32 9 9	12.1	RBP4, VEGFA, LPL, LEP, JUN, CD40
50	geldanamycin32 9 9	10.9	PIK3CG, VEGFA, RAF1, PARK2, JUN, CDC25C

Cellular components related to cystic fibrosis according to GeneDecks:

(show all 14)

id	Name	GO ID	Score	Top Affiliating Genes
1	plasma membrane	GO:005886	25.8	PIK3CG, RECK, SGK1, WASL, GCA, SELL
2	extracellular region	GO:005576	19.9	REG1A, VWF, SFTPA1, SFTPD, GC, SERPINE2
3	integral to plasma membrane	GO:005887	18.3	SELL, SELP, BPI, CLCA2, CLCA4, CLCA1
4	extracellular space	GO:005615	16.7	REG3A, REN, SFTPA1, SFTPB, SFTPC, SFTPD
5	cytosol	GO:005829	16.4	PIK3CG, PDE3A, PDE4A, WASL, GCLC, GC
6	apical plasma membrane	GO:016324	15.6	VAMP3, CLCA4, SCNN1A, SCNN1B, SCNN1G, UMOD
7	basolateral plasma membrane	GO:016323	13.8	BEST1, KCNQ1, UMOD, P2RY1, P2RY2, P2RY4
8	perinuclear region of cytoplasm	GO:048471	13.1	PDE4A, GC, LMTK2, MYO6, RAD51, PARK2
9	membrane	GO:016020	13.1	PIK3CG, PDE4A, RECK, REN, VEGFA, VDAC1
10	cell surface	GO:009986	12.2	VEGFA, LPL, CLCN3, MSN, FURIN, CD36
11	Golgi lumen	GO:005796	12.2	MUC1, MUC2, MUC3A, MUC3B, MUC4, MUC5AC
12	external side of plasma membrane	GO:009897	11.4	VWF, SELL, SELP, SCNN1A, SCNN1B, SCNN1G
13	membrane raft	GO:045121	11.1	RAB5A, UMOD, FURIN, CD79A, CD36, CD14
14	platelet alpha granule lumen	GO:031093	9.9	VWF, SERPINA1, VEGFA, FN1, IGF1, F8

Biological processes related to cystic fibrosis according to GeneDecks:

(show all 40)

id	Name	GO ID	Score	Top Affiliating Genes
1	small molecule metabolic process	GO:044281	18.4	PIK3CG, WASL, GCLC, GC, NAGLU, NAT1
2	transport	GO:006810	15.3	CLCA2, CLCA4, CLCN3, CLCN4, CLCA1, CLCA3P
3	inflammatory response	GO:006954	15.0	PIK3CG, SELP, SERPINA3, BMP6, CHUK, S100A12
4	transmembrane transport	GO:055085	14.9	RAB11A, CFTR, ATP2A3, ATP2A2, ATP1B1, FTL
5	immune response	GO:006955	14.5	BPI, BMP6, CLNK, CHUK, XBP1, CFP
6	signal transduction	GO:007165	14.3	PDE4A, VDR, RASSF6, RAF1, CLIC1, SCGB1A1
7	innate immune response	GO:045087	13.9	PIK3CG, SFTPD, BPIFA1, CHUK, UBC, LBP
8	response to drug	GO:042493	13.7	PDE3A, PDE4A, REN, LPL, BGLAP, BCHE
9	cell-cell signaling	GO:007267	13.0	S100A9, CEACAM6, CD80, MLN, INS, IL10
10	G-protein coupled receptor signaling pathway	GO:007186	12.9	PIK3CG, VIP, SCTR, P2RY1, FPR1, MLN
11	excretion	GO:007588	12.8	CLCN5, KCNJ1, SCTR, SCNN1A, SCNN1B, SCNN1G
12	defense response to bacterium	GO:042742	12.6	BPI, S100A12, CFP, IL10, CCL20, IFNG
13	aging	GO:007568	12.6	VDR, P2RY1, P2RY2, ATP6AP1, FURIN, JUN
14	response to lipopolysaccharide	GO:032496	12.4	SFTPB, SFTPC, SELP, SERPINA1, UGT1A1, LBP
15	response to glucocorticoid stimulus	GO:051384	12.1	SFTPB, SFTPC, BGLAP, BCHE, LCAT, IL10
16	chemotaxis	GO:006935	12.1	S100A8, S100A9, FPR1, FGF2, IL4, MAPK14
17	positive regulation of apoptotic process	GO:043065	11.8	BAX, MMP9, IL1B, FASLG, FAS, ANXA5
18	blood coagulation	GO:007596	11.8	PIK3CG, PDE3A, VWF, SELL, SELP, SERPINE2
19	cellular response to mechanical stimulus	GO:071260	11.8	SFTPB, SFTPC, CD40, IL13, IL18, IL1B
20	negative regulation of growth of symbiont in host	GO:044130	11.7	LBP, CD36, IL10, MBL2, IFNG, CAMP
21	response to ethanol	GO:045471	11.7	RBP4, BGLAP, CHAT, LCT, S100A8, S100A9
22	positive regulation of I-kappaB kinase/NF-kappaB cascade	GO:043123	11.6	PARK2, CHUK, UBC, S100A12, CD36, CD40
23	lipopolysaccharide-mediated signaling pathway	GO:031663	11.4	LBP, IL18, IL1B, MAPK3, MAPK14, CCL2
24	cell surface receptor signaling pathway	GO:007166	11.3	P2RY1, JMJD6, CD36, CD14, CD4, MIF
25	MyD88-dependent toll-like receptor signaling pathway	GO:002755	11.3	CHUK, UBC, ATF1, JUN, MAPK8, MAPK3
26	positive regulation of vasodilation	GO:045909	11.3	INS, F2RL1, CALCA, HMOX1, NOS1, NOS3
27	anti-apoptosis	GO:006916	11.2	GCLC, MUC1, UBC, MPO, CD40LG, GSTP1
28	positive regulation of smooth muscle cell proliferation	GO:048661	11.2	JUN, IL13, IL6, IL6R, CCL5, IGF1
29	response to hypoxia	GO:001666	11.2	SFTPB, SERPINA1, VEGFA, RAF1, SCNN1B, SCNN1G
30	positive regulation of transcription from RNA polymerase II promoter	GO:045944	11.1	BRCA1, VEGFA, VDR, MYO6, BMP6, UBC
31	Toll signaling pathway	GO:008063	11.1	CHUK, UBC, LBP, ATF1, JUN, CD14
32	positive regulation of nitric oxide biosynthetic process	GO:045429	11.1	INS, IL1B, IFNG, ICAM1, HSP90AA1, HSP90AB1
33	positive regulation of cell proliferation	GO:008284	10.9	REG1A, VIP, VEGFA, LEP, ITGAV, INS
34	neutrophil chemotaxis	GO:030593	10.9	PIK3CG, IL1B, IL8, CCL2, CCL3, IFNG
35	positive regulation of chemokine production	GO:032722	10.8	LBP, P2RY2, IL18, IL6, IL6R, TNF
36	negative regulation of apoptotic process	GO:043066	10.8	PIK3CG, PDE3A, VEGFA, RAF1, LEP, ITGAV
37	positive regulation of peptidyl-tyrosine phosphorylation	GO:050731	10.6	VEGFA, CD36, CD4, CD80, MIF, INS
38	platelet activation	GO:030168	10.5	PIK3CG, VWF, SELP, SERPINA1, VEGFA, RAF1
39	regulation of blood pressure	GO:008217	10.4	REN, SGK1, LEP, CALCA, HMOX1, PTGS2
40	platelet degranulation	GO:002576	10.3	VWF, SELP, SERPINA1, VEGFA, CD36, FN1

Molecular functions related to cystic fibrosis according to GeneDecks:

(show all 9)

id	Name	GO ID	Score	Top Affiliating Genes
1	protein binding	GO:005515	27.4	SLC25A17, ADA, ADRB3, ADRB1, ADIPOQ, HDAC2
2	receptor binding	GO:005102	13.3	GJA1, SLC9A3R2, SLC9A3R1, KNG1, C3, ADCYAP1
3	protein homodimerization activity	GO:042803	13.2	VWF, SFTPC, GCA, IL6R, ENPP1, ANXA1
4	chemokine activity	GO:008009	11.8	PF4V1, CXCL1, CXCL10, CXCL2, CXCL3, CXCL6
5	heme binding	GO:020037	11.6	CYP2C19, CYP2E1, CYP2D6, SRC, NOS1, NOS2
6	enzyme binding	GO:019899	11.3	TGFB1, HIF1A, A2M, NFKBIA, PLAUR, PPARG
7	heparin binding	GO:008201	11.2	CCL5, CTSG, PF4, PF4V1, LTF, ABP1
8	cytokine activity	GO:005125	10.2	VEGFA, BMP6, CD40LG, MIF, FGF2, IL10
9	hormone activity	GO:005179	10.0	EDN1, EDN2, EDN3, PRL, NPPA, NPPB