-
1
-
-
0024423668
-
Identification of the cystic fibrosis gene: Genetic analysis
-
Kerem B, Rommens JM, Buchanan JA, et al. Identification of the cystic fibrosis gene: Genetic analysis. Science 1989; 245: 1073-80
-
(1989)
Science
, vol.245
, pp. 1073-1080
-
-
Kerem, B.1
Rommens, J.M.2
Buchanan, J.A.3
-
2
-
-
0024424270
-
Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
-
Riordan JR, Rommens JM, Kerem B, et al. Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA. Science 1989; 245: 1066-73
-
(1989)
Science
, vol.245
, pp. 1066-1073
-
-
Riordan, J.R.1
Rommens, J.M.2
Kerem, B.3
-
3
-
-
0024453308
-
Identification of the cystic fibrosis gene: Chromosome walking and jumping
-
Rommens JM, Iannuzzi MC, Kerem B, et al. Identification of the cystic fibrosis gene: Chromosome walking and jumping. Science 1989; 245: 1059-65
-
(1989)
Science
, vol.245
, pp. 1059-1065
-
-
Rommens, J.M.1
Iannuzzi, M.C.2
Kerem, B.3
-
4
-
-
76449091330
-
-
American Lung Association [Last accessed 1 January 2015]
-
American Lung Association. In: State of Lung Disease in Diverse Communities 2010. 2010; 41-4. Available from: http://www.lung.org/assets/documents/publications/lung-disease-data/solddc-2010.pdf [Last accessed 1 January 2015]
-
(2010)
State of Lung Disease in Diverse Communities 2010
, pp. 41-44
-
-
-
5
-
-
51349100398
-
The prevalence of cystic fibrosis in the European
-
Farrell PM. The prevalence of cystic fibrosis in the European Union. J Cyst Fibros 2008; 7: 450-3
-
(2008)
Union J Cyst Fibros
, vol.7
, pp. 450-453
-
-
Farrell, P.M.1
-
6
-
-
0025787393
-
In vivo cellspecific expression of the cystic fibrosis transmembrane conductance regulator
-
Trezise AE, Buchwald M. In vivo cellspecific expression of the cystic fibrosis transmembrane conductance regulator. Nature 1991; 353: 434-7
-
(1991)
Nature
, vol.353
, pp. 434-437
-
-
Trezise, A.E.1
Buchwald, M.2
-
7
-
-
0032616420
-
The cystic fibrosis transmembrane conductance regulator and its function in epithelial transport
-
Kunzelmann K. The cystic fibrosis transmembrane conductance regulator and its function in epithelial transport. Rev Physiol Biochem Pharmacol 1999; 137: 1-70
-
(1999)
Rev Physiol Biochem Pharmacol
, vol.137
, pp. 1-70
-
-
Kunzelmann, K.1
-
8
-
-
50649123290
-
CFTR function and prospects for therapy
-
Riordan JR. CFTR function and prospects for therapy. Ann Rev Biochem 2008; 77: 701-26
-
(2008)
Ann Rev Biochem
, vol.77
, pp. 701-726
-
-
Riordan, J.R.1
-
9
-
-
0025942250
-
Immunocytochemical localization of the cystic fibrosis gene product CFTR
-
Crawford I, Maloney PC, Zeitlin PL, et al. Immunocytochemical localization of the cystic fibrosis gene product CFTR. Proc Natl Acad Sci U S A 1991; 88: 9262-6
-
(1991)
Proc Natl Acad Sci U S A
, vol.88
, pp. 9262-9266
-
-
Crawford, I.1
Maloney, P.C.2
Zeitlin, P.L.3
-
12
-
-
0344876536
-
CFTR pharmacology and its role in intestinal fluid secretion
-
Thiagarajah JR, Verkman AS. CFTR pharmacology and its role in intestinal fluid secretion. Curr Opin Pharmacol 2003; 3: 594-9
-
(2003)
Curr Opin Pharmacol
, vol.3
, pp. 594-599
-
-
Thiagarajah, J.R.1
Verkman, A.S.2
-
14
-
-
0027162649
-
Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis
-
Welsh MJ, Smith AE. Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. Cell 1993; 73: 1251-4.
-
(1993)
Cell
, vol.73
, pp. 1251-1254
-
-
Welsh, M.J.1
Smith, A.E.2
-
16
-
-
79958746554
-
Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking, and opportunities for mutation-specific treatment
-
Rogan MP, Stoltz DA, Hornick DB. Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking, and opportunities for mutation-specific treatment. Chest 2011; 139: 1480-90.
-
(2011)
Chest
, vol.139
, pp. 1480-1490
-
-
Rogan, M.P.1
Stoltz, D.A.2
Hornick, D.B.3
-
17
-
-
33744977423
-
Cystic fibrosis screening in assisted reproduction
-
Gazvani R, Lewis-Jones I. Cystic fibrosis screening in assisted reproduction. Curr Opin Obstet Gynecol 2006; 18(3): 268-72
-
(2006)
Curr Opin Obstet Gynecol
, vol.18
, Issue.3
, pp. 268-272
-
-
Gazvani, R.1
Lewis-Jones, I.2
-
18
-
-
0026753172
-
Abnormal localization of cystic fibrosis transmembrane conductance regulator in primary cultures of cystic fibrosis airway epithelia
-
Denning GM, Ostedgaard LS, Welsh MJ. Abnormal localization of cystic fibrosis transmembrane conductance regulator in primary cultures of cystic fibrosis airway epithelia. J Cell Biol 1992; 118: 551-9
-
(1992)
J Cell Biol
, vol.118
, pp. 551-559
-
-
Denning, G.M.1
Ostedgaard, L.S.2
Welsh, M.J.3
-
19
-
-
0026325533
-
Altered chloride ion channel kinetics associated with the delta F508 cystic fibrosis mutation
-
Dalemans W, Barbry P, Champigny G, et al. Altered chloride ion channel kinetics associated with the delta F508 cystic fibrosis mutation. Nature 1991; 354: 526-8
-
(1991)
Nature
, vol.354
, pp. 526-528
-
-
Dalemans, W.1
Barbry, P.2
Champigny, G.3
-
20
-
-
0026907529
-
Mislocalization of delta F508 CFTR in cystic fibrosis sweat gland
-
Kartner N, Augustinas O, Jensen TJ, et al. Mislocalization of delta F508 CFTR in cystic fibrosis sweat gland. Nat Genet 1992; 1: 321-7
-
(1992)
Nat Genet
, vol.1
, pp. 321-327
-
-
Kartner, N.1
Augustinas, O.2
Jensen, T.J.3
-
21
-
-
0026688929
-
Immunocytochemical analysis reveals differences between the subcellular localization of normal and delta Phe508 recombinant cystic fibrosis transmembrane conductance regulator
-
Dalemans W, Hinnrasky J, Slos P, et al. Immunocytochemical analysis reveals differences between the subcellular localization of normal and delta Phe508 recombinant cystic fibrosis transmembrane conductance regulator. Exp Cell Res 1992; 201: 235-40
-
(1992)
Exp Cell Res
, vol.201
, pp. 235-240
-
-
Dalemans, W.1
Hinnrasky, J.2
Slos, P.3
-
22
-
-
84895067117
-
Pulmonary complications of cystic fibrosis
-
Ng MY, Flight W, Smith E. Pulmonary complications of cystic fibrosis. Clin Radiol 2014; 69: e153-62
-
(2014)
Clin Radiol
, vol.69
, pp. e153-e162
-
-
Ng, M.Y.1
Flight, W.2
Smith, E.3
-
23
-
-
84900315796
-
Lung inflammation in cystic fibrosis: Pathogenesis and novel therapies
-
Dhooghe B, Noe?l S, Huaux F, Leal T. Lung inflammation in cystic fibrosis: pathogenesis and novel therapies. Clin Biochem 2014; 47: 539-46
-
(2014)
Clin Biochem
, vol.47
, pp. 539-546
-
-
Dhooghe, B.1
Noel, S.2
Huaux, F.3
Leal, T.4
-
24
-
-
84887728119
-
The future of antimicrobial therapy in the era of antibiotic resistance in cystic fibrosis pulmonary infection
-
McCaughey G, Gilpin D, Elborn J, Tunney MM. The future of antimicrobial therapy in the era of antibiotic resistance in cystic fibrosis pulmonary infection. Expert Rev Respir Med 2013; 7: 385-96
-
(2013)
Expert Rev Respir Med
, vol.7
, pp. 385-396
-
-
McCaughey, G.1
Gilpin, D.2
Elborn, J.3
Tunney, M.M.4
-
25
-
-
66849089934
-
Cystic fibrosis: Pathogenesis and future treatment strategies
-
Ratjen FA. Cystic fibrosis: pathogenesis and future treatment strategies. Respir Care 2009; 54: 595-605
-
(2009)
Respir Care
, vol.54
, pp. 595-605
-
-
Ratjen, F.A.1
-
27
-
-
84880046935
-
Early lung disease in cystic fibrosis
-
Grasemann H, Ratjen F. Early lung disease in cystic fibrosis. Lancet Respir Med 2013; 1: 148-57
-
(2013)
Lancet Respir Med
, vol.1
, pp. 148-157
-
-
Grasemann, H.1
Ratjen, F.2
-
29
-
-
84887017757
-
Antibiotics for treatment and prevention of exacerbations of chronic obstructive pulmonary disease
-
Wilson R, Sethi S, Anzueto A, Miravitlles M. Antibiotics for treatment and prevention of exacerbations of chronic obstructive pulmonary disease. J Infect 2013; 67: 497-515
-
(2013)
J Infect
, vol.67
, pp. 497-515
-
-
Wilson, R.1
Sethi, S.2
Anzueto, A.3
Miravitlles, M.4
-
30
-
-
84901850614
-
Cystic fibrosis: A review of pathophysiology and current treatment recommendations
-
Peters S. Cystic fibrosis: a review of pathophysiology and current treatment recommendations. S D Med 2014; 67: 148-51, 153
-
(2014)
S D Med
, vol.67
, pp. 148-153
-
-
Peters, S.1
-
31
-
-
0037460729
-
Cystic fibrosis
-
Ratjen F, Döring G. Cystic fibrosis. Lancet 2003; 361: 681-9
-
(2003)
Lancet
, vol.361
, pp. 681-689
-
-
Ratjen, F.1
Döring, G.2
-
32
-
-
34848821636
-
New pulmonary therapies for cystic fibrosis
-
Ratjen F. New pulmonary therapies for cystic fibrosis. Curr Opin Pulm Med 2007; 13: 541-6
-
(2007)
Curr Opin Pulm Med
, vol.13
, pp. 541-546
-
-
Ratjen, F.1
-
33
-
-
33750302487
-
Treatment of early Pseudomonas aeruginosa infection in patients with cystic fibrosis
-
Ratjen F. Treatment of early Pseudomonas aeruginosa infection in patients with cystic fibrosis. Curr Opin Pulm Med 2006; 12: 428-32
-
(2006)
Curr Opin Pulm Med
, vol.12
, pp. 428-432
-
-
Ratjen, F.1
-
34
-
-
48249099755
-
Cystic fibrosis, vectormediated gene therapy, and relevance of toll-like receptors: A review of problems, progress, and possibilities
-
Atkinson TJ. Cystic fibrosis, vectormediated gene therapy, and relevance of toll-like receptors: a review of problems, progress, and possibilities. Curr Gene Ther 2008; 8: 201-7
-
(2008)
Curr Gene Ther
, vol.8
, pp. 201-207
-
-
Atkinson, T.J.1
-
36
-
-
84884802285
-
Moving forward: Cystic fibrosis gene therapy
-
Griesenbach U, Alton EW. Moving forward: cystic fibrosis gene therapy. Hum Mol Genet 2013; 22: R52-8.
-
(2013)
Hum Mol Genet
, vol.22
, pp. R52-R58
-
-
Griesenbach, U.1
Alton, E.W.2
-
37
-
-
84875175557
-
Gene therapy in cystic fibrosis
-
Prickett M, Jain M. Gene therapy in cystic fibrosis. Transl Res 2013; 161: 255-64
-
(2013)
Transl Res
, vol.161
, pp. 255-264
-
-
Prickett, M.1
Jain, M.2
-
38
-
-
0026781952
-
Processing of mutant cytsic fibrosis transmembrane conductance regulator is temperature-sensitive
-
Denning G, Anderson M, Amara J, et al. Processing of mutant cytsic fibrosis transmembrane conductance regulator is temperature-sensitive. Nature 1992; 358: 761-3.
-
(1992)
Nature
, vol.358
, pp. 761-763
-
-
Denning, G.1
Anderson, M.2
Amara, J.3
-
39
-
-
0030042386
-
Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation
-
Sato S, Ward CL, Krouse ME, et al. Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation. J Biol Chem 1996; 271: 635-8.
-
(1996)
J Biol Chem
, vol.271
, pp. 635-638
-
-
Sato, S.1
Ward, C.L.2
Krouse, M.E.3
-
40
-
-
0030154620
-
Chemical chaperones correct the mutant phenotype of the DF508 cystic fibrosis transmembrane conductance regulator protein
-
Brown CR, Hong-Brown LQ, Biwersi J, et al. Chemical chaperones correct the mutant phenotype of the DF508 cystic fibrosis transmembrane conductance regulator protein. Cell Stress Chaperones 1996; 1: 117-25
-
(1996)
Cell Stress Chaperones
, vol.1
, pp. 117-125
-
-
Brown, C.R.1
Hong-Brown, L.Q.2
Biwersi, J.3
-
41
-
-
0031889082
-
A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in F508delhomozygous cystic fibrosis patients: Partial restoration of nasal epithelial CFTR function
-
Rubenstein RC, Zeitlin PL. A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in F508delhomozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function. Am J Respir Crit Care Med 1998; 157: 484-90
-
(1998)
Am J Respir Crit Care Med
, vol.157
, pp. 484-490
-
-
Rubenstein, R.C.1
Zeitlin, P.L.2
-
42
-
-
0030809817
-
In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR
-
Rubenstein RC, Egan ME, Zeitlin PL. In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR. J Clin Invest 1997; 100: 2457-65
-
(1997)
J Clin Invest
, vol.100
, pp. 2457-2465
-
-
Rubenstein, R.C.1
Egan, M.E.2
Zeitlin, P.L.3
-
43
-
-
84879000844
-
Cystic fibrosis transmembrane regulator correctors and potentiators
-
Rowe SM, Verkman AS. Cystic fibrosis transmembrane regulator correctors and potentiators. Cold Spring Harb Perspect Med 2013; 3(7): pii: a009761
-
(2013)
Cold Spring Harb Perspect Med
, vol.3
, Issue.7
-
-
Rowe, S.M.1
Verkman, A.S.2
-
44
-
-
84864757410
-
Cystic fibrosis transmembrane conductance regulator-modifying medications: The future of cystic fibrosis treatment
-
Pettit RS. Cystic fibrosis transmembrane conductance regulator-modifying medications: the future of cystic fibrosis treatment. Ann Pharmacother 2012; 46: 1065-75
-
(2012)
Ann Pharmacother
, vol.46
, pp. 1065-1075
-
-
Pettit, R.S.1
-
45
-
-
84897556769
-
Ivacaftor: A review of its use in patients with cystic fibrosis
-
Deeks ED. Ivacaftor: a review of its use in patients with cystic fibrosis. Drugs 2013; 73: 1595-604
-
(2013)
Drugs
, vol.73
, pp. 1595-1604
-
-
Deeks, E.D.1
-
46
-
-
84877932776
-
Repairing mutated proteins-development of small molecules targeting defects in the cystic fibrosis transmembrane conductance regulator
-
Merk D, Schubert-Zsilavecz M. Repairing mutated proteins-development of small molecules targeting defects in the cystic fibrosis transmembrane conductance regulator. Expert Opin Drug Discov 2013; 8: 691-708
-
(2013)
Expert Opin Drug Discov
, vol.8
, pp. 691-708
-
-
Merk, D.1
Schubert-Zsilavecz, M.2
-
47
-
-
84856463093
-
Identification and validation of hits from high throughput screens for CFTR modulators
-
Pasyk S, Molinski S, Yu W, et al. Identification and validation of hits from high throughput screens for CFTR modulators. Curr Pharm Des 2012; 18: 628-41
-
(2012)
Curr Pharm des
, vol.18
, pp. 628-641
-
-
Pasyk, S.1
Molinski, S.2
Yu, W.3
-
48
-
-
77249176209
-
Cystic fibrosis transmembrane conductance regulator modulators for personalized drug treatment of cystic fibrosis: Progress to date
-
Becq F. Cystic fibrosis transmembrane conductance regulator modulators for personalized drug treatment of cystic fibrosis: progress to date. Drugs 2010; 70: 241-59
-
(2010)
Drugs
, vol.70
, pp. 241-259
-
-
Becq, F.1
-
51
-
-
81755163563
-
Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809
-
Van Goor F, Hadida S, Grootenhuis PD, et al. Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809. Proc Natl Acad Sci U S A 2011; 108: 18843-8.
-
(2011)
Proc Natl Acad Sci U S A
, vol.108
, pp. 18843-18843
-
-
Van Goor, F.1
Hadida, S.2
Grootenhuis, P.D.3
-
52
-
-
20944442087
-
Phenylglycine and sulfonamide correctors of defective delta F508 and G551D cystic fibrosis transmembrane conductance regulator chloride-channel gating
-
Pedemonte N, Sonawane ND, Taddei A, et al. Phenylglycine and sulfonamide correctors of defective delta F508 and G551D cystic fibrosis transmembrane conductance regulator chloride-channel gating. Mol Pharmacol 2005; 67: 1797-807
-
(2005)
Mol Pharmacol
, vol.67
, pp. 1797-1807
-
-
Pedemonte, N.1
Sonawane, N.D.2
Taddei, A.3
-
53
-
-
0042317111
-
Nanomolar affinity small molecule correctors of defective Delta F508-CFTR chloride channel gating
-
Yang H, Shelat AA, Guy RK, et al. Nanomolar affinity small molecule correctors of defective Delta F508-CFTR chloride channel gating. J Biol Chem 2003; 278: 35079-85
-
(2003)
J Biol Chem
, vol.278
, pp. 35079-35085
-
-
Yang, H.1
Shelat, A.A.2
Guy, R.K.3
-
55
-
-
84940201830
-
-
[Last accessed on 28 January 2015] Vertex Pharmaceuticals. U.S. Food Drug Administration Approves KALYDECO(ivacaftor) for Use in Eight Additional Mutations that Cause Cystic Fibrosis
-
Vertex Pharmaceuticals. U.S. Food and Drug Administration Approves KALYDECO(ivacaftor) for Use in Eight Additional Mutations that Cause Cystic Fibrosis. 2014. Available from: http://investors.vrtx.com/releasedetail. cfm?ReleaseID=827435 [Last accessed on 28 January 2015]
-
(2014)
-
-
-
56
-
-
84884773595
-
VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1
-
Ren HY, Grove DE, De La Rosa O, et al. VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1. Mol Biol Cell 2013; 24: 3016-24
-
(2013)
Mol Biol Cell
, vol.24
, pp. 3016-3024
-
-
Ren, H.Y.1
Grove, D.E.2
De La Rosa, O.3
-
57
-
-
84855202429
-
Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del- CFTR mutation
-
Clancy JP, Rowe SM, Accurso FJ, et al. Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del- CFTR mutation. Thorax 2012; 67: 12-18
-
(2012)
Thorax
, vol.67
, pp. 12-18
-
-
Clancy, J.P.1
Rowe, S.M.2
Accurso, F.J.3
-
58
-
-
84904002908
-
A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: A phase 2 randomised controlled trial
-
Boyle MP, Bell SC, Konstan MW, et al. A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial. Lancet Respir Med 2014; 2: 527-38
-
(2014)
Lancet Respir Med
, vol.2
, pp. 527-538
-
-
Boyle, M.P.1
Bell, S.C.2
Konstan, M.W.3
-
59
-
-
84940210962
-
-
A study of lumacaftor in combination with ivacaftor in cystic fibrosis subjects aged 12 years and older who are homozygous for the F508del-CFTR mutation (TRANSPORT) [Last accessed on 16 March 2015]
-
A study of lumacaftor in combination with ivacaftor in cystic fibrosis subjects aged 12 years and older who are homozygous for the F508del-CFTR mutation (TRANSPORT). Available from: https://clinicaltrials.gov/ct2/show/record/NCT01807949 [Last accessed on 16 March 2015]
-
-
-
-
60
-
-
84940211589
-
-
A Study of Lumacaftor in Combination With Ivacaftor in Cystic Fibrosis Subjects Aged 12 Years and Older Who Are Homozygous for the F508del-CFTR Mutation (TRAFFIC)[Last accessed on 16 March 2015]
-
A Study of Lumacaftor in Combination With Ivacaftor in Cystic Fibrosis Subjects Aged 12 Years and Older Who Are Homozygous for the F508del-CFTR Mutation (TRAFFIC). Available from: https://clinicaltrials.gov/ct2/show/NCT01807923 [Last accessed on 16 March 2015]
-
-
-
-
62
-
-
84907223425
-
Potentiator ivacaftor abrogates pharmacological correction of DF508 CFTR in cystic fibrosis
-
Cholon DM, Quinney NL, Fulcher ML, et al. Potentiator ivacaftor abrogates pharmacological correction of DF508 CFTR in cystic fibrosis. Sci Transl Med 2014; 6(246): 246ra96
-
(2014)
Sci Transl Med
, vol.6
, Issue.246
, pp. 246ra96
-
-
Cholon, D.M.1
Quinney, N.L.2
Fulcher, M.L.3
-
63
-
-
84940203406
-
-
Seeking Alpha [Last accessed on 23 March 2015]
-
Seeking Alpha. Vertex Pharma off on trial data for VX-661. 2015. Available from: http://seekingalpha.com/news/2385656-vertexpharma-off-on-trial-data-for-vxminus-661?auth-param=11sf95: 1ah0949: c0eace6e7379e65e5b921 6347ff7e872&uprof=. 61 [Last accessed on 23 March 2015]
-
(2015)
Vertex Pharma off on Trial Data for VX-661
-
-
-
64
-
-
84903159409
-
Novel picolinamide-based cystic fibrosis transmembrane regulator modulators: Evaluation of WO2013038373, WO2013038376, WO2013038381, WO2013038386 and WO2013038390
-
Norman P. Novel picolinamide-based cystic fibrosis transmembrane regulator modulators: evaluation of WO2013038373, WO2013038376, WO2013038381, WO2013038386 and. WO2013038390. Expert Opin Ther Pat 2014.24(7): 829-37.
-
(2014)
Expert Opin Ther Pat
, vol.24
, Issue.7
, pp. 829-837
-
-
Norman, P.1
-
69
-
-
24644464284
-
Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening
-
Pedemonte N, Lukacs GL, Du K, et al. Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening. J Clin Invest 2005; 115: 2564-71
-
(2005)
J Clin Invest
, vol.115
, pp. 2564-2571
-
-
Pedemonte, N.1
Lukacs, G.L.2
Du, K.3
-
70
-
-
84912000365
-
DF508-CFTR correctors: Synthesis and evaluation of thiazole-tethered imidazolones, oxazoles, oxadiazoles, and thiadiazoles
-
Ye L, Hu B, El-Badri F, et al. DF508-CFTR correctors: Synthesis and evaluation of thiazole-tethered imidazolones, oxazoles, oxadiazoles, and thiadiazoles. Bioorg Med Chem Lett 2014; 24: 5840-4
-
(2014)
Bioorg Med Chem Lett
, vol.24
, pp. 5840-5844
-
-
Ye, L.1
Hu, B.2
El-Badri, F.3
-
71
-
-
84864984030
-
Click-based synthesis of triazolobithiazole DF508-CFTR correctors for cystic fibrosis
-
Donald MB, Rodriguez KX, Shay H, et al. Click-based synthesis of triazolobithiazole DF508-CFTR correctors for cystic fibrosis. Bioorg Med Chem 2012; 20: 5247-53
-
(2012)
Bioorg Med Chem
, vol.20
, pp. 5247-5253
-
-
Donald, M.B.1
Rodriguez, K.X.2
Shay, H.3
-
72
-
-
77952018949
-
Pyrazolylthiazole as DeltaF508-cystic fibrosis transmembrane conductance regulator correctors with improved hydrophilicity compared to bithiazoles
-
Ye L, Knapp JM, Sangwung P, et al. Pyrazolylthiazole as DeltaF508-cystic fibrosis transmembrane conductance regulator correctors with improved hydrophilicity compared to bithiazoles. J Med Chem 2013; 53: 3772-81
-
(2013)
J Med Chem
, vol.53
, pp. 3772-3781
-
-
Ye, L.1
Knapp, J.M.2
Sangwung, P.3
-
73
-
-
53549097399
-
Potent s-cis-locked bithiazole correctors of DeltaF508 cystic fibrosis transmembrane conductance regulator cellular processing for cystic fibrosis therapy
-
Yu GJ, Yoo CL, Yang B, et al. Potent s-cis-locked bithiazole correctors of DeltaF508 cystic fibrosis transmembrane conductance regulator cellular processing for cystic fibrosis therapy. J Med Chem 2008; 51: 6044-54
-
(2008)
J Med Chem
, vol.51
, pp. 6044-6054
-
-
Yu, G.J.1
Yoo, C.L.2
Yang, B.3
-
74
-
-
27144481548
-
Rescue of DeltaF508 and other misprocessed CFTR mutants by a novel quinazoline compound
-
Loo TW, Bartlett MC, Clarke DM. Rescue of DeltaF508 and other misprocessed CFTR mutants by a novel quinazoline compound. Mol Pharm 2005; 2: 407-13
-
(2005)
Mol Pharm
, vol.2
, pp. 407-413
-
-
Loo, T.W.1
Bartlett, M.C.2
Clarke, D.M.3
-
75
-
-
33645530653
-
The chemical chaperone CFcor-325 repairs folding defects in the transmembrane domains of CFTRprocessing mutants
-
Loo TW, Bartlett MC, Wang Y, Clarke DM. The chemical chaperone CFcor-325 repairs folding defects in the transmembrane domains of CFTRprocessing mutants. Biochem J 2006; 395: 537-42
-
(2006)
Biochem J
, vol.395
, pp. 537-542
-
-
Loo, T.W.1
Bartlett, M.C.2
Wang, Y.3
Clarke, D.M.4
-
76
-
-
33744831154
-
Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules
-
Van Goor F, Straley KS, Cao D, et al. Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules. Am J Physiol Lung Cell Mol Physiol 2006; 290: L1117-30
-
(2006)
Am J Physiol Lung Cell Mol Physiol
, vol.290
, pp. L1117-L1130
-
-
Van Goor, F.1
Straley, K.S.2
Cao, D.3
-
77
-
-
29544440095
-
Sulfamoyl-4-oxoquinoline-3- carboxamides: Novel potentiators of defective DeltaF508-cystic fibrosis transmembrane conductance regulator chloride channel gating
-
Suen YF, Robins L, Yang B, et al. Sulfamoyl-4-oxoquinoline-3- carboxamides: novel potentiators of defective DeltaF508-cystic fibrosis transmembrane conductance regulator chloride channel gating. Bioorg Med Chem Lett 2006; 16: 537-40
-
(2006)
Bioorg Med Chem Lett
, vol.16
, pp. 537-540
-
-
Suen, Y.F.1
Robins, L.2
Yang, B.3
-
78
-
-
80053179441
-
Cyanoquinolines with independent corrector and potentiator activities restore DPhe508-cystic fibrosis transmembrane conductance regulator chloride channel function in cystic fibrosis
-
Phuan PW, Yang B, Knapp JM, et al. Cyanoquinolines with independent corrector and potentiator activities restore DPhe508-cystic fibrosis transmembrane conductance regulator chloride channel function in cystic fibrosis. Mol Pharmacol 2011; 80: 683-93
-
(2011)
Mol Pharmacol
, vol.80
, pp. 683-693
-
-
Phuan, P.W.1
Yang, B.2
Knapp, J.M.3
-
79
-
-
84856850146
-
Structure-activity relationships of cyanoquinolines with correctorpotentiator activity in DF508 cystic fibrosis transmembrane conductance regulator protein
-
Knapp JM, Wood AB, Phuan PW, et al. Structure-activity relationships of cyanoquinolines with correctorpotentiator activity in DF508 cystic fibrosis transmembrane conductance regulator protein. J Med Chem 2012; 55: 1242-51
-
(2012)
J Med Chem
, vol.55
, pp. 1242-1251
-
-
Knapp, J.M.1
Wood, A.B.2
Phuan, P.W.3
-
83
-
-
84903649442
-
Trimethylangelicin promotes the functional rescue of mutant F508del CFTR protein in cystic fibrosis airway cells
-
Favia M, Mancini MT, Bezzerri V, et al. Trimethylangelicin promotes the functional rescue of mutant F508del CFTR protein in cystic fibrosis airway cells. Am J Physiol Lung Cell Mol Physiol 2014; 307: L48-61
-
(2014)
Am J Physiol Lung Cell Mol Physiol
, vol.307
, pp. L48-61
-
-
Favia, M.1
Mancini, M.T.2
Bezzerri, V.3
-
84
-
-
46749153053
-
Identification of natural coumarin compounds that rescue defective DeltaF508-CFTR chloride channel gating
-
Xu LN, NaWL, Liu X, et al. Identification of natural coumarin compounds that rescue defective DeltaF508-CFTR chloride channel gating. Clin Exp Pharmacol Physiol 2008; 35: 878-83
-
(2008)
Clin Exp Pharmacol Physiol
, vol.35
, pp. 878-883
-
-
Xu, L.N.1
Nawl Liu, X.2
-
85
-
-
84860232519
-
Stimulation of airway and intestinal mucosal secretion by natural coumarin CFTR activators
-
Yang H, Xu LN, Sui YJ, et al. Stimulation of Airway and Intestinal Mucosal Secretion by Natural Coumarin CFTR Activators. Front Pharmacol 2011; 2: 52
-
(2011)
Front Pharmacol
, vol.2
, pp. 52
-
-
Yang, H.1
Xu, L.N.2
Sui, Y.J.3
-
86
-
-
70450162772
-
The structure and pharmacological functions of coumarins and their derivatives
-
Wu L, Wang X, Xu W, et al. The structure and pharmacological functions of coumarins and their derivatives. Curr Med Chem 2009; 16: 4236-60
-
(2009)
Curr Med Chem
, vol.16
, pp. 4236-4260
-
-
Wu, L.1
Wang, X.2
Xu, W.3
-
91
-
-
84907211027
-
Some gating potentiators, including VX-770, diminish DF508-CFTR functional expression
-
Veit G, Avramescu RG, Perdomo D, et al. Some gating potentiators, including VX-770, diminish DF508-CFTR functional expression. Sci Transl Med 2014; 6(246): 246ra97
-
(2014)
Sci Transl Med
, vol.6
, Issue.246
, pp. 246ra97
-
-
Veit, G.1
Avramescu, R.G.2
Perdomo, D.3
-
92
-
-
84879410121
-
Mechanism-based corrector combination restores DF508-CFTR folding and function
-
Okiyoneda T, Veit G, Dekkers JF, et al. Mechanism-based corrector combination restores DF508-CFTR folding and function. Nat Chem Biol 2013; 444-54
-
Nat Chem Biol
, vol.2013
, pp. 444-454
-
-
Okiyoneda, T.1
Veit, G.2
Dekkers, J.F.3
-
93
-
-
84862908028
-
Correction of both NBD1 energetics and domain interface is required to restore DF508 CFTR folding and function
-
Rabeh WM, Bossard F, Xu H, et al. Correction of both NBD1 energetics and domain interface is required to restore DF508 CFTR folding and function. Cell 2012; 148: 150-63.
-
(2012)
Cell
, vol.148
, pp. 150-163
-
-
Rabeh, W.M.1
Bossard, F.2
Xu, H.3
-
94
-
-
84880894290
-
Revertants, low temperature, and correctors reveal the mechanism of F508del-CFTR rescue by VX-809 and suggest multiple agents for full correction
-
Farinha CM, King-Underwood J, Sousa M, et al. Revertants, low temperature, and correctors reveal the mechanism of F508del-CFTR rescue by VX-809 and suggest multiple agents for full correction. Chem Biol 2013; 20: 943-55
-
(2013)
Chem Biol
, vol.20
, pp. 943-955
-
-
Farinha, C.M.1
King-Underwood, J.2
Sousa, M.3
-
95
-
-
84856629737
-
CFTR: Folding, misfolding and correcting the DF508 conformational defect
-
Lukacs GL, Verkman AS. CFTR: folding, misfolding and correcting the DF508 conformational defect. Trends Mol Med 2012; 18: 81-91
-
(2012)
Trends Mol Med
, vol.18
, pp. 81-91
-
-
Lukacs, G.L.1
Verkman, A.S.2
-
96
-
-
78649904312
-
Clinical studies with traditional Chinese medicine in the past decade and future research and development
-
Jiang M, Yang J, Zhang C, et al. Clinical studies with traditional Chinese medicine in the past decade and future research and development. Planta Med 2010; 76: 2048-64
-
(2010)
Planta Med
, vol.76
, pp. 2048-2064
-
-
Jiang, M.1
Yang, J.2
Zhang, C.3
-
97
-
-
77955058763
-
The role of traditional Chinese herbal medicines in cancer therapy-from TCM theory to mechanistic insights
-
Hsiao WL, Liu L. The role of traditional Chinese herbal medicines in cancer therapy-from TCM theory to mechanistic insights. Planta Med 2010; 76: 1118-31
-
(2010)
Planta Med
, vol.76
, pp. 1118-1131
-
-
Hsiao, W.L.1
Liu, L.2
-
98
-
-
84899423417
-
Identification of resveratrol oligomers as inhibitors of cystic fibrosis transmembrane conductance regulator by high-throughput screening of natural products from Chinese medicinal plants
-
Zhang Y, Yu B, Sui Y, et al. Identification of resveratrol oligomers as inhibitors of cystic fibrosis transmembrane conductance regulator by high-throughput screening of natural products from chinese medicinal plants. PLoS One 2014; 9: e94302
-
(2014)
PLoS One
, vol.9
, pp. e94302
-
-
Zhang, Y.1
Yu, B.2
Sui, Y.3
-
99
-
-
84916910820
-
Oridonin: A small molecule inhibitor of cystic fibrosis transmembrane conductance regulator (CFTR) isolated from traditional Chinese medicine
-
Luan J, Zhang Y, Yang S, et al. Oridonin: A small molecule inhibitor of cystic fibrosis transmembrane conductance regulator (CFTR) isolated from traditional Chinese medicine. Fitoterapia 2014; 100C: 88-94
-
(2014)
Fitoterapia
, vol.100 C
, pp. 88-94
-
-
Luan, J.1
Zhang, Y.2
Yang, S.3
-
100
-
-
84924090087
-
Bioactivity-guided Fractionation of an Antidiarrheal Chinese Herb Rhodiola kirilowii (Regel) Maxim Reveals (-)- epicatechin-3-gallate and (-)- epigallocatechin-3-gallate as Inhibitors of Cystic Fibrosis Transmembrane Conductance Regulator
-
Chen L, Yu B, Zhang Y, et al. Bioactivity-guided Fractionation of an Antidiarrheal Chinese Herb Rhodiola kirilowii (Regel) Maxim Reveals (-)- epicatechin-3-gallate and (-)- epigallocatechin-3-gallate as Inhibitors of Cystic Fibrosis Transmembrane Conductance Regulator. PLoS ONE 2015; 10(3): e0119122
-
(2015)
PLoS ONE
, vol.10
, Issue.3
, pp. e0119122
-
-
Chen, L.1
Yu, B.2
Zhang, Y.3
-
101
-
-
84926090443
-
Combined effects of VX-770 and VX-809 on several functional abnormalities of F508del- CFTR channels
-
Kopeikin Z, Yuksek Z, Yang HY, Bompadre SG. Combined effects of VX-770 and VX-809 on several functional abnormalities of F508del- CFTR channels. J Cyst Fibros 2014; 13: 508-14
-
(2014)
J Cyst Fibros
, vol.13
, pp. 508-514
-
-
Kopeikin, Z.1
Yuksek, Z.2
Yang, H.Y.3
Bompadre, S.G.4
-
102
-
-
84879000844
-
Cystic fibrosis transmembrane regulator correctors and potentiators
-
Rowe SM, Verkman AS. Cystic fibrosis transmembrane regulator correctors and potentiators. Cold Spring Harb Perspect Med 2013; 3: a009761
-
(2013)
Cold Spring Harb Perspect Med
, vol.3
, pp. a009761
-
-
Rowe, S.M.1
Verkman, A.S.2
|