-
1
-
-
33645130994
-
Cystic fibrosis since 1938
-
Davis PB. Cystic fibrosis since 1938. Am J Respir Crit Care Med 2006;173:475-482.
-
(2006)
Am J Respir Crit Care Med
, vol.173
, pp. 475-482
-
-
Davis, P.B.1
-
2
-
-
33745755929
-
Gene therapy progress and prospects: Cystic fibrosis
-
Griesenbach U, Geddes DM, Alton EW. Gene therapy progress and prospects: cystic fibrosis. Gene Ther 2006;13:1061-1067.
-
(2006)
Gene Ther
, vol.13
, pp. 1061-1067
-
-
Griesenbach, U.1
Geddes, D.M.2
Alton, E.W.3
-
3
-
-
33846595770
-
New pharmacological approaches for treatment of cystic fibrosis
-
Bush A, Alton EWFW, Davies JC, Griesenbach U, Jaffe A, editors, London: Karger;
-
Rubenstein RC. New pharmacological approaches for treatment of cystic fibrosis. In: Bush A, Alton EWFW, Davies JC, Griesenbach U, Jaffe A, editors. Progress in respiratory research: cystic fibrosis in the 21st century. London: Karger; 2006. p. 212-220.
-
(2006)
Progress in respiratory research: Cystic fibrosis in the 21st century
, pp. 212-220
-
-
Rubenstein, R.C.1
-
4
-
-
53349116445
-
Animal models
-
Bush A, Alton EW, Davies JC, Griesenbach U, Jaffe A, editors, London: Karger;
-
Dorin JR. Animal models. In: Bush A, Alton EW, Davies JC, Griesenbach U, Jaffe A, editors. Progress in respiratory research: cystic fibrosis in the 21st century. London: Karger; 2006. p. 84-92.
-
(2006)
Progress in respiratory research: Cystic fibrosis in the 21st century
, pp. 84-92
-
-
Dorin, J.R.1
-
5
-
-
0028111842
-
Anomalies in ion transport in cf mouse tracheal epithelium
-
Grubb BR, Paradiso AM, Boucher RC. Anomalies in ion transport in cf mouse tracheal epithelium. Am J Physiol 1994;267:C293-C300.
-
(1994)
Am J Physiol
, vol.267
-
-
Grubb, B.R.1
Paradiso, A.M.2
Boucher, R.C.3
-
6
-
-
0027967986
-
Inefficient gene transfer by adenovirus vector to cystic fibrosis airway epithelia of mice and humans
-
Grubb BR, Pickles RJ, Ye H, Yankaskas JR, Vick RN, Engelhardt JF, Wilson JM, Johnson LG, Boucher RC. Inefficient gene transfer by adenovirus vector to cystic fibrosis airway epithelia of mice and humans. Nature 1994;371:802-806.
-
(1994)
Nature
, vol.371
, pp. 802-806
-
-
Grubb, B.R.1
Pickles, R.J.2
Ye, H.3
Yankaskas, J.R.4
Vick, R.N.5
Engelhardt, J.F.6
Wilson, J.M.7
Johnson, L.G.8
Boucher, R.C.9
-
7
-
-
0026649122
-
An animal model for cystic fibrosis made by gene targeting
-
Snouwaert JN, Brigman KK, Latour AM, Malouf NN, Boucher RC, Smithies O, Koller BH. An animal model for cystic fibrosis made by gene targeting. Science 1992;257:1083-1088.
-
(1992)
Science
, vol.257
, pp. 1083-1088
-
-
Snouwaert, J.N.1
Brigman, K.K.2
Latour, A.M.3
Malouf, N.N.4
Boucher, R.C.5
Smithies, O.6
Koller, B.H.7
-
8
-
-
9044236531
-
Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype-phenotype correlations
-
Delaney SJ, Alton EW, Smith SN, Lunn DP, Farley R, Lovelock PK, Thomson SA, Hume DA, Lamb D, Porteous DJ, et al. Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype-phenotype correlations. EMBO J 1996;15:955-963.
-
(1996)
EMBO J
, vol.15
, pp. 955-963
-
-
Delaney, S.J.1
Alton, E.W.2
Smith, S.N.3
Lunn, D.P.4
Farley, R.5
Lovelock, P.K.6
Thomson, S.A.7
Hume, D.A.8
Lamb, D.9
Porteous, D.J.10
-
9
-
-
0029153220
-
Generation and characterization of a delta F508 cystic fibrosis mouse model
-
Colledge WH, Abella BS, Southern KW, Ratcliff R, Jiang C, Cheng SH, MacVinish LJ, Anderson JR, Cuthbert AW, Evans MJ. Generation and characterization of a delta F508 cystic fibrosis mouse model. Nat Genet 1995;10:445-452.
-
(1995)
Nat Genet
, vol.10
, pp. 445-452
-
-
Colledge, W.H.1
Abella, B.S.2
Southern, K.W.3
Ratcliff, R.4
Jiang, C.5
Cheng, S.H.6
MacVinish, L.J.7
Anderson, J.R.8
Cuthbert, A.W.9
Evans, M.J.10
-
10
-
-
0030882184
-
Intestinal physiology and pathology in gene-targeted mouse models of cystic fibrosis
-
Grubb BR, Gabriel SE. Intestinal physiology and pathology in gene-targeted mouse models of cystic fibrosis. Am J Physiol 1997;273:G258-G266.
-
(1997)
Am J Physiol
, vol.273
-
-
Grubb, B.R.1
Gabriel, S.E.2
-
11
-
-
0028557095
-
Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR
-
Zhou L, Dey CR, Wert SE, DuVall MD, Frizzell RA, Whitsett JA. Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR. Science 1994;266:1705-1708.
-
(1994)
Science
, vol.266
, pp. 1705-1708
-
-
Zhou, L.1
Dey, C.R.2
Wert, S.E.3
DuVall, M.D.4
Frizzell, R.A.5
Whitsett, J.A.6
-
12
-
-
0034077631
-
Cystic fibrosis transmembrane conductance regulator-dependent regulation of epithelial inducible nitric oxide synthase expression
-
Steagall WK, Elmer HL, Brady KG, Kelley TJ. Cystic fibrosis transmembrane conductance regulator-dependent regulation of epithelial inducible nitric oxide synthase expression. Am J Respir Cell Mol Biol 2000;22:45-50.
-
(2000)
Am J Respir Cell Mol Biol
, vol.22
, pp. 45-50
-
-
Steagall, W.K.1
Elmer, H.L.2
Brady, K.G.3
Kelley, T.J.4
-
13
-
-
0033721158
-
Airway gene transfer in mouse nasal-airways: Importance of identification of epithelial type for assessment of gene transfer
-
Parsons DW, Hopkins PJ, Bourne AJ, Boucher RC, Martin AJ. Airway gene transfer in mouse nasal-airways: importance of identification of epithelial type for assessment of gene transfer. Gene Ther 2000;7:1810-1815.
-
(2000)
Gene Ther
, vol.7
, pp. 1810-1815
-
-
Parsons, D.W.1
Hopkins, P.J.2
Bourne, A.J.3
Boucher, R.C.4
Martin, A.J.5
-
14
-
-
0032615611
-
The in vivo effects of milrinone on the airways of cystic fibrosis mice and human subjects
-
Smith SN, Middleton PG, Chadwick S, Jaffe A, Bush KA, Rolleston S, Farley R, Delaney SJ, Wainwright B, Geddes DM, et al. The in vivo effects of milrinone on the airways of cystic fibrosis mice and human subjects. Am J Respir Cell Mol Biol 1999;20:129-134.
-
(1999)
Am J Respir Cell Mol Biol
, vol.20
, pp. 129-134
-
-
Smith, S.N.1
Middleton, P.G.2
Chadwick, S.3
Jaffe, A.4
Bush, K.A.5
Rolleston, S.6
Farley, R.7
Delaney, S.J.8
Wainwright, B.9
Geddes, D.M.10
-
15
-
-
0028991826
-
In vivo nasal potential difference: Techniques and protocols for assessing efficacy of gene transfer in cystic fibrosis
-
Knowles MR, Paradiso AM, Boucher RC. In vivo nasal potential difference: techniques and protocols for assessing efficacy of gene transfer in cystic fibrosis. Hum Gene Ther 1995;6:445-455.
-
(1995)
Hum Gene Ther
, vol.6
, pp. 445-455
-
-
Knowles, M.R.1
Paradiso, A.M.2
Boucher, R.C.3
-
16
-
-
0030869664
-
Normalization of ion transport in murine cystic fibrosis nasal epithelium using gene transfer
-
MacVinish LJ, Goddard C, Colledge WH, Higgins CF, Evans MJ, Cuthbert AW. Normalization of ion transport in murine cystic fibrosis nasal epithelium using gene transfer. Am J Physiol 1997;273:C734-C740.
-
(1997)
Am J Physiol
, vol.273
-
-
MacVinish, L.J.1
Goddard, C.2
Colledge, W.H.3
Higgins, C.F.4
Evans, M.J.5
Cuthbert, A.W.6
-
17
-
-
0026918861
-
Efficiency of gene transfer for restoration of normal airway epithelial function in cystic fibrosis
-
Johnson LG, Olsen JC, Sarkadi B, Moore KL, Swanstrom R, Boucher RC. Efficiency of gene transfer for restoration of normal airway epithelial function in cystic fibrosis. Nat Genet 1992;2:21-25.
-
(1992)
Nat Genet
, vol.2
, pp. 21-25
-
-
Johnson, L.G.1
Olsen, J.C.2
Sarkadi, B.3
Moore, K.L.4
Swanstrom, R.5
Boucher, R.C.6
-
18
-
-
0029813969
-
A demonstration using mouse models that successful gene therapy for cystic fibrosis requires only partial gene correction
-
Dorin JR, Farley R, Webb S, Smith SN, Farini E, Delaney SJ, Wainwright BJ, Alton EW, Porteous DJ. A demonstration using mouse models that successful gene therapy for cystic fibrosis requires only partial gene correction. Gene Ther 1996;3:797-801.
-
(1996)
Gene Ther
, vol.3
, pp. 797-801
-
-
Dorin, J.R.1
Farley, R.2
Webb, S.3
Smith, S.N.4
Farini, E.5
Delaney, S.J.6
Wainwright, B.J.7
Alton, E.W.8
Porteous, D.J.9
-
19
-
-
0038663174
-
Effect of genotype on phenotype and mortality in cystic fibrosis: A retrospective cohort study
-
McKone EF, Emerson SS, Edwards KL, Aitken ML. Effect of genotype on phenotype and mortality in cystic fibrosis: a retrospective cohort study. Lancet 2003;361:1671-1676.
-
(2003)
Lancet
, vol.361
, pp. 1671-1676
-
-
McKone, E.F.1
Emerson, S.S.2
Edwards, K.L.3
Aitken, M.L.4
-
20
-
-
0027361622
-
Noninvasive liposome-mediated gene delivery can correct the ion transport defect in cystic fibrosis mutant mice
-
Alton EW, Middleton PG, Caplen NJ, Smith SN, Steel DM, Munkonge FM, Jeffery PK, Geddes DM, Hart SL, Williamson R, et al. Noninvasive liposome-mediated gene delivery can correct the ion transport defect in cystic fibrosis mutant mice. Nat Genet 1993;5:135-142.
-
(1993)
Nat Genet
, vol.5
, pp. 135-142
-
-
Alton, E.W.1
Middleton, P.G.2
Caplen, N.J.3
Smith, S.N.4
Steel, D.M.5
Munkonge, F.M.6
Jeffery, P.K.7
Geddes, D.M.8
Hart, S.L.9
Williamson, R.10
-
21
-
-
0033586375
-
Cationic lipid-mediated CFTR gene transfer to the lungs and nose of patients with cystic fibrosis: A double-blind placebo-controlled trial
-
xAlton EW, Stern M, Farley R, Jaffe A, Chadwick SL, Phillips J, Davies J, Smith SN, Browning J, Davies MG, et al. Cationic lipid-mediated CFTR gene transfer to the lungs and nose of patients with cystic fibrosis: a double-blind placebo-controlled trial. Lancet 1999;353:947-954.
-
(1999)
Lancet
, vol.353
, pp. 947-954
-
-
xAlton, E.W.1
Stern, M.2
Farley, R.3
Jaffe, A.4
Chadwick, S.L.5
Phillips, J.6
Davies, J.7
Smith, S.N.8
Browning, J.9
Davies, M.G.10
-
22
-
-
19944398726
-
Compacted DNA nanoparticles administered to the nasal mucosa of cystic fibrosis subjects are safe and demonstrate partial to complete cystic fibrosis transmembrane regulator reconstitution
-
Konstan MW, Davis PB, Wagener JS, Hilliard KA, Stern RC, Milgram LJ, Kowalczyk TH, Hyatt SL, Fink TL, Gedeon CR, et al. Compacted DNA nanoparticles administered to the nasal mucosa of cystic fibrosis subjects are safe and demonstrate partial to complete cystic fibrosis transmembrane regulator reconstitution. Hum Gene Ther 2004;15:1255-1269.
-
(2004)
Hum Gene Ther
, vol.15
, pp. 1255-1269
-
-
Konstan, M.W.1
Davis, P.B.2
Wagener, J.S.3
Hilliard, K.A.4
Stern, R.C.5
Milgram, L.J.6
Kowalczyk, T.H.7
Hyatt, S.L.8
Fink, T.L.9
Gedeon, C.R.10
|