-
1
-
-
0031687701
-
What is a cystic fibrosis diagnosis?
-
Rosenstein BJ: What is a cystic fibrosis diagnosis? Clin Chest Med 1998;19:423-441.
-
(1998)
Clin Chest Med
, vol.19
, pp. 423-441
-
-
Rosenstein, B.J.1
-
2
-
-
0031425079
-
Strategics for correcting the delta F508 CFTR protein-folding detect
-
Brown CR, Hong-Brown LQ, Welch WJ: Strategics for correcting the delta F508 CFTR protein-folding detect. J Bioenerg Biomembr 1997;29:491-502.
-
(1997)
J Bioenerg Biomembr
, vol.29
, pp. 491-502
-
-
Brown, C.R.1
Hong-Brown, L.Q.2
Welch, W.J.3
-
3
-
-
0026907529
-
Mislocalization of delta F508 CFTR in cystic fibrosis sweat gland
-
Kartner N, Augustinas O, Jensen TJ, Naismith AL, Riordan JR: Mislocalization of delta F508 CFTR in cystic fibrosis sweat gland. Nat Genet 1992;1:321-327.
-
(1992)
Nat Genet
, vol.1
, pp. 321-327
-
-
Kartner, N.1
Augustinas, O.2
Jensen, T.J.3
Naismith, A.L.4
Riordan, J.R.5
-
4
-
-
0025242929
-
Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
-
Cheng SH, Gregory RJ, Marshall J, Paul S, Souza DW, White GA, O'Riordan CR, Smith AE: Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell 1990;63:827-834.
-
(1990)
Cell
, vol.63
, pp. 827-834
-
-
Cheng, S.H.1
Gregory, R.J.2
Marshall, J.3
Paul, S.4
Souza, D.W.5
White, G.A.6
O'Riordan, C.R.7
Smith, A.E.8
-
5
-
-
0025863209
-
Generation of cAMP-activated chloride currents by expression of CFTR
-
Anderson MP, Rich DP, Gregory RJ, Smith AE, Welsh MJ: Generation of cAMP-activated chloride currents by expression of CFTR. Science 1991;251:679-682.
-
(1991)
Science
, vol.251
, pp. 679-682
-
-
Anderson, M.P.1
Rich, D.P.2
Gregory, R.J.3
Smith, A.E.4
Welsh, M.J.5
-
7
-
-
0029912194
-
Regulation of epithelial sodium channels by the cystic fibrosis transmembrane conductance regulator
-
Ismailov II, Awayda MS, Jovov B, Berdiev BK, Fuller CM, Dedman JR, Kaetzel M, Benos DJ: Regulation of epithelial sodium channels by the cystic fibrosis transmembrane conductance regulator. J Biol Chem 1996;271:4725-4732.
-
(1996)
J Biol Chem
, vol.271
, pp. 4725-4732
-
-
Ismailov, I.I.1
Awayda, M.S.2
Jovov, B.3
Berdiev, B.K.4
Fuller, C.M.5
Dedman, J.R.6
Kaetzel, M.7
Benos, D.J.8
-
10
-
-
0027191065
-
CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship
-
Gabriel SE, Clarke LL, Boucher RC, Stutts MJ: CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship. Nature 1993;363:263-268.
-
(1993)
Nature
, vol.363
, pp. 263-268
-
-
Gabriel, S.E.1
Clarke, L.L.2
Boucher, R.C.3
Stutts, M.J.4
-
11
-
-
0027464248
-
Outwardly rectifying chloride channels and CF: A divorce and remarriage
-
Guggino WB: Outwardly rectifying chloride channels and CF: A divorce and remarriage. J Biocnerg Biomembr 1993;25:27-35.
-
(1993)
J Biocnerg Biomembr
, vol.25
, pp. 27-35
-
-
Guggino, W.B.1
-
12
-
-
0028980536
-
CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP
-
Schwiebert EM, Egan ME, Hwang TH, Fulmer SB, Allen SS, Cutting GR, Guggino WB: CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP. Cell 1995;81:1063-1073.
-
(1995)
Cell
, vol.81
, pp. 1063-1073
-
-
Schwiebert, E.M.1
Egan, M.E.2
Hwang, T.H.3
Fulmer, S.B.4
Allen, S.S.5
Cutting, G.R.6
Guggino, W.B.7
-
13
-
-
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.1
Olsen, J.C.2
Sarkadi, B.3
Moore, K.L.4
Swanstrom, R.5
Boucher, R.C.6
-
14
-
-
0031718588
-
Clinical implications of cystic fibrosis transmembrane conductance regulator mutations
-
Fid SB (ed). Philadelphia, Saunders
-
Mickle JE, Cutting GR: Clinical implications of cystic fibrosis transmembrane conductance regulator mutations; in Fid SB (ed): Cystic Fibrosis. Clin Chest Med. Philadelphia, Saunders, 1999, vol 19, pp 443-458.
-
(1999)
Cystic Fibrosis. Clin Chest Med.
, vol.19
, pp. 443-458
-
-
Mickle, J.E.1
Cutting, G.R.2
-
15
-
-
0033618404
-
C-terminal truncations destabilize the cystic fibrosis transmembrane conductance regulator without impairing its biogenesis. A novel class of mutation
-
Haardt M, Benharouga M, Lechardeur D, Kartner N, Lukacs GL: C-terminal truncations destabilize the cystic fibrosis transmembrane conductance regulator without impairing its biogenesis. A novel class of mutation. J Biol Chem 1999;274:21873-21877.
-
(1999)
J Biol Chem
, vol.274
, pp. 21873-21877
-
-
Haardt, M.1
Benharouga, M.2
Lechardeur, D.3
Kartner, N.4
Lukacs, G.L.5
-
16
-
-
0018379922
-
Phenotypic suppression of nonsense mutants in yeast by aminoglycoside antibiotics
-
Palmer E, Wilhelm JM, Sherman F: Phenotypic suppression of nonsense mutants in yeast by aminoglycoside antibiotics. Nature 1979;277: 148-150.
-
(1979)
Nature
, vol.277
, pp. 148-150
-
-
Palmer, E.1
Wilhelm, J.M.2
Sherman, F.3
-
17
-
-
0018344962
-
Phenotypic suppression and misreading Saccharomyces cerevisiae
-
Singh A, Ursic D, Davies J: Phenotypic suppression and misreading Saccharomyces cerevisiae. Nature 1979;277:146-148.
-
(1979)
Nature
, vol.277
, pp. 146-148
-
-
Singh, A.1
Ursic, D.2
Davies, J.3
-
18
-
-
0030702773
-
Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line
-
Bedwell DM, Kaenjak A, Benos DJ, Bebok Z, Bubien JK, Hong J, Tousson A, Clancy JP, Sorscher EJ: Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line. Nat Med 1997;3:1280-1284.
-
(1997)
Nat Med
, vol.3
, pp. 1280-1284
-
-
Bedwell, D.M.1
Kaenjak, A.2
Benos, D.J.3
Bebok, Z.4
Bubien, J.K.5
Hong, J.6
Tousson, A.7
Clancy, J.P.8
Sorscher, E.J.9
-
19
-
-
0029994529
-
Aminoglycoside antibiotics restore CFTR function by overcoming premature slop mutations
-
Howard M, Frizzell RA, Bedwell DM: Aminoglycoside antibiotics restore CFTR function by overcoming premature slop mutations (see comments). Nat Med 1996;2:467-469.
-
(1996)
Nat Med
, vol.2
, pp. 467-469
-
-
Howard, M.1
Frizzell, R.A.2
Bedwell, D.M.3
-
20
-
-
0032720705
-
Aminoglycoside antibiotics restore dystrophin function to skeletal muscles of mdx mice
-
Barton-Davis ER, Cordier L, Shoturma DI, Leland SF, Sweeney HL: Aminoglycoside antibiotics restore dystrophin function to skeletal muscles of mdx mice (see comments). J Clin Invest 1999;104:375-381.
-
(1999)
J Clin Invest
, vol.104
, pp. 375-381
-
-
Barton-Davis, E.R.1
Cordier, L.2
Shoturma, D.I.3
Leland, S.F.4
Sweeney, H.L.5
-
21
-
-
0000503027
-
A controlled trial of gentamicin to suppress premature stop codons in CF patients
-
Clancy JP, Bcbok Z, Jones J, Walker L, King C, Greer H, Wing L, Lyrene R, Sorscher EJ, Bedwell DM: A controlled trial of gentamicin to suppress premature stop codons in CF patients (abstract). Pediatr Pulmonol 1999;S19:239-240.
-
(1999)
Pediatr Pulmonol
, vol.S19
, pp. 239-240
-
-
Clancy, J.P.1
Bcbok, Z.2
Jones, J.3
Walker, L.4
King, C.5
Greer, H.6
Wing, L.7
Lyrene, R.8
Sorscher, E.J.9
Bedwell, D.M.10
-
22
-
-
0034073736
-
A pilot study of the effect of gentamicin on nasal potential difference measurements in cystic fibrosis patients carrying stop mutations
-
Wilschanski M, Famini C, Blau H, Rivlin J, Augarten A, Avital A, Kerem B, Kerem E: A pilot study of the effect of gentamicin on nasal potential difference measurements in cystic fibrosis patients carrying stop mutations. Am J Respir Crit Care Med 2000;161:860-865.
-
(2000)
Am J Respir Crit Care Med
, vol.161
, pp. 860-865
-
-
Wilschanski, M.1
Famini, C.2
Blau, H.3
Rivlin, J.4
Augarten, A.5
Avital, A.6
Kerem, B.7
Kerem, E.8
-
23
-
-
0027390125
-
Effects of the delta F508 mutation on the structure, function, and folding of the first nucleotide-binding domain of CFTR
-
Thomas PJ, Pedersen PL: Effects of the delta F508 mutation on the structure, function, and folding of the first nucleotide-binding domain of CFTR. J Bioenerg Biomembr 1993;25:11-19.
-
(1993)
J Bioenerg Biomembr
, vol.25
, pp. 11-19
-
-
Thomas, P.J.1
Pedersen, P.L.2
-
24
-
-
0026649584
-
The cystic fibrosis transmembrane conductance regulator. Effects of the most common cystic fibrosis-causing mutation on the secondary structure and stability of a synthetic peptide
-
Thomas PJ, Shenbagamurthi P, Sondek J, Hullihen JM, Pedersen PL: The cystic fibrosis transmembrane conductance regulator. Effects of the most common cystic fibrosis-causing mutation on the secondary structure and stability of a synthetic peptide. J Biol Chem 1992; 267:5727-5730.
-
(1992)
J Biol Chem
, vol.267
, pp. 5727-5730
-
-
Thomas, P.J.1
Shenbagamurthi, P.2
Sondek, J.3
Hullihen, J.M.4
Pedersen, P.L.5
-
25
-
-
0027524866
-
The cystic fibrosis transmembrane conductance regulator. Overexpression, purification, and characterization of wild type and delta F508 mutant forms of the first nucleotide binding fold in fusion with the maltose-binding protein
-
Ko YH, Thomas PJ, Delannoy MR, Pedersen PL: The cystic fibrosis transmembrane conductance regulator. Overexpression, purification, and characterization of wild type and delta F508 mutant forms of the first nucleotide binding fold in fusion with the maltose-binding protein. J Biol Chem 1993;268:24330-24338.
-
(1993)
J Biol Chem
, vol.268
, pp. 24330-24338
-
-
Ko, Y.H.1
Thomas, P.J.2
Delannoy, M.R.3
Pedersen, P.L.4
-
26
-
-
0028977988
-
- channel is functional when retained in endoplasmic reticulum of mammalian cells
-
- channel is functional when retained in endoplasmic reticulum of mammalian cells. J Biol Chem 1995;270:12347-12350.
-
(1995)
J Biol Chem
, vol.270
, pp. 12347-12350
-
-
Pasyk, E.A.1
Foskett, J.K.2
-
27
-
-
0033536032
-
Cystic fibrosis transmembrane conductance regulator solution structures of peptides based on the Phc508 region, the most common site of disease-causing AF508 mutation
-
Massiah MA, Ko YH, Pedersen PL, Mildvan AS: Cystic fibrosis transmembrane conductance regulator Solution structures of peptides based on the Phc508 region, the most common site of disease-causing AF508 mutation. Biochemistry 1999;38:7453-7461.
-
(1999)
Biochemistry
, vol.38
, pp. 7453-7461
-
-
Massiah, M.A.1
Ko, Y.H.2
Pedersen, P.L.3
Mildvan, A.S.4
-
28
-
-
0344390884
-
A PDZ-interacting domain in CFTR is an apical membrane polarization signal
-
Moyer BD, Denton J, Karlson KH, Reynolds D, Wang S, Mickle JE, Milcwski M, Cutting GR, Guggino WB, Li M, Stanton BA: A PDZ-interacting domain in CFTR is an apical membrane polarization signal. J Clin Invest 1999; 104:1353-1361.
-
(1999)
J Clin Invest
, vol.104
, pp. 1353-1361
-
-
Moyer, B.D.1
Denton, J.2
Karlson, K.H.3
Reynolds, D.4
Wang, S.5
Mickle, J.E.6
Milcwski, M.7
Cutting, G.R.8
Guggino, W.B.9
Li, M.10
Stanton, B.A.11
-
29
-
-
0032953205
-
CFTR channel insertion to the apical surface in rat duodenal villus epithelial cells is upregulated by VIP in vivo
-
Ameen NA, Martensson B, Bourguinon L, Marino C, Isenberg J, McLaughlin GE: CFTR channel insertion to the apical surface in rat duodenal villus epithelial cells is upregulated by VIP in vivo. J Cell Sci 1999;112:887-894.
-
(1999)
J Cell Sci
, vol.112
, pp. 887-894
-
-
Ameen, N.A.1
Martensson, B.2
Bourguinon, L.3
Marino, C.4
Isenberg, J.5
McLaughlin, G.E.6
-
30
-
-
0028236014
-
Rapid endocytosis of the cystic fibrosis transmembranc conductance regulator chloride channel
-
Prince LS, Workman RBJ, Marchase RB: Rapid endocytosis of the cystic fibrosis transmembranc conductance regulator chloride channel. Proc Natl Acad Sci USA 1994;91:5192-5196.
-
(1994)
Proc Natl Acad Sci USA
, vol.91
, pp. 5192-5196
-
-
Prince, L.S.1
Workman, R.B.J.2
Marchase, R.B.3
-
31
-
-
0030896451
-
Correcting temperature-sensitive protein folding defects
-
Brown CR, Hong-Brown LQ, Welch WJ: Correcting temperature-sensitive protein folding defects. J Clin Invest 1997;99:1432-1444.
-
(1997)
J Clin Invest
, vol.99
, pp. 1432-1444
-
-
Brown, C.R.1
Hong-Brown, L.Q.2
Welch, W.J.3
-
32
-
-
0026781952
-
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
-
Denning GM, Anderson MP, Amara JF, Marshall J, Smith AE, Welsh MJ: Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive (see comments). Nature 1992;358:761-764.
-
(1992)
Nature
, vol.358
, pp. 761-764
-
-
Denning, G.M.1
Anderson, M.P.2
Amara, J.F.3
Marshall, J.4
Smith, A.E.5
Welsh, M.J.6
-
33
-
-
0028896289
-
Differential expression of ORCC and CFTR induced by low temperature in CF airway epithelial cells
-
Egan ME, Schweibert EM, Guggino WB: Differential expression of ORCC and CFTR induced by low temperature in CF airway epithelial cells. Am J Physiol 1995;268:C243-C251.
-
(1995)
Am J Physiol
, vol.268
-
-
Egan, M.E.1
Schweibert, E.M.2
Guggino, W.B.3
-
34
-
-
0028858161
-
Multiple proteolytic systems, including the proteasome, contribute to CFTR processing
-
Jensen TJ, Loo MA, Pind S, Williams DB, Goldberg AL, Riordan JR: Multiple proteolytic systems, including the proteasome, contribute to CFTR processing. Cell 1995;83:129-135.
-
(1995)
Cell
, vol.83
, pp. 129-135
-
-
Jensen, T.J.1
Loo, M.A.2
Pind, S.3
Williams, D.B.4
Goldberg, A.L.5
Riordan, J.R.6
-
35
-
-
0028006681
-
Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins
-
Ward CL, Kopito RR: Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins. J Biol Chem 1994;269:25710-25718.
-
(1994)
J Biol Chem
, vol.269
, pp. 25710-25718
-
-
Ward, C.L.1
Kopito, R.R.2
-
36
-
-
0030042386
-
Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation
-
Sato S, Ward CL, Krouse ME, Wine JJ, Kopito RR: Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation. J Biol Chem 1996;271:635-638.
-
(1996)
J Biol Chem
, vol.271
, pp. 635-638
-
-
Sato, S.1
Ward, C.L.2
Krouse, M.E.3
Wine, J.J.4
Kopito, R.R.5
-
37
-
-
0030154620
-
Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein
-
Brown CR, Hong-Brown LQ, Biwersi J, Verkman AS, Welch WJ: Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein. Cell Stress Chaperones 1996; 1:117-125.
-
(1996)
Cell Stress Chaperones
, vol.1
, pp. 117-125
-
-
Brown, C.R.1
Hong-Brown, L.Q.2
Biwersi, J.3
Verkman, A.S.4
Welch, W.J.5
-
38
-
-
0030881843
-
The final stage of gene expression: Chaperones and the regulation of protein fate
-
Ashkenas J, Byers PH: The final stage of gene expression: Chaperones and the regulation of protein fate. Am J Hum Genet 1997;61:267-272.
-
(1997)
Am J Hum Genet
, vol.61
, pp. 267-272
-
-
Ashkenas, J.1
Byers, P.H.2
-
39
-
-
0031853136
-
Partial restoration of cAMP-stimulated CFTR chloride channel activity in ΔF50X eells by deoxyspergualin
-
Jiang C, Fang SL, Xiao YF, O'Connor SP, Nadler SG, Lee DW, Jefferson DM, Kaplan JM, Smith AE, Cheng SH: Partial restoration of cAMP-stimulated CFTR chloride channel activity in ΔF50X eells by deoxyspergualin. Am J Physiol 1998;275:C171-C178.
-
(1998)
Am J Physiol
, vol.275
-
-
Jiang, C.1
Fang, S.L.2
Xiao, Y.F.3
O'Connor, S.P.4
Nadler, S.G.5
Lee, D.W.6
Jefferson, D.M.7
Kaplan, J.M.8
Smith, A.E.9
Cheng, S.H.10
-
40
-
-
0030798979
-
The molecular chaperone Hsc70 assists the in vitro folding of the N-rerminal nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator
-
Strickland E, Qu BH, Millen L, Thomas PJ: The molecular chaperone Hsc70 assists the in vitro folding of the N-rerminal nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator. J Biol Chem 1997;272:25421-25424.
-
(1997)
J Biol Chem
, vol.272
, pp. 25421-25424
-
-
Strickland, E.1
Qu, B.H.2
Millen, L.3
Thomas, P.J.4
-
41
-
-
0028232167
-
Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis ot the cystic fibrosis transmembrane conductance regulator
-
Pind S, Riordan JR, Williams DB: Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis ot the cystic fibrosis transmembrane conductance regulator. J Biol Chem 1994;269: 12784-12788.
-
(1994)
J Biol Chem
, vol.269
, pp. 12784-12788
-
-
Pind, S.1
Riordan, J.R.2
Williams, D.B.3
-
42
-
-
0027488993
-
The common variant of cystic fibrosis transmembrane conductance regulator is recognized by Hsp70 and degraded in a pre-Golgi nonlysosomal compartment
-
Yang Y, Janich S, Cohn JA, Wilson JM: The common variant of cystic fibrosis transmembrane conductance regulator is recognized by Hsp70 and degraded in a pre-Golgi nonlysosomal compartment. Proc Natl Acad Sci USA 1993;90:9480-9484.
-
(1993)
Proc Natl Acad Sci USA
, vol.90
, pp. 9480-9484
-
-
Yang, Y.1
Janich, S.2
Cohn, J.A.3
Wilson, J.M.4
-
43
-
-
0030809817
-
In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbuty-rate 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-phenylbuty-rate in cystic fibrosis epithelial cells containing delta F508-CFTR. J Clin Invest 1997;100: 2457-2465.
-
(1997)
J Clin Invest
, vol.100
, pp. 2457-2465
-
-
Rubenstein, R.C.1
Egan, M.E.2
Zeitlin, P.L.3
-
45
-
-
0032871691
-
Butyrate increases apical membrane CFTR but reduces chloride secretion in MDCK cells
-
Mover BD, Loffing-Cueni D, Loffing J, Reynolds D, Stanton BA: Butyrate increases apical membrane CFTR but reduces chloride secretion in MDCK cells. Am J Physiol 1999;277: F271-F276.
-
(1999)
Am J Physiol
, vol.277
-
-
Mover, B.D.1
Loffing-Cueni, D.2
Loffing, J.3
Reynolds, D.4
Stanton, B.A.5
-
46
-
-
0032743305
-
Defective function of the cystic fibrosis-causing missense mutation G551D is recovered by genistein
-
Illek B, Zhang L, Lewis NC, Moss RB, Dong JY, Fischer H: Defective function of the cystic fibrosis-causing missense mutation G551D is recovered by genistein. Am J Physiol 1999;277: C833-C839.
-
(1999)
Am J Physiol
, vol.277
-
-
Illek, B.1
Zhang, L.2
Lewis, N.C.3
Moss, R.B.4
Dong, J.Y.5
Fischer, H.6
-
47
-
-
0034099743
-
Sodium 4-phenylbutyrate downregulates hsc70: Implications for intracellular trafficking of ΔF508-CFTR
-
Rubenstein RC, Zeitlin PL: Sodium 4-phenylbutyrate downregulates hsc70: Implications for intracellular trafficking of ΔF508-CFTR. Am J Physiol 2000;278:C259-C267.
-
(2000)
Am J Physiol
, vol.278
-
-
Rubenstein, R.C.1
Zeitlin, P.L.2
-
48
-
-
0032867811
-
Processing of CFTR bearing the P574H mutation differs from wild-type and ΔF508-CFTR
-
Ostedgaard LS, Zeiher B, Welsh MJ: Processing of CFTR bearing the P574H mutation differs from wild-type and ΔF508-CFTR. J Cell Sci 1999;112:2091-2098.
-
(1999)
J Cell Sci
, vol.112
, pp. 2091-2098
-
-
Ostedgaard, L.S.1
Zeiher, B.2
Welsh, M.J.3
-
49
-
-
0031889082
-
A pilot clinical trial of sodium 4-phenylbutyrate(Huphenyl) in delta F508-homozygous cystic fibrosis patients: Evidence of restoration of nasal epithelial CFTR function
-
Rubenstein RC, Zeitlin PL: A pilot clinical trial of sodium 4-phenylbutyrate(Huphenyl) in delta F508-homozygous cystic fibrosis patients: Evidence of restoration of nasal epithelial CFTR function. Am J Respir Crit Care Med 1998;157:484-490.
-
(1998)
Am J Respir Crit Care Med
, vol.157
, pp. 484-490
-
-
Rubenstein, R.C.1
Zeitlin, P.L.2
-
50
-
-
0031730433
-
Gene redundancy and pharmacological gene therapy: Implications for X-linked adrenoleukodystrophy
-
Kemp S, Wei HM, Lu JF, Braiterman LT, McGuinness MC, Moser AB, Watkins PA, Smith KD: Gene redundancy and pharmacological gene therapy: Implications for X-linked adrenoleukodystrophy (see comments). Nat Med 1998;4:1261-1268.
-
(1998)
Nat Med
, vol.4
, pp. 1261-1268
-
-
Kemp, S.1
Wei, H.M.2
Lu, J.F.3
Braiterman, L.T.4
McGuinness, M.C.5
Moser, A.B.6
Watkins, P.A.7
Smith, K.D.8
-
51
-
-
0031694568
-
Alpha-1-antitrypsin deficiency
-
Perlmutter DH: Alpha-1-antitrypsin deficiency. Semin Liver Dis 1998;18:217-225.
-
(1998)
Semin Liver Dis
, vol.18
, pp. 217-225
-
-
Perlmutter, D.H.1
-
52
-
-
0029788023
-
Degradation of a mutant secretory protein, alpha 1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity
-
Qu D, Teckman JH, Omura S, Perlmutter DM: Degradation of a mutant secretory protein, alpha 1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity. J Biol Chem 1996:271:22791-22795.
-
(1996)
J Biol Chem
, vol.271
, pp. 22791-22795
-
-
Qu, D.1
Teckman, J.H.2
Omura, S.3
Perlmutter, D.M.4
-
53
-
-
0031763141
-
Flavonoids stimulate Cl conductance of human airway epithelium in vitro and in vivo
-
Illek B, Fischer H: Flavonoids stimulate Cl conductance of human airway epithelium in vitro and in vivo. Am J Physiol 1998;275: L902-L910.
-
(1998)
Am J Physiol
, vol.275
-
-
Illek, B.1
Fischer, H.2
-
54
-
-
0026611093
-
1 adenosine-receptor antagonists activate chloride efflux from cystic fibrosis cells
-
1 adenosine-receptor antagonists activate chloride efflux from cystic fibrosis cells. Proc Natl Acad Sci USA 1992;89: 5562-5566.
-
(1992)
Proc Natl Acad Sci USA
, vol.89
, pp. 5562-5566
-
-
Eidelman, O.1
Guay-Broder, C.2
Van Galen, P.J.3
Jacobson, K.A.4
Fox, C.5
Turner, R.J.6
Cabantchik, Z.I.7
Pollard, H.B.8
-
55
-
-
0032489513
-
Direct activation of cystic fibrosis transmembrane conductance regulator channels by 8-cyclopentyl-1.3-dipropylxanthine (CPX) and 1.3-diallyl-8-cyclohexylxanthine (DAX)
-
Arispe N, Ma J, Jacobson KA, Pollard MB: Direct activation of cystic fibrosis transmembrane conductance regulator channels by 8-cyclopentyl-1.3-dipropylxanthine (CPX) and 1.3-diallyl-8-cyclohexylxanthine (DAX). J Biol Chem 1997;273:5727-5734.
-
(1997)
J Biol Chem
, vol.273
, pp. 5727-5734
-
-
Arispe, N.1
Ma, J.2
Jacobson, K.A.3
Pollard, M.B.4
-
57
-
-
0030943895
-
8-cyclopentyl-1.3-dipropylxanthine and other xanthines differentially hind to the wild-type and delta F508 first nucleotide binding fold (NBF-1) domains of the cystic fibrosis transmembrane conductance regulator
-
Cohen BE, Lee G, Jacobson KA, Kim YC, Huang Z, Sorscher FJ, Pollard HB: 8-Cyclopentyl-1.3-dipropylxanthine and other xanthines differentially hind to the wild-type and delta F508 first nucleotide binding fold (NBF-1) domains of the cystic fibrosis transmembrane conductance regulator. Biochemistry 1997;36:6455-6461.
-
(1997)
Biochemistry
, vol.36
, pp. 6455-6461
-
-
Cohen, B.E.1
Lee, G.2
Jacobson, K.A.3
Kim, Y.C.4
Huang, Z.5
Sorscher, F.J.6
Pollard, H.B.7
-
58
-
-
0029899053
-
Delta F508-CFTR channels: Kinetics, activation by forskolin, and potentiation by xanthines
-
Haws CM, Nepomuceno IB, Krouse ME, Wakelee H, Law T, Xia Y, Nguyen H, Wine JJ: Delta F508-CFTR channels: kinetics, activation by forskolin, and potentiation by xanthines. Am J Physiol 1996;270:C1544-C1555.
-
(1996)
Am J Physiol
, vol.270
-
-
Haws, C.M.1
Nepomuceno, I.B.2
Krouse, M.E.3
Wakelee, H.4
Law, T.5
Xia, Y.6
Nguyen, H.7
Wine, J.J.8
-
59
-
-
0029556721
-
CFTR-mediated chloride permeability is regulated by type III phosphodiesterases in airway epithelial cells
-
Kelley TJ, Al-Nakkash L, Drumm ML: CFTR-mediated chloride permeability is regulated by type III phosphodiesterases in airway epithelial cells. Am J Respir Cell Mol Biol 1995;13:657-664.
-
(1995)
Am J Respir Cell Mol Biol
, vol.13
, pp. 657-664
-
-
Kelley, T.J.1
Al-Nakkash, L.2
Drumm, M.L.3
-
60
-
-
0029665963
-
Activation of endogenous ΔF508 cystic fibrosis transmembrane conductance regulator by phosphodiesterase inhibition
-
Kelley TJ, Al-Nakkash L, Cotton CU, Drumm ML: Activation of endogenous ΔF508 cystic fibrosis transmembrane conductance regulator by phosphodiesterase inhibition. J Clin Invest 1996;98:513-520.
-
(1996)
J Clin Invest
, vol.98
, pp. 513-520
-
-
Kelley, T.J.1
Al-Nakkash, L.2
Cotton, C.U.3
Drumm, M.L.4
-
61
-
-
0030900255
-
In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant ΔF508 in murine nasal epithelium
-
Kelley TJ, Thomas K, Milgram LJ, Drumm ML: In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant ΔF508 in murine nasal epithelium. Proc Natl Acad Sci USA 1997;94:2604-2608.
-
(1997)
Proc Natl Acad Sci USA
, vol.94
, pp. 2604-2608
-
-
Kelley, T.J.1
Thomas, K.2
Milgram, L.J.3
Drumm, M.L.4
-
64
-
-
0028860909
-
Correlation of sweat chloride concentration with classes of the ostic fibrosis transmembrane conductance regulator gene mutations
-
Wilschanski M, Zielenski J, Markiewicx D, Tsui LC, Corey M, Levison H, Durie PR: Correlation of sweat chloride concentration with classes of the ostic fibrosis transmembrane conductance regulator gene mutations. J Pediatr 1995;127:705-710.
-
(1995)
J Pediatr
, vol.127
, pp. 705-710
-
-
Wilschanski, M.1
Zielenski, J.2
Markiewicx, D.3
Tsui, L.C.4
Corey, M.5
Levison, H.6
Durie, P.R.7
-
65
-
-
0027517995
-
Correlation between genotype and phenolypc in patients with cystic fibrosis. The cystic fibrosis genotype-phenotype consortium
-
Anonymous: Correlation between genotype and phenolypc in patients with cystic fibrosis. The Cystic Fibrosis Genotype-Phenotype Consortium (see comments). N F.ngl J Med 1993; 329:1308-1313.
-
(1993)
N F.ngl J Med
, vol.329
, pp. 1308-1313
-
-
-
67
-
-
0033019713
-
Adenosine and its nucleotides activate wild-type and RI17H CFTR through an A2B receptor-coupled pathway
-
Claney JP, Ruiz FE, Sorscher FJ: Adenosine and its nucleotides activate wild-type and RI17H CFTR through an A2B receptor-coupled pathway. Am J Physiol 1999;276:C361-C369.
-
(1999)
Am J Physiol
, vol.276
-
-
Claney, J.P.1
Ruiz, F.E.2
Sorscher, F.J.3
|