-
1
-
-
0026651508
-
Chloride channels in the apical membrane of normal and cystic fibrosis airway and intestinal epithelia
-
1. Anderson, M.P.; Sheppard, D.N.; Berger, H.A.; Welsh, M.J. Chloride channels in the apical membrane of normal and cystic fibrosis airway and intestinal epithelia. Am. J. Physiol. 263: L1-L14;1992.
-
(1992)
Am. J. Physiol.
, vol.263
-
-
Anderson, M.P.1
Sheppard, D.N.2
Berger, H.A.3
Welsh, M.J.4
-
2
-
-
0025871551
-
Calcium and cAMP activate different chloride channels in the apical membrane of normal and cystic fibrosis epithelia
-
2. Anderson, M.P.; Welsh, M.J. Calcium and cAMP activate different chloride channels in the apical membrane of normal and cystic fibrosis epithelia. Proc. Natl. Acad. Sci. U.S.A. 88: 6003-6007;1991.
-
(1991)
Proc. Natl. Acad. Sci. U.S.A.
, vol.88
, pp. 6003-6007
-
-
Anderson, M.P.1
Welsh, M.J.2
-
3
-
-
0025300405
-
Enhanced intestinal glucose and alanine transport in cystic fibrosis
-
3. Baxter, P.; Goldhill, J.; Hardcastle, J.; Hardcastle, P.T.; Taylor, C.J. Enhanced intestinal glucose and alanine transport in cystic fibrosis. Gut 31:817-820;1990.
-
(1990)
Gut
, vol.31
, pp. 817-820
-
-
Baxter, P.1
Goldhill, J.2
Hardcastle, J.3
Hardcastle, P.T.4
Taylor, C.J.5
-
4
-
-
0024584560
-
Abnormal jejunal potential difference in cystic fibrosis
-
4. Baxter, P.S.; Read, N.W.; Hardcastle, P.T.; Wilson, A.J.; Hardcastle, J.; Taylor, C.J. Abnormal jejunal potential difference in cystic fibrosis. Lancet 1:464-466;1989.
-
(1989)
Lancet
, vol.1
, pp. 464-466
-
-
Baxter, P.S.1
Read, N.W.2
Hardcastle, P.T.3
Wilson, A.J.4
Hardcastle, J.5
Taylor, C.J.6
-
5
-
-
0023695605
-
Altered intestinal chloride transport in cystic fibrosis
-
5. Berschneider, H.M.; Knowles, M.R.; Azizkhan, R.G.; Boucher, R.C.; Tobey, N.A.; Orlando, R.C.; Powell, D.W. Altered intestinal chloride transport in cystic fibrosis. FASEB J. 2:2625-2629;1988.
-
(1988)
FASEB J.
, vol.2
, pp. 2625-2629
-
-
Berschneider, H.M.1
Knowles, M.R.2
Azizkhan, R.G.3
Boucher, R.C.4
Tobey, N.A.5
Orlando, R.C.6
Powell, D.W.7
-
6
-
-
0024465128
-
Chloride secretory response of cystic fibrosis human airway epithelia: Preservation of calcium but not protein kinase C- and A-dependent mechanisms
-
6. Boucher, R.C.; Cheng, E.H.C.; Paradiso, A.M.; Stutts, M.J.; Knowles, M.R.; Earp, H.S. Chloride secretory response of cystic fibrosis human airway epithelia: preservation of calcium but not protein kinase C- and A-dependent mechanisms. J. Clin. Invest. 84:1424-1431;1989.
-
(1989)
J. Clin. Invest.
, vol.84
, pp. 1424-1431
-
-
Boucher, R.C.1
Cheng, E.H.C.2
Paradiso, A.M.3
Stutts, M.J.4
Knowles, M.R.5
Earp, H.S.6
-
8
-
-
0026640380
-
Defective epithelial chloride transport in a gene targeted mouse model of cystic fibrosis
-
8. Clarke, L.L.; Grubb, B.R.; Gabriel, S.E.; Smithies, O.; Koller, B.H.; Boucher, R.C. Defective epithelial chloride transport in a gene targeted mouse model of cystic fibrosis. Science 257: 1125-1128;1992.
-
(1992)
Science
, vol.257
, pp. 1125-1128
-
-
Clarke, L.L.1
Grubb, B.R.2
Gabriel, S.E.3
Smithies, O.4
Koller, B.H.5
Boucher, R.C.6
-
9
-
-
0028047286
-
Relationship of a non-CFTR mediated chloride conductance to organ-level disease in cftr(-/ -) mice
-
9. Clarke, L.L.; Grubb, B.R.; Yankaskas, J.R.; Cotton, C.U.; McKenzie, A.; Boucher, R.C. Relationship of a non-CFTR mediated chloride conductance to organ-level disease in cftr(-/ -) mice. Proc. Natl. Acad. Sci. U.S.A. 91:479-483;1994.
-
(1994)
Proc. Natl. Acad. Sci. U.S.A.
, vol.91
, pp. 479-483
-
-
Clarke, L.L.1
Grubb, B.R.2
Yankaskas, J.R.3
Cotton, C.U.4
McKenzie, A.5
Boucher, R.C.6
-
10
-
-
0028878728
-
The genetic advantage hypothesis in cystic fibrosis heterozygotes: A murine study
-
10. Cuthbert, A.W.; Halstead, J.; Ratcliff, R.; Colledge, W.H.; Evans, M.J. The genetic advantage hypothesis in cystic fibrosis heterozygotes: a murine study. J. Physiol. (Lond.) 482:449-454;1995.
-
(1995)
J. Physiol. (Lond.)
, vol.482
, pp. 449-454
-
-
Cuthbert, A.W.1
Halstead, J.2
Ratcliff, R.3
Colledge, W.H.4
Evans, M.J.5
-
11
-
-
0026730466
-
Cystic fibrosis in the mouse by targeted insertional mutagenesis
-
11. Dorin, J.R.; Dickinson, P.; Alton, E.W.F.W.; Smith, S.N.; Geddes, D.M.; Stevenson, B.J.; Kimber, W.L.; Fleming, S.; Clarke, A.R.; Hooper, M.L.; Anderson, L.; Beddington, R.S.P.; Porteous, D.J. Cystic fibrosis in the mouse by targeted insertional mutagenesis. Nature 359:211-215;1992.
-
(1992)
Nature
, vol.359
, pp. 211-215
-
-
Dorin, J.R.1
Dickinson, P.2
Alton, E.W.F.W.3
Smith, S.N.4
Geddes, D.M.5
Stevenson, B.J.6
Kimber, W.L.7
Fleming, S.8
Clarke, A.R.9
Hooper, M.L.10
Anderson, L.11
Beddington, R.S.P.12
Porteous, D.J.13
-
12
-
-
0026687233
-
Defective regulation of outwardly rectifying Cl channels by protein kinase A corrected by insertion of CFTR
-
12. Egan, M.; Flotte, T.; Afione, S.; Solow, R.; Zeitlin, P.L.; Carter, B.J.; Guggino, W.B. Defective regulation of outwardly rectifying Cl channels by protein kinase A corrected by insertion of CFTR. Nature 358:581-584;1992.
-
(1992)
Nature
, vol.358
, pp. 581-584
-
-
Egan, M.1
Flotte, T.2
Afione, S.3
Solow, R.4
Zeitlin, P.L.5
Carter, B.J.6
Guggino, W.B.7
-
13
-
-
0022007108
-
Enhanced glucose absorption in the jejunum of patients with cystic fibrosis
-
13. Frase, L.L.; Strickland, A.D.; Kachel, G.W.; Krejs, G.J. Enhanced glucose absorption in the jejunum of patients with cystic fibrosis. Gastroenterology 88:478-484;1985.
-
(1985)
Gastroenterology
, vol.88
, pp. 478-484
-
-
Frase, L.L.1
Strickland, A.D.2
Kachel, G.W.3
Krejs, G.J.4
-
14
-
-
0022516747
-
Altered regulation of airway epithelial cell chloride channels in cystic fibrosis
-
14. Frizzell, R.A.; Rechkemmer, G.; Shoemaker, R.L. Altered regulation of airway epithelial cell chloride channels in cystic fibrosis. Science 233:558-560;1986.
-
(1986)
Science
, vol.233
, pp. 558-560
-
-
Frizzell, R.A.1
Rechkemmer, G.2
Shoemaker, R.L.3
-
15
-
-
0028062781
-
Cystic fibrosis heterozygote resistance to cholera toxin in the cystic fibrosis mouse model
-
15. Gabriel, S.E.; Brigman, K.N.; Koller, B.H.; Boucher, R.C.; Stutts, M.J. Cystic fibrosis heterozygote resistance to cholera toxin in the cystic fibrosis mouse model. Science 266:107-109;1994.
-
(1994)
Science
, vol.266
, pp. 107-109
-
-
Gabriel, S.E.1
Brigman, K.N.2
Koller, B.H.3
Boucher, R.C.4
Stutts, M.J.5
-
16
-
-
0027191065
-
CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship
-
16. Gabriel, S.E.; Clarke, L.L.; Boucher, R.C.; Stutts, M.J. CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship. Nature 363:263-268;1993.
-
(1993)
Nature
, vol.363
, pp. 263-268
-
-
Gabriel, S.E.1
Clarke, L.L.2
Boucher, R.C.3
Stutts, M.J.4
-
17
-
-
0023908058
-
Rectum has abnormal ion transport but normal cAMP-binding proteins in cystic fibrosis
-
17. Goldstein, J.L.; Nash, N.T.; Al-Bazzaz, F.; Layden, T.J.; Rao, M.C. Rectum has abnormal ion transport but normal cAMP-binding proteins in cystic fibrosis. Am. J. Physiol. 254:C719-C724;1988.
-
(1988)
Am. J. Physiol.
, vol.254
-
-
Goldstein, J.L.1
Nash, N.T.2
Al-Bazzaz, F.3
Layden, T.J.4
Rao, M.C.5
-
18
-
-
0026017859
-
In vivo evidence of altered chloride but not potassium secretion in cystic fibrosis rectal mucosa
-
18. Goldstein, J.L.; Shapiro, A.B.; Rao, M.C.; Layden, T.J. In vivo evidence of altered chloride but not potassium secretion in cystic fibrosis rectal mucosa. Gastroenterology 101:1012-1019;1991.
-
(1991)
Gastroenterology
, vol.101
, pp. 1012-1019
-
-
Goldstein, J.L.1
Shapiro, A.B.2
Rao, M.C.3
Layden, T.J.4
-
19
-
-
0028915902
-
Ion transport across the jejunum in normal and cystic fibrosis mice
-
19. Grubb, B.R. Ion transport across the jejunum in normal and cystic fibrosis mice. Am. J. Physiol. 268:G505-G513;1995.
-
(1995)
Am. J. Physiol.
, vol.268
-
-
Grubb, B.R.1
-
20
-
-
0025776179
-
Failure of cholinergic stimulation to induce a secretory response from the rectal mucosa in cystic fibrosis
-
20. Hardcastle, J.; Hardcastle, P.T.; Taylor, C.J.; Goldhill, J. Failure of cholinergic stimulation to induce a secretory response from the rectal mucosa in cystic fibrosis. Gut 32:1035-1039; 1991.
-
(1991)
Gut
, vol.32
, pp. 1035-1039
-
-
Hardcastle, J.1
Hardcastle, P.T.2
Taylor, C.J.3
Goldhill, J.4
-
21
-
-
0011090980
-
Duodenal mucosal anion transport in the cystic fibrosis (CF) mouse model
-
21. Harline, M.C.; Clarke, L.L. Duodenal mucosal anion transport in the cystic fibrosis (CF) mouse model. Pediatr. Pulmonol. Suppl. 10:197;1994.
-
(1994)
Pediatr. Pulmonol. Suppl.
, vol.10
, pp. 197
-
-
Harline, M.C.1
Clarke, L.L.2
-
22
-
-
0020596438
-
Relative ion permeability of normal and cystic fibrosis nasal epithelium
-
22. Knowles, M.; Gatzy, J.; Boucher, R. Relative ion permeability of normal and cystic fibrosis nasal epithelium. J. Clin. Invest. 71:1410-1417;1983.
-
(1983)
J. Clin. Invest.
, vol.71
, pp. 1410-1417
-
-
Knowles, M.1
Gatzy, J.2
Boucher, R.3
-
23
-
-
0019809960
-
Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis
-
23. Knowles, M.R.; Gatzy, J.; Boucher, R. Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis. N. Engl. J. Med. 305:1489-1495;1981.
-
(1981)
N. Engl. J. Med.
, vol.305
, pp. 1489-1495
-
-
Knowles, M.R.1
Gatzy, J.2
Boucher, R.3
-
24
-
-
0025862979
-
Abnormal epithelial transport in cystic fibrosis jejunum
-
24. O'Loughlin, E.V.; Hunt, D.M.; Gaskin, K.J.; Stiel, D.; Bruzuszcak, I.M.; Martin, H.C.O.; Bambach, C.; Smith, R. Abnormal epithelial transport in cystic fibrosis jejunum. Am. J. Physiol. 260:G758-G763;1991.
-
(1991)
Am. J. Physiol.
, vol.260
-
-
O'Loughlin, E.V.1
Hunt, D.M.2
Gaskin, K.J.3
Stiel, D.4
Bruzuszcak, I.M.5
Martin, H.C.O.6
Bambach, C.7
Smith, R.8
-
25
-
-
0027379757
-
A severe phenotype in mice with a duplication of exon 3 in the cystic fibrosis locus
-
25. O'Neal, W.K.; Hasty, P.; McCray, Jr., P.B.; Casey, B.; Rivera-Ferez, J.; Welsh, M.J.; Beaudet, A.L.; Bradley, A. A severe phenotype in mice with a duplication of exon 3 in the cystic fibrosis locus. Hum. Mol. Genet. 2:1561-1569;1993.
-
(1993)
Hum. Mol. Genet.
, vol.2
, pp. 1561-1569
-
-
O'Neal, W.K.1
Hasty, P.2
McCray P.B., Jr.3
Casey, B.4
Rivera-Ferez, J.5
Welsh, M.J.6
Beaudet, A.L.7
Bradley, A.8
-
26
-
-
0024601741
-
Colonic and esophageal transepithelial potential difference in cystic fibrosis
-
26. Orlando, R.C.; Powell, D.W.; Groom, R.D.; Berschneider, H.M.; Boucher, R.C.; Knowles, M.R. Colonic and esophageal transepithelial potential difference in cystic fibrosis. Gastroenterology 96:1041-1048;1989.
-
(1989)
Gastroenterology
, vol.96
, pp. 1041-1048
-
-
Orlando, R.C.1
Powell, D.W.2
Groom, R.D.3
Berschneider, H.M.4
Boucher, R.C.5
Knowles, M.R.6
-
27
-
-
0020383472
-
Effect of amiloride on potential difference across rectal mucosa in cystic fibrosis patients
-
27. Patton, C.J.; Jenkins, M.Q.; Briggman, J.V.; Spicer, S.S. Effect of amiloride on potential difference across rectal mucosa in cystic fibrosis patients. Pediatr. Res. 16:1035-1036;1982.
-
(1982)
Pediatr. Res.
, vol.16
, pp. 1035-1036
-
-
Patton, C.J.1
Jenkins, M.Q.2
Briggman, J.V.3
Spicer, S.S.4
-
28
-
-
0028232830
-
Bicarbonate conductance and pH regulatory capability of cystic fibrosis transmembrane conductance regulator
-
28. Poulsen, J.H.; Fischer, H.; Illek, B.; Machen, T.E. Bicarbonate conductance and pH regulatory capability of cystic fibrosis transmembrane conductance regulator. Proc. Natl. Acad. Sci. U.S.A. 91:5340-5344;1994.
-
(1994)
Proc. Natl. Acad. Sci. U.S.A.
, vol.91
, pp. 5340-5344
-
-
Poulsen, J.H.1
Fischer, H.2
Illek, B.3
Machen, T.E.4
-
29
-
-
0002448937
-
Abnormalities in electrolyte secretion in cystic fibrosis sweat glands due to decreased anion permeability
-
Quinton, P.M.; Martinez, S.R.; Hopfer, E. (eds). San Francisco: San Francisco Press
-
29. Quinton, P.M. Abnormalities in electrolyte secretion in cystic fibrosis sweat glands due to decreased anion permeability. In: Quinton, P.M.; Martinez, S.R.; Hopfer, E. (eds). Fluid and Electrolyte Abnormalities in Exocrine Glands in Cystic Fibrosis. San Francisco: San Francisco Press; 1982:53-76.
-
(1982)
Fluid and Electrolyte Abnormalities in Exocrine Glands in Cystic Fibrosis
, pp. 53-76
-
-
Quinton, P.M.1
-
30
-
-
0020700816
-
Chloride impermeability in cystic fibrosis
-
30. Quinton, P.M. Chloride impermeability in cystic fibrosis. Nature 301:421-422;1983.
-
(1983)
Nature
, vol.301
, pp. 421-422
-
-
Quinton, P.M.1
-
31
-
-
0027172739
-
Production of a severe cystic fibrosis mutation in mice by gene targeting
-
31. Ratcliff, R.; Evans, M.J.; Cuthbert, A.W.; MacVinish, L.J.; Foster, D.; Anderson, J.R.; Colledge, W.H. Production of a severe cystic fibrosis mutation in mice by gene targeting. Nat. Genet. 4:35-41;1993.
-
(1993)
Nat. Genet.
, vol.4
, pp. 35-41
-
-
Ratcliff, R.1
Evans, M.J.2
Cuthbert, A.W.3
MacVinish, L.J.4
Foster, D.5
Anderson, J.R.6
Colledge, W.H.7
-
32
-
-
0024424270
-
Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
-
32. Riordan, J.R.; Rommens, J.M.; Kerem, B.; Alon, N.; Rozmahel, R.; Grzelczak, Z.; Zielenski, J.; Lok, S.; Plavsic, N.; Chou, J.; Drumm, M.L.; Iannuzzi, M.C.; Collins, F.S.; Tsui, L. Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA. Science 245:1066-1073;1989.
-
(1989)
Science
, vol.245
, pp. 1066-1073
-
-
Riordan, J.R.1
Rommens, J.M.2
Kerem, B.3
Alon, N.4
Rozmahel, R.5
Grzelczak, Z.6
Zielenski, J.7
Lok, S.8
Plavsic, N.9
Chou, J.10
Drumm, M.L.11
Iannuzzi, M.C.12
Collins, F.S.13
Tsui, L.14
-
33
-
-
0028919433
-
Bioelectric characteristics of exon 10 insertional cystic fibrosis mouse: Comparison with humans
-
33. Smith, S.N.; Steel, D.M.; Middleton, P.C.; Munkonge, F.M.; Geddes, D.M.; Caplen, N.J.; Porteous, D.J.; Dorin, J.R.; Alton, E.W.F.W. Bioelectric characteristics of exon 10 insertional cystic fibrosis mouse: comparison with humans. Am. J. Physiol. 268:C297-C307;1995.
-
(1995)
Am. J. Physiol.
, vol.268
-
-
Smith, S.N.1
Steel, D.M.2
Middleton, P.C.3
Munkonge, F.M.4
Geddes, D.M.5
Caplen, N.J.6
Porteous, D.J.7
Dorin, J.R.8
Alton, E.W.F.W.9
-
34
-
-
0026649122
-
An animal model for cystic fibrosis made by gene targeting
-
34. Snouwaert, J.; Brigman, K.K.; Latour, A.M.; Malouf, N.N.; Boucher, R.C.; Smithies, O.; Koller, B.H. An animal model for cystic fibrosis made by gene targeting. Science 257:1083-1088;1992.
-
(1992)
Science
, vol.257
, pp. 1083-1088
-
-
Snouwaert, J.1
Brigman, K.K.2
Latour, A.M.3
Malouf, N.N.4
Boucher, R.C.5
Smithies, O.6
Koller, B.H.7
-
35
-
-
0023730817
-
Failure to induce secretion in jejunal biopsies from children with cystic fibrosis
-
35. Taylor, C.J.; Baxter, P.S.; Hardcastle, J.; Hardcastle, P.T. Failure to induce secretion in jejunal biopsies from children with cystic fibrosis. Gut 29:957-962;1988.
-
(1988)
Gut
, vol.29
, pp. 957-962
-
-
Taylor, C.J.1
Baxter, P.S.2
Hardcastle, J.3
Hardcastle, P.T.4
-
36
-
-
0028218460
-
Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype
-
36. Veeze, H.J.; Halley, D.J.; Bijman, J.; de Jongste, J.C.; de Jonge, H.R.; Sinaasappel, M. Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype. J. Clin. Invest. 93:461-466;1994.
-
(1994)
J. Clin. Invest.
, vol.93
, pp. 461-466
-
-
Veeze, H.J.1
Halley, D.J.2
Bijman, J.3
De Jongste, J.C.4
De Jonge, H.R.5
Sinaasappel, M.6
-
37
-
-
0025772974
-
Ion transport abnormalities in rectal suction biopsies from children with cystic fibrosis
-
37. Veeze, H.J.; Sinaasappel, M.; Bijman, J.; Bouquet, J.; de Jonge, H.R. Ion transport abnormalities in rectal suction biopsies from children with cystic fibrosis. Gastroenterology 101:398-403;1991.
-
(1991)
Gastroenterology
, vol.101
, pp. 398-403
-
-
Veeze, H.J.1
Sinaasappel, M.2
Bijman, J.3
Bouquet, J.4
De Jonge, H.R.5
-
38
-
-
0022518142
-
Chloride and potassium channels in cystic fibrosis airway epithelia
-
38. Welsh, M.J.; Liedtke, C.M. Chloride and potassium channels in cystic fibrosis airway epithelia. Nature 322:467-470;1986.
-
(1986)
Nature
, vol.322
, pp. 467-470
-
-
Welsh, M.J.1
Liedtke, C.M.2
-
39
-
-
0027470092
-
+/glucose cotransporter
-
+/glucose cotransporter. Annu. Rev. Physiol. 55:575-589;1993.
-
(1993)
Annu. Rev. Physiol.
, vol.55
, pp. 575-589
-
-
Wright, E.M.1
|