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Volumn 79, Issue 1 SUPPL. 1, 1999, Pages

Pathophysiology of gene-targeted mouse models for cystic fibrosis

Author keywords

[No Author keywords available]

Indexed keywords

AMILORIDE; BUTYRIC ACID; CALCIUM ION; CHLORIDE ION; CYCLIC AMP; CYCLIC AMP DERIVATIVE; LIPOSOME; LOPERAMIDE; MESSENGER RNA; MILRINONE; SODIUM ION; TRANSMEMBRANE CONDUCTANCE REGULATOR;

EID: 0032962320     PISSN: 00319333     EISSN: None     Source Type: Journal    
DOI: 10.1152/physrev.1999.79.1.S193     Document Type: Review
Times cited : (355)

References (133)
  • 2
    • 0025863209 scopus 로고
    • Generation of cAMP-activated chloride currents by expression of CFTR
    • ANDERSON, M. P., D. P. RICH, R. J. GREGORY, A. E. SMITH, AND M. J. WELSH. Generation of cAMP-activated chloride currents by expression of CFTR. Science 251: 679-682, 1991.
    • (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
  • 3
    • 0025871551 scopus 로고
    • Calcium and cAMP activate different chloride channels in the apical membrane of normal and cystic fibrosis epithelia
    • ANDERSON, M. P., AND M. J. WELSH. Calcium and cAMP activate different chloride channels in the apical membrane of normal and cystic fibrosis epithelia. Proc. Natl. Acad. Sci. USA 88: 6003-6007, 1991.
    • (1991) Proc. Natl. Acad. Sci. USA , vol.88 , pp. 6003-6007
    • Anderson, M.P.1    Welsh, M.J.2
  • 4
    • 0001851591 scopus 로고
    • Cellular mechanisms of ion transport in the small intestine
    • edited by L. R. Johnson. New York: Raven
    • ARMSTRONG, W. M. Cellular mechanisms of ion transport in the small intestine. In: Physiology of the Gastrointestinal Tract, edited by L. R. Johnson. New York: Raven, 1987, p. 1259
    • (1987) Physiology of the Gastrointestinal Tract , pp. 1259
    • Armstrong, W.M.1
  • 7
    • 0025300405 scopus 로고
    • Enhanced intestinal glucose and alanine transport in cystic fibrosis
    • BAXTER, P., J. GOLDHILL, J. HARDCASTLE, P. T. HARDCASTLE, AND C. J. TAYLOR. 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
  • 8
    • 0026532895 scopus 로고
    • Purification and functional reconstitution of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)
    • BEAR, C. E., C. LI, N. KARTNER, R. J. BRIDGES, T. J. JENSEN, M. RAMJEESINGH, AND J. R. RIORDAN. Purification and functional reconstitution of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). Cell 68: 809-818, 1992.
    • (1992) Cell , vol.68 , pp. 809-818
    • Bear, C.E.1    Li, C.2    Kartner, N.3    Bridges, R.J.4    Jensen, T.J.5    Ramjeesingh, M.6    Riordan, J.R.7
  • 10
    • 0023616360 scopus 로고
    • Hyposecretion of β-adrenergically induced sweating in cystic fibrosis heterozygotes
    • BEHM, J. K., G. HAGIWARA, N. J. LEWISTON, P. M. QUINTON, AND J. J. WINE. Hyposecretion of β-adrenergically induced sweating in cystic fibrosis heterozygotes. Pediatr. Res. 22: 271-276, 1987.
    • (1987) Pediatr. Res. , vol.22 , pp. 271-276
    • Behm, J.K.1    Hagiwara, G.2    Lewiston, N.J.3    Quinton, P.M.4    Wine, J.J.5
  • 13
    • 0002314552 scopus 로고
    • Cystic fibrosis
    • edited by C. R. Scriver, A. L. Beaudet, W. S. Sly, D. Valle, J. B. Stansbury, J. B. Wyngaarden, and D. S. Fredrickson. New York: McGraw-Hill
    • BOAT, T. F., M. J. WELSH, AND A. L. BEAUDET. Cystic fibrosis. In: The Metabolic Basis of Inherited Disease, edited by C. R. Scriver, A. L. Beaudet, W. S. Sly, D. Valle, J. B. Stansbury, J. B. Wyngaarden, and D. S. Fredrickson. New York: McGraw-Hill, 1989, p. 2649-2680.
    • (1989) The Metabolic Basis of Inherited Disease , pp. 2649-2680
    • Boat, T.F.1    Welsh, M.J.2    Beaudet, A.L.3
  • 14
    • 0028074748 scopus 로고
    • Pathophysiology of gastrointestinal complications of cystic fibrosis
    • BOROWITZ, D. Pathophysiology of gastrointestinal complications of cystic fibrosis. Semin. Respir. Crit. Care Med, 15: 391-401, 1994.
    • (1994) Semin. Respir. Crit. Care Med , vol.15 , pp. 391-401
    • Borowitz, D.1
  • 15
    • 0024465128 scopus 로고
    • Chloride secretory response of cystic fibrosis human airway epithelia: Preservation of calcium but not protein kinase C-and A-dependent mechanisms
    • BOUCHER, R. C., E. H. C. CHENG, A. M. PARADISO, M. J. STUTTS, M. R. KNOWLES, AND H. S. EARP. 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
  • 16
  • 18
    • 0024345843 scopus 로고
    • Increased sulfation of glycoconjugates by cultured nasal epithelial cells from patients with cystic fibrosis
    • CHENG, P., T. F. BOAT, K. CRANFILL, J. R. YANKASKAS, AND R. C. BOUCHER. Increased sulfation of glycoconjugates by cultured nasal epithelial cells from patients with cystic fibrosis. J. Clin. Invest. 84: 68-72, 1989.
    • (1989) J. Clin. Invest. 84 , pp. 68-72
    • Cheng, P.1    Boat, T.F.2    Cranfill, K.3    Yankaskas, J.R.4    Boucher, R.C.5
  • 20
    • 0025242929 scopus 로고
    • Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
    • CHENG, S. H., R. J. GREGORY, J. MARSHALL, S. PAUL, D. W. SOUZA, G. A. WHITE, C. O'RIORDAN, AND A. E. SMITH. Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell 63: 827-834, 1990.
    • (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.7    Smith, A.E.8
  • 21
    • 0026640380 scopus 로고
    • Defective epithelial chloride transport in a gene targeted mouse model of cystic fibrosis
    • CLARKE, L. L., B. R. GRUBB, S. E. GABRIEL, O. SMITHIES, B. H. KOLLER, AND R. C. BOUCHER. 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
  • 26
    • 0002234247 scopus 로고
    • Secretion by the major salivary glands
    • edited by L. R. Johnson, D. H. Alpers, J. Christensen, E. D. Jacobson, and J. H. Walsh. New York: Raven
    • COOK, D. I., E. W. VAN LENNEP, M. L. ROBERTS, AND J. A. YOUNG. Secretion by the major salivary glands. In: Physiology of the Gastrointestinal Tract, edited by L. R. Johnson, D. H. Alpers, J. Christensen, E. D. Jacobson, and J. H. Walsh. New York: Raven, 1994, p. 1061-1118.
    • (1994) Physiology of the Gastrointestinal Tract , pp. 1061-1118
    • Cook, D.I.1    Van Lennep, E.W.2    Roberts, M.L.3    Young, J.A.4
  • 27
    • 0344868184 scopus 로고
    • cAMP inhibits fluid absorption in normal and CF mouse intestine
    • COTTON, C. U. cAMP inhibits fluid absorption in normal and CF mouse intestine (Abstract). Pediatr. Pulmonol. Suppl. 12: 194, 1995.
    • (1995) Pediatr. Pulmonol. Suppl. , vol.12 , pp. 194
    • Cotton, C.U.1
  • 28
  • 29
    • 0028131769 scopus 로고
    • Ion-transporting activity in the murine colonic epithelium of normal animals and animals with cystic fibrosis
    • CUTHBERT, A. W., L. J. MacVINISH, M. E. HICKMAN, R. RATCLIFF, W. H. COLLEDGE, AND M. J. EVANS. Ion-transporting activity in the murine colonic epithelium of normal animals and animals with cystic fibrosis. Pflügers Arch. 428: 508-515, 1994.
    • (1994) Pflügers Arch. , vol.428 , pp. 508-515
    • Cuthbert, A.W.1    Macvinish, L.J.2    Hickman, M.E.3    Ratcliff, R.4    Colledge, W.H.5    Evans, M.J.6
  • 33
    • 0025991109 scopus 로고
    • Defective regulation of electrolyte and protein secretion in submandibular saliva of cystic fibrosis patients
    • DAVIES, H., J. BAGG, M. C. GOODCHILD, AND M. A. McPHERSON. Defective regulation of electrolyte and protein secretion in submandibular saliva of cystic fibrosis patients. Acta Paediatr. Scand. 80: 1094-1095, 1991.
    • (1991) Acta Paediatr. Scand. , vol.80 , pp. 1094-1095
    • Davies, H.1    Bagg, J.2    Goodchild, M.C.3    McPherson, M.A.4
  • 34
    • 0344005143 scopus 로고    scopus 로고
    • CFTR is detected immunologically in intestinal villi and is required for cAMP-and cGMP-inhibition of fluid absorption in the jejunum but not in the ileum
    • DE JONGE, H. R., N. A. AMEEN, W. E. M. BOOMAARS, P. J. FRENCH, J. BIJMAN, B. SCHOLTE, AND C. R. MARINO. CFTR is detected immunologically in intestinal villi and is required for cAMP-and cGMP-inhibition of fluid absorption in the jejunum but not in the ileum (Abstract). Pediatr. Pulmonol. Suppl. 13: 218, 1996.
    • (1996) Pediatr. Pulmonol. Suppl. , vol.13 , pp. 218
    • De Jonge, H.R.1    Ameen, N.A.2    Boomaars, W.E.M.3    French, P.J.4    Bijman, J.5    Scholte, B.6    Marino, C.R.7
  • 37
    • 0028929793 scopus 로고
    • Increased expression of sulfated gp300 and acinar tissue pathology in pancreas of CFTR(-/-) mice
    • DE LISLE, R. C. Increased expression of sulfated gp300 and acinar tissue pathology in pancreas of CFTR(-/-) mice. Am. J. Physiol. 268 (Gastrointest. Liver Physiol. 31): G717-G723, 1995.
    • (1995) Am. J. Physiol. 268 (Gastrointest. Liver Physiol. 31) , vol.268
    • De Lisle, R.C.1
  • 38
    • 0026781952 scopus 로고
    • Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
    • DENNING, G. M., M. P. ANDERSON, J. F. AMARA, J. MARSHALL, A. E. SMITH, AND M. J. WELSH. Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive. Nature 358: 761-764, 1992.
    • (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
  • 40
    • 0028003254 scopus 로고
    • Long-term survival of the exon 10 insertional cystic fibrosis mutant mouse is a consequence of low level residual wild-type CFTR gene expression
    • DORIN, J. R., B. J. STEVENSON, S. FLEMING, E. W. F. W. ALTON, P. DICKINSON, AND D. J. PORTEOUS. Long-term survival of the exon 10 insertional cystic fibrosis mutant mouse is a consequence of low level residual wild-type CFTR gene expression. Mamm. Genome 5: 465-472, 1994.
    • (1994) Mamm. Genome , vol.5 , pp. 465-472
    • Dorin, J.R.1    Stevenson, B.J.2    Fleming, S.3    Alton, E.W.F.W.4    Dickinson, P.5    Porteous, D.J.6
  • 43
    • 0025951832 scopus 로고
    • Small-intestinal abnormalities in cystic fibrosis patients
    • EGGERMONT, E., AND K. DE BOECK. Small-intestinal abnormalities in cystic fibrosis patients. Eur. J. Pediatr. 150: 824-828, 1991.
    • (1991) Eur. J. Pediatr. , vol.150 , pp. 824-828
    • Eggermont, E.1    De Boeck, K.2
  • 45
    • 0344921091 scopus 로고
    • Intracellular mediators of secretion in the small intestine
    • edited by H. J. Binder. New York: Liss
    • FIELD, M. Intracellular mediators of secretion in the small intestine. In: Mechanisms of Intestinal Secretion, edited by H. J. Binder. New York: Liss, 1979, p. 83-91.
    • (1979) Mechanisms of Intestinal Secretion , pp. 83-91
    • Field, M.1
  • 47
    • 0027523995 scopus 로고
    • Regulation of membrane chloride currents in rat bile duct epithelial cells
    • FITZ, J. G., S. BASAVAPPA, J. McGILL, O. MELHUS, AND J. A. COHN. Regulation of membrane chloride currents in rat bile duct epithelial cells. J. Clin. Invest. 91: 319-328, 1993.
    • (1993) J. Clin. Invest. , vol.91 , pp. 319-328
    • Fitz, J.G.1    Basavappa, S.2    McGill, J.3    Melhus, O.4    Cohn, J.A.5
  • 48
    • 0022007108 scopus 로고
    • Enhanced glucose absorption in the jejunum of patients with cystic fibrosis
    • FRASE, L. L., A. D. STRICKLAND, G. W. KACHEL, AND G. J. KREJS. Enhanced glucose absorption in the jejunum of patients with cystic fibrosis. Gastroenterotogy 88: 478-484, 1985.
    • (1985) Gastroenterotogy , vol.88 , pp. 478-484
    • Frase, L.L.1    Strickland, A.D.2    Kachel, G.W.3    Krejs, G.J.4
  • 50
    • 0028940005 scopus 로고
    • Functions of the cystic fibrosis transmembrane conductance regulator protein
    • FRIZZELL, R. A. Functions of the cystic fibrosis transmembrane conductance regulator protein. Am. J. Respir. Crit. Care Med. 151, Suppl.: S54-S58, 1995.
    • (1995) Am. J. Respir. Crit. Care Med. , vol.151 , Issue.SUPPL.
    • Frizzell, R.A.1
  • 51
    • 0022516747 scopus 로고
    • Altered regulation of airway epithelial cell chloride channels in cystic fibrosis
    • FRIZZELL, R. A., G. RECHKEMMER, AND R. L. SHOEMAKER. 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
  • 52
    • 0028062781 scopus 로고
    • Cystic fibrosis heterozygote resistance to cholera toxin in the cystic fibrosis mouse model
    • GABRIEL, S. E., K. N. BRIGMAN, B. H. KOLLER, R. C. BOUCHER, AND M. J. STUTTS. 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
  • 53
    • 0027191065 scopus 로고
    • CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship
    • GABRIEL, S. E., L. L. CLARKE, R. C. BOUCHER, AND M. J. STUTTS. 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
  • 54
    • 0029825547 scopus 로고    scopus 로고
    • Modification of nasal membrane potential difference with inhaled amiloride and loperamide in the cystic fibrosis (CF) mouse
    • GHOSAL, S., C. J. TAYLOR, AND J. McGAW. Modification of nasal membrane potential difference with inhaled amiloride and loperamide in the cystic fibrosis (CF) mouse. Thorax 51: 1229-1232, 1996.
    • (1996) Thorax , vol.51 , pp. 1229-1232
    • Ghosal, S.1    Taylor, C.J.2    McGaw, J.3
  • 56
    • 0026017859 scopus 로고
    • In vivo evidence of altered chloride but not potassium secretion in cystic fibrosis rectal mucosa
    • GOLDSTEIN, J. L., A. B. SHAPIRO, M. C. RAO, AND T. J. LAYDEN. 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
  • 59
    • 0031259876 scopus 로고    scopus 로고
    • Ion transport across the murine intestine in the absence and presence of CFTR
    • GRUBB, B. R. Ion transport across the murine intestine in the absence and presence of CFTR. Comp. Biochem. Physiol. A Physiol. 188: 277-283, 1997.
    • (1997) Comp. Biochem. Physiol. A Physiol. , vol.188 , pp. 277-283
    • Grubb, B.R.1
  • 64
    • 0022506787 scopus 로고
    • Role of aldosterone in the regulation of sodium and chloride transport in the distal colon of sodium-depleted rats
    • HALEVY, J., M. E. BUDINGER, J. P. HAYSLETT, AND H. J. BINDER. Role of aldosterone in the regulation of sodium and chloride transport in the distal colon of sodium-depleted rats. Gastroenterology 91: 1227-1233, 1986.
    • (1986) Gastroenterology , vol.91 , pp. 1227-1233
    • Halevy, J.1    Budinger, M.E.2    Hayslett, J.P.3    Binder, H.J.4
  • 66
    • 0025776179 scopus 로고
    • Failure of cholinergic stimulation to induce a secretory response from the rectal mucosa in cystic fibrosis
    • HARDCASTLE, J., P. T. HARDCASTLE, C. J. TAYLOR, AND J. GOLDHILL. 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
  • 67
    • 0026347526 scopus 로고
    • Comparative aspects of nasal airway anatomy: Relevance to inhalation toxicology
    • HARKEMA, J. R. Comparative aspects of nasal airway anatomy: relevance to inhalation toxicology. Toxicol. Pathol. 19: 321-336, 1991.
    • (1991) Toxicol. Pathol. , vol.19 , pp. 321-336
    • Harkema, J.R.1
  • 69
    • 0011090980 scopus 로고
    • Duodenal mucosal anion transport in the cystic fibrosis (CF) mouse model
    • HARLINE, M. C., AND L. L. CLARKE. Duodenal mucosal anion transport in the cystic fibrosis (CF) mouse model (Abstract). Pediatr. Pulmonol. Suppl. 10: 197, 1994.
    • (1994) Pediatr. Pulmonol. Suppl. , vol.10 , pp. 197
    • Harline, M.C.1    Clarke, L.L.2
  • 71
    • 0025043485 scopus 로고
    • Vasa aplasia and cystic fibrosis
    • HEATON, N. D., AND J. P. PRYOR. Vasa aplasia and cystic fibrosis. Br. J. Urol. 66: 538-540, 1990.
    • (1990) Br. J. Urol. , vol.66 , pp. 538-540
    • Heaton, N.D.1    Pryor, J.P.2
  • 74
    • 0029890425 scopus 로고    scopus 로고
    • Exocrine pancreatic alterations in long-lived surviving cystic fibrosis mice
    • IP, W. F., I. BRONSVELD, G. KENT, M. COREY, AND P. R. DURIE. Exocrine pancreatic alterations in long-lived surviving cystic fibrosis mice. Pediatr. Res. 40: 242-249, 1996.
    • (1996) Pediatr. Res. , vol.40 , pp. 242-249
    • Ip, W.F.1    Bronsveld, I.2    Kent, G.3    Corey, M.4    Durie, P.R.5
  • 75
    • 0028912888 scopus 로고
    • 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
    • JOHNSON, L. G., S. E. BOYLES, J. WILSON, AND R. C. BOUCHER. 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. J. Clin. Invest. 95: 1377-1382, 1995.
    • (1995) J. Clin. Invest. , vol.95 , pp. 1377-1382
    • Johnson, L.G.1    Boyles, S.E.2    Wilson, J.3    Boucher, R.C.4
  • 77
    • 0030900255 scopus 로고    scopus 로고
    • In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant delta F508 in murine nasal epithelium
    • KELLEY, T. J., K. THOMAS, L. J. H. MILGRAM, AND M. L. DRUMM. In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant delta F508 in murine nasal epithelium. Proc. Natl. Acad. Sci. USA 94: 2604-2608, 1997.
    • (1997) Proc. Natl. Acad. Sci. USA , vol.94 , pp. 2604-2608
    • Kelley, T.J.1    Thomas, K.2    Milgram, L.J.H.3    Drumm, M.L.4
  • 80
    • 0019809960 scopus 로고
    • Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis
    • KNOWLES, M., J. GATZY, AND R. BOUCHER. 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.1    Gatzy, J.2    Boucher, R.3
  • 81
    • 0020596438 scopus 로고
    • Relative ion permeability of normal and cystic fibrosis nasal epithelium
    • KNOWLES, M., J. GATZY, AND R. BOUCHER. 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
  • 82
    • 0025874599 scopus 로고
    • Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis
    • KNOWLES, M. R., L. L. CLARKE, AND R. C. BOUCHER. Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis. N. Engl. J. Med. 325: 533-538, 1991.
    • (1991) N. Engl. J. Med. , vol.325 , pp. 533-538
    • Knowles, M.R.1    Clarke, L.L.2    Boucher, R.C.3
  • 84
    • 0020610435 scopus 로고
    • Abnormal ion permeation through cystic fibrosis respiratory epithelium
    • KNOWLES, M. R., M. J. STUTTS, A. SPOCK, N. FISCHER, J. T. GATZY, AND R. C. BOUCHER. Abnormal ion permeation through cystic fibrosis respiratory epithelium. Science 221: 1067-1070, 1983.
    • (1983) Science , vol.221 , pp. 1067-1070
    • Knowles, M.R.1    Stutts, M.J.2    Spock, A.3    Fischer, N.4    Gatzy, J.T.5    Boucher, R.C.6
  • 85
    • 0026689409 scopus 로고
    • Altering genes in animals by gene targeting
    • KOLLER, B. H., AND O. SMITHIES. Altering genes in animals by gene targeting. Annu. Rev. Immunol. 10: 705-730, 1992.
    • (1992) Annu. Rev. Immunol. , vol.10 , pp. 705-730
    • Koller, B.H.1    Smithies, O.2
  • 88
    • 0027483610 scopus 로고
    • The cystic fibrosis mutation (Delta-F508) does not influence the chloride channel activity of CFTR
    • LI, C., M. RAMJEESINGH, E. REYES, T. JENSEN, X. CHANG, J. M. ROMMENS, AND C. E. BEAR. The cystic fibrosis mutation (Delta-F508) does not influence the chloride channel activity of CFTR. Nature Genet. 3: 311-316, 1993.
    • (1993) Nature Genet. , vol.3 , pp. 311-316
    • Li, C.1    Ramjeesingh, M.2    Reyes, E.3    Jensen, T.4    Chang, X.5    Rommens, J.M.6    Bear, C.E.7
  • 93
    • 0016417460 scopus 로고
    • Pathology of cystic fibrosis; review of the literature and comparison with 146 autopsied cases
    • edited by H. S. Rosenberg and R. Bolande. New York: Year Book
    • OPPENHEIMER, E. H., AND J. R. ESTERLY. Pathology of cystic fibrosis; review of the literature and comparison with 146 autopsied cases. In: Perspectives in Pediatrie Pathology, edited by H. S. Rosenberg and R. Bolande. New York: Year Book, 1976, p. 241-278.
    • (1976) Perspectives in Pediatrie Pathology , pp. 241-278
    • Oppenheimer, E.H.1    Esterly, J.R.2
  • 95
    • 0019364354 scopus 로고
    • The cells of the tracheobronchial epithelium of the mouse: A quantitative light and electron microscope study
    • PACK, R. J., L. H. AL-UGAILY, AND G. MORRIS. The cells of the tracheobronchial epithelium of the mouse: a quantitative light and electron microscope study. J. Anat. 132: 71-84, 1981.
    • (1981) J. Anat. , vol.132 , pp. 71-84
    • Pack, R.J.1    Al-Ugaily, L.H.2    Morris, G.3
  • 96
    • 0019441929 scopus 로고
    • Gastrointestinal manifestations of cystic fibrosis: A review
    • PARK, R. W., AND R. J. GRAND. Gastrointestinal manifestations of cystic fibrosis: a review. Gastroenterology 81: 1143-1161, 1981.
    • (1981) Gastroenterology , vol.81 , pp. 1143-1161
    • Park, R.W.1    Grand, R.J.2
  • 97
    • 0028977988 scopus 로고
    • Mutant (deltaF508) cystic fibrosis transmembrane conductance regulator Cl channel is functional when retained in endoplasmic reticulum of mammalian cells
    • PASYK, E. A., AND J. K. FOSKETT. Mutant (deltaF508) cystic fibrosis transmembrane conductance regulator Cl channel is functional when retained in endoplasmic reticulum of mammalian cells. J. Biol. Chem. 270: 12347-12350, 1995.
    • (1995) J. Biol. Chem. , vol.270 , pp. 12347-12350
    • Pasyk, E.A.1    Foskett, J.K.2
  • 99
    • 0031015968 scopus 로고    scopus 로고
    • Cystic fibrosis transmembrane conductance regulator mediates the cyclic adenosine monophosphate-induced fluid secretion but not the inhibition of resorption in mouse gallbladder epithelium
    • PETERS, R. H. P. C., J. H. VAN DOORNINCK, P. J. FRENCH, R. RATCLIFF, M. J. EVANS, W. H. COLLEDGE, J. BIJMAN, AND B. J. SCHOLTE. Cystic fibrosis transmembrane conductance regulator mediates the cyclic adenosine monophosphate-induced fluid secretion but not the inhibition of resorption in mouse gallbladder epithelium. Hepatology 25: 270-277, 1997.
    • (1997) Hepatology , vol.25 , pp. 270-277
    • Peters, R.H.P.C.1    Van Doorninck, J.H.2    French, P.J.3    Ratcliff, R.4    Evans, M.J.5    Colledge, W.H.6    Bijman, J.7    Scholte, B.J.8
  • 100
    • 0019126848 scopus 로고
    • Tetracycline discoloration, enamel defects, and dental caries in patients with cystic fibrosis
    • PRIMOSCH, R. E. fetracycline discoloration, enamel defects, and dental caries in patients with cystic fibrosis. Oral Surg. 50: 301-308, 1980.
    • (1980) Oral Surg. , vol.50 , pp. 301-308
    • Primosch, R.E.1
  • 101
    • 0020700816 scopus 로고
    • Chloride impermeability in cystic fibrosis
    • QUINTON, P. M. Chloride impermeability in cystic fibrosis. Nature 301: 421-422, 1983.
    • (1983) Nature , vol.301 , pp. 421-422
    • Quinton, P.M.1
  • 102
    • 0020533435 scopus 로고
    • Higher bioelectric potentials due to decreased chloride absorption in the sweat glands of patients with cystic fibrosis
    • QUINTON, P. M., AND J. BIJMAN. Higher bioelectric potentials due to decreased chloride absorption in the sweat glands of patients with cystic fibrosis. N. Engl. J. Med. 308: 1185-1189, 1983.
    • (1983) N. Engl. J. Med. , vol.308 , pp. 1185-1189
    • Quinton, P.M.1    Bijman, J.2
  • 104
    • 0025155528 scopus 로고
    • Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells
    • RICH, D. P., M. P. ANDERSON, R. J. GREGORY, S. H. CHENG, S. PAUL, D. M. JEFFERSON, J. D. McCANN, K. W. KLINGER, A. E. SMITH, AND M. J. WELSH. Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells. Nature 347:358-363, 1990.
    • (1990) Nature , vol.347 , pp. 358-363
    • Rich, D.P.1    Anderson, M.P.2    Gregory, R.J.3    Cheng, S.H.4    Paul, S.5    Jefferson, D.M.6    McCann, J.D.7    Klinger, K.W.8    Smith, A.E.9    Welsh, M.J.10
  • 108
    • 0023885474 scopus 로고
    • Variable reduction in beta-adrenergic sweat secretion in cystic fibrosis heterozygotes
    • SATO, K., AND F. SATO. Variable reduction in beta-adrenergic sweat secretion in cystic fibrosis heterozygotes. J. Lab. Clin. Med. 111: 511-518, 1988.
    • (1988) J. Lab. Clin. Med. , vol.111 , pp. 511-518
    • Sato, K.1    Sato, F.2
  • 109
    • 0030042386 scopus 로고    scopus 로고
    • Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation
    • SATO, S., C. L. WARD, M. E. KROUSE, J. J. WINE, AND R. R. KOPITO. Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation. J. Biol. Chem. 271: 635-638, 1996.
    • (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
  • 110
    • 0028980536 scopus 로고
    • CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP
    • SCHWIEBERT, E. M., M. E. EGAN, T. HWANG, S. B. FULMER, S. S. ALLEN, G. R. CUTTING, AND W. B. GUGGINO. CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP. Cell 81: 1063-1073, 1995.
    • (1995) Cell , vol.81 , pp. 1063-1073
    • Schwiebert, E.M.1    Egan, M.E.2    Hwang, T.3    Fulmer, S.B.4    Allen, S.S.5    Cutting, G.R.6    Guggino, W.B.7
  • 117
    • 0023730817 scopus 로고
    • Failure to induce secretion in jejunal biopsies from children with cystic fibrosis
    • TAYLOR, C. J., P. S. BAXTER, J. HARDCASTLE, AND P. T. HARDCASTLE. 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
  • 120
    • 0025772974 scopus 로고
    • Ion transport abnormalities in rectal suction biopsies from children with cystic fibrosis
    • VEEZE, H. J., M. SINAASAPPEL, J. BIJMAN, J. BOUQUET, AND H. R. DE JONGE. 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
  • 121
    • 0027162649 scopus 로고
    • Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis
    • WELSH, M. J., AND A. E. SMITH. Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. Cell 73: 1251-1254, 1993.
    • (1993) Cell , vol.73 , pp. 1251-1254
    • Welsh, M.J.1    Smith, A.E.2
  • 123
    • 0028819164 scopus 로고
    • Regulation of human airway surface liquid
    • WIDDICOMBE, J. H., AND J. G. WIDDICOMBE. Regulation of human airway surface liquid. Respir. Physiol. 99: 3-12, 1995.
    • (1995) Respir. Physiol. , vol.99 , pp. 3-12
    • Widdicombe, J.H.1    Widdicombe, J.G.2
  • 126
    • 0030276150 scopus 로고    scopus 로고
    • Enamel mineral composition of normal and cystic fibrosis transgenic mice
    • WRIGHT, J. T., K. I. HALL, AND B. R. GRUBB. Enamel mineral composition of normal and cystic fibrosis transgenic mice. Adv. Dent. Res. 10: 270-275, 1996.
    • (1996) Adv. Dent. Res. , vol.10 , pp. 270-275
    • Wright, J.T.1    Hall, K.I.2    Grubb, B.R.3
  • 127
    • 0030307289 scopus 로고    scopus 로고
    • Abnormal enamel development in a cystic fibrosis transgenic mouse model
    • WRIGHT, J. T., C. L. KIEFER, K. I. HALL, AND B. R. GRUBB. Abnormal enamel development in a cystic fibrosis transgenic mouse model. J. Dent. Res. 75: 966-973, 1996.
    • (1996) J. Dent. Res. , vol.75 , pp. 966-973
    • Wright, J.T.1    Kiefer, C.L.2    Hall, K.I.3    Grubb, B.R.4
  • 128
    • 0028170353 scopus 로고
    • Correction of cAMP-stimulated fluid secretion in cystic fibrosis airway epithelia: Efficiency of adenovirus-mediated gene transfer in vitro
    • ZABNER, J., L. A. COUTURE, A. E. SMITH, AND M. J. WELSH. Correction of cAMP-stimulated fluid secretion in cystic fibrosis airway epithelia: efficiency of adenovirus-mediated gene transfer in vitro. Hum. Gene Ther. 5: 585-593, 1994.
    • (1994) Hum. Gene Ther. , vol.5 , pp. 585-593
    • Zabner, J.1    Couture, L.A.2    Smith, A.E.3    Welsh, M.J.4
  • 130
    • 0029820857 scopus 로고    scopus 로고
    • Adenovirus-mediated gene transfer to ciliated airway epithelia requires prolonged incubation time
    • ZABNER, J., B. G. ZEIHER, E. FRIEDMAN, AND M. J. WELSH. Adenovirus-mediated gene transfer to ciliated airway epithelia requires prolonged incubation time. J. Virol. 70: 6994-7003, 1996.
    • (1996) J. Virol. , vol.70 , pp. 6994-7003
    • Zabner, J.1    Zeiher, B.G.2    Friedman, E.3    Welsh, M.J.4
  • 133
    • 0028557095 scopus 로고
    • Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR
    • ZHOU, L, C. R. DEY, S. E. WERT, M. D. DUVALL, R. A. FRIZZELL, AND J. A. WHITSETT. Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR. Science 266: 1705-1708, 1994.
    • (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


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