Intracellular CFTR: Localization and function

Physiological Reviews

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

  Bradbury, Neil A ^a  

^a UNIVERSITY OF PITTSBURGH SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CHLORIDE CHANNEL; CYCLIC AMP; PROTON TRANSPORTING ADENOSINE TRIPHOSPHATASE; TRANSMEMBRANE CONDUCTANCE REGULATOR;

ACIDIFICATION; BACTERIAL COLONIZATION; CELL PH; CELL VACUOLE; CELLULAR DISTRIBUTION; CYSTIC FIBROSIS; ENDOCYTOSIS; ENDOSOME; EXOCYTOSIS; GOLGI COMPLEX; HUMAN; MEMBRANE VESICLE; NONHUMAN; PRIORITY JOURNAL; PROTEIN LOCALIZATION; PROTEIN PROCESSING; PSEUDOMONAS; REVIEW;

EID: 0032911839     PISSN: 00319333     EISSN: None     Source Type: Journal    
DOI: 10.1152/physrev.1999.79.1.S175     Document Type: Review

References (158)

1. AL-AWQATI, Q. Proton translocating ATPases. Annu. Rev. Cell. Biol. 2: 179-199, 1986.
2. AL-AWQATI, Q. Chloride channels of intracellular organelles. Curr. Opin. Cell Biol. 1: 504-508, 1995.
3. ANBARI, M., K. V. ROOT, AND R. W. VAN DYKE. Role of Na,K-ATPase in regulating acidification of early rat liver endocytic vesicles. Hepatology 19: 1034-1043, 1994.
4. 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: 679-691, 1991.
5. ANDERSON, R. G. W., J. R. FALCK, J. L. GOLDSTEIN, AND M. S. BROWN. Visualization of acidic organelles in intact cells by electron microscopy. Proc. Natl. Acad. Sci. USA 81: 4838-4842, 1984.
6. BAE, H.-R., AND A. S. VERKMAN. Protein kinase A regulates chloride conductance in endocytic vesicles from proximal tubules. Nature 348: 637-639, 1990.
7. BARASCH, J., AND Q. AL-AWQATI. Defective acidification of the biosynthetic pathway in cystic fibrosis. J. Cell Sci. 17: 229-233, 1993.
8. BARASCH, J., M. D. GERSHON, E. A. NUNEZ, H. TAMIR, AND Q. AL-AWQATI. Thyrotropin induces the acidification of the secretory granules of parafollicular cells by increasing the chloride conductance of the granular membrane. J Cell Biol. 107: 2137-2147, 1988.
9. BARASCH, J., B. KISS, A. PRINCE, L. LAIMAN, D. GRUENERT, AND Q. AL-AWQATI. Defective acidification of intracellular organelles in cystic fibrosis. Nature 352: 70-73, 1991.
10. BARDON, A. Cystic fibrosis. Carbohydrate metabolism in CF and in animal models for CF. Acta Paediatr. Scand. 332: 1-30, 1987.
11. BAUBICHON-CORTAY, H., M. SERRES-GUILLAUMOND, P. LOUISOT, AND P. BROQUET. A brain sialyltransferase having a narrow specificity for O-glycosyl linked oligosaccharide chains. Carbohydr. Res. 149: 209-223, 1986.
12. BEAR, C. E., C. H. LI, N. KARTNER, R. J. BRIDGES, T. J. JENSEN, M. RAMJEESINGH, AND J. R. RIORDAN. Purification and functional expression of the cystic fibrosis transmembrane conductance regulator (CFTR). Cell 68: 809-818, 1992.
13. BECQ, F., T. J. JENSEN, X.-B. CHANG, A. SAVOIA, J. M. ROMMENS, L.-C. TSUI, M. BUCHWALD, J. R. RIORDAN, AND J. HANRAHAN. Phosphatase inhibitors activate normal and defective CFTR chloride channels. Proc. Natl. Acad. Sci. USA 91: 9160-9164, 1994.
14. BEDROSSIAN, C. W., S. D. GREENBERG, D. B. SINGER, J. J. HANSEN, AND H. S. ROSENBERG. The lung in cystic fibrosis. A quantitative study including prevalence of pathologic findings among different age groups. Hum. Pathol. 7: 195-204, 1976.
15. 3H label from the Golgi apparatus into dense and multivesicular bodies in the duodenal columnar cells and hepatocytes of the rat. J. Cell Biol. 51: 875-881, 1971.
16. BIWERSI, J., N. EMANS, AND A. S. VERKMAN. Cystic fibrosis transmembrane conductance regulator activation stimulates endosome fusion in vivo. Proc. Natl. Acad. Sci. USA 93: 12484-12489, 1996.
17. BIWERSI, J., AND A. S. VERKMAN. Functional CFTR in the endosomal compartment of CFTR-expressing fibroblasts and T84 cells. Am. J. Physiol. 266 (Cell Physiol. 35): C149-C156, 1994.
18. BLOMFIELD, J., J. DASCALU, E. W. VAN LENNEP, AND J. M. BROWN. Hypersecretion of zymogen granules in the pathogenesis of cystic fibrosis. Gut 14: 558-565, 1973.
19. BOAT, T. F., P. W. CHENG, R. N. IYER, D. M. CARLSON, AND I. POLONY. Human respiratory tract secretions, mucous glycoproteins of non-purulent tracheobronchial secretions, and sputum of patients with bronchitis and cystic fibrosis. Arch. Biochem. Biophys. 77: 95-103, 1976.
20. BOAT, T. F., J. L. KLEINERMAN, D. M. CARLSON, W. H. MALONEY, AND L. W. MATHEWS. Human respiratory tract secretions. 1. Mucous glycoproteins secreted by cultured nasal polyp epithelium from patients with allergic rhinitis and with cystic fibrosis. Am. Rev. Respir. Dis. 110: 428-441, 1974.
21. BOSMAN, H. B., A. HAGOPIAN, AND E. H. EYLAR. Glycoprotein biosynthesis. Characterization of two glycoprotein fucosyltransferases in HeLa cells. Arch. Biochem. Biophys. 128: 470-481, 1968.
22. + transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation. J. Clin. Invest. 78: 1245-1252, 1986.
23. BRADBURY, N. A., AND R. J. BRIDGES. Endocytosis is regulated by protein kinase A, but not protein kinase C in a secretory epithelial cell line. Biochem. Biophys. Res. Commun. 184: 1173-1180, 1992.
24. BRADBURY, N. A., AND R. J. BRIDGES. Role of membrane trafficking in plasma membrane solute transport. Am. J. Physiol. 267 (Cell Physiol 36): C1-C24, 1994.
25. BRADBURY, N. A., J. A. COHN, C. J. VENGLARIK, AND R. J. BRIDGES. Biochemical and biophysical identification of cystic fibrosis transmembrane conductance chloride channels as components of endocytic clathrin coated vesicles. J. Biol. Chem. 269: 8296-8302, 1994.
26. BRADBURY, N. A., T. JILLING, G. BERTA, E. J. SORSCHER, R. J. BRIDGES, AND K. L. KIRK. Regulation of plasma membrane recycling by CFTR. Science 256: 530-532, 1992.
27. BRADBURY, N. A., T. JILLING, K. L. KIRK, AND R. J. BRIDGES. Regulated endocytosis in a chloride secretory epithelial cell line. Am. J. Physiol. 262 (Cell Physiol. 31): C752-C759, 1992.
28. BUCHWALD, M. Abnormal levels of 3′:5′-cyclic AMP in isoproterenol-stimulated fibroblasts from patients with cystic fibrosis. Proc. Natl. Acad. Sci. USA 73: 2899-2903, 1976.
29. +-ATPase in living cells. Proc. Natl. Acad. Sci. USA 86: 544-548, 1989.
30. CAPASSO, J. M., AND C. B. HIRSCHBERG. Effect of atractylosides, palmitoyl coenzyme A, and anion transport inhibitors on translocation of nucleotide sugars and nucleotide sulphate into Golgi vesicles. J. Biol. Chem. 289: 4263-4266, 1984.
31. CAPASSO, J. M., AND C. B. HIRSCHBERG. Mechanisms of glycosylation and sulphation in the Golgi apparatus: evidence for nucleotide sugar/nucleoside monophospate and nucleotide sulfate/nucleoside monophosphate antiports in the Golgi apparatus membrane. Proc. Natl. Acad. Sci. USA 81: 7051-7055, 1984.
32. CARLSON, D. M., G. W. JOURDIAN, AND S. ROSEMAN. The sialic acids. XIV. Synthesis of sialyl-lactose by a sialyltransferase from rat mammary gland. J. Biol Chem. 218: 5742-5749, 1973.
33. CARTER, S. R., A. SLOMIANY, K. GWOZDZINSKI, Y. H. LIAU, AND B. L. SLOMIANY. Enzymatic sulfation of mucus glycoprotein in gastric mucosa. Effect of ethanol. J. Biol. Chem. 263: 11977-11984, 1988.
34. CARTY, S., R. G. JOHNSON, AND A. SCARPA. Serotonin transport in isolated platelet granules. J. Biol. Chem. 256: 11244-11250, 1981.
35. CHEN, S. Y. Adenylate cyclase and exocytosis of membrane-coating granules in oral mucosa. J. Submicrosc. Cytol. 14: 253-262, 1982.
36. CHENG, P.-W., R. C. BOUCHER, J. M. YANKASKAS, AND T. F. BOAT. Glycoconjugates secreted by cultured human nasal epithelial cells. In: Cellular and Molecular Basis of Cystic Fibrosis, edited by G. Mastella and P. M. Quiton. San Francisco, CA: San Francisco Press, 1988, p. 233-238.
37. CHENG, S. H., R. J. GREGORY, J. MARSHALL, S. PAUL, D. W. SOUZA, G. A. WHITE, C. R. 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.
38. CLAMP, J. R., AND M. GOUGH. Study of the oligosaccharide units from mucus glycoproteins of meconium from normal infants and from cases of cystic fibrosis with meconium ileus. Clin. Sci. 57: 445-451, 1979.
39. COHN, J. A. CFTR localization: implications for cell and tissue pathophysiology. In: Cystic Fibrosis Current Topics, edited by J. A. Dodge, D. J. H. Brock, and J. H. Widdicombe. Chichester, UK: Wiley, 1994, p. 173-192.
40. COHN, J. A., A. C. NAIRN, C. R. MARINO, O. MELHUS, AND J. KOLE. Characterization of the cystic fibrosis transmebrane conductance regulator in a colonocyte cell line. Proc. Natl. Acad. Sci. USA 89: 2340-2344, 1992.
41. COREY, J. L., N. DAVIDONS, H. A. LESTER, N. BRECHA, AND M. W. QUICK. Protein kinase C modulates the activity of a cloned γ-aminobutyric acid transporter expressed in Xenopus oocytes via regulated subcellular redistribution of the transporter. J. Biol. Chem. 269: 14759-14767, 1994.
42. CRAWFORD, I., P. C. MALONEY, P. L. ZEITLIN, W. B. GUGGINO, S. C. HYDE, H. TURLEY, K. C. GATTER, A. HARRIS, AND C. F. HIGGINS. Immunocytochemical localization of the cystic fibrosis gene product CFTR. Proc. Natl. Acad. Sci. USA 88: 9262-9266, 1991.
43. DALEMANS, W., J. HINNRASKY, P. SLOS, D. DREYER, C. FUCHEY, A. PAVIRANI, AND E. PUCHELLE. Immunocytochemical analysis reveals the difference between the subcellular localization of normal and ΔPhe508 recombinant cystic fibrosis transmembrane conductance regulator. Exp. Cell Res. 201: 235-240, 1992.
44. DAVIS, P. B., L. DIECKMAN, T. F. BOAT, R. C. STERN, AND C. F. DOERSHUK. Beta adrenergic receptors in lymphocytes and granulocytes from patients with cystic fibrosis. J. Clin. Invest. 71: 1787-1795, 1983.
45. DAVIS, P. B., AND S. C. LAUNDON. Adenylate cyclase in leukocytes from patients with cystic fibrosis. J. Lab. Clin. Med. 96: 75-84, 1980.
46. DE LISLE, R. C., AND J. A. WILLIAMS. Regulation of membrane fusion in secretory exocytosis. Annu. Rev. Physiol. 48: 225-238, 1986.
47. DENNING, G. M., L. S. OSTEGAARD, S. H. CHENG, A. E. SMITH, AND M. J. WELSH. Localization of cystic fibrosis transmembrane conductance regulator in chloride secretory epithelia J. Clin. Invest, 89: 339-349, 1991.
48. DHO, S., S. GRINSTEIN, AND J. K. FOSKETT. Plasma membrane recycling in CFTR-expressing CHO cells. Biochim. Biophys. Acta 1225: 78-82, 1993.
49. DIMANGO, E., A. J. RATNER, R. BRYAN, S. TABIBI, AND A. PRINCE. Activation of NF-kappaB by adherant Pseudomonas aeruginosa in normal and cystic fibrosis respiratory epithelial cells. J. Clin. Invest, 101: 2598-2605, 1998.
50. DISCHE, Z., P. A. DISANT'AGNESE, C. PALLAVICINI, AND J. YOULOS. Composition of mucoprotein fractions from duodenal fluid of patients with cystic fibrosis of the pancreas and from controls. Pediatrics 24: 74-91, 1959.
51. DODGE, J. A., D. J. H. BROCK, AND J. H. WIDDICOMBE. Cystic Fibrosis: Current Topics, edited by J. A. Dodge, D. J. H. Brock, and J. H. Widdicombe. New York: Wiley, 1993.
52. 2+ and cyclic AMP in regulation of intestinal Na, K, and Cl transport. Annu. Rev. Physiol. 48: 135-150, 1986.
53. DRUMM, M. L., H. A. POPE, W. H. CLIFF, J. M. ROMMENS, S. A. MARVIN, L.-C. TSUI, F. S. COLLINS, R. A. FRIZZELL, AND J. M. WILSON. Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer. Cell 62: 1227-1233, 1990.
54. DRUMM, M. L., D. J. WILKINSON, L. S. SMIT, R. T. WORRELL, T. V. STRONG, R. A. FRIZZELL, D. C. DAWSON, AND F. S. COLLINS. Chloride conductance expressed by the ΔF508 and other mutant CFTRs in Xenopus oocytes. Science 254: 1797-1799, 1991.
55. DUNN, K. W., J. PARK, C. E. SEMRAD, D. L. GELMAN, T. SHEVELL, AND T. E. McGRAW. Regulation of endocytic trafficking and acidification are independent of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 269: 5336-5345, 1994.
56. FARBER, S. Some organic digestive disturbances in early life. J. Mich. Med. Soc. 44: 587-594, 1945.
57. FEIGAL, R. J., M. S. TOMCZYK, AND B. L. SHAPIRO. The calcium abnormality in cystic fibrosis mitochondria: relative role of respiration and ATP hydrolysis. Life Sci. 30: 93-98, 1982.
58. FRATES, R. C., T. T. KAIZU, AND J. A. LAST. Mucous glycoproteins secreted by respiratory epithelial tissue from cystic fibrosis patients. Pediatr. Res. 17: 30-35, 1983.
59. FULLER, S. D., AND K. SIMONS. Transferrin receptor polarity and recycling accuracy in "tight" and "leaky" strains of Madin-Darby canine kidney cells. J. Cell Biol. 103: 1767-1779, 1986.
60. FULMER, S. B., E. M. SCHWIEBERT, M. M. MORALES, W. B. GUGGINO, AND G. R. CUTTING. Two cystic fibrosis transmembrane conductance regulator mutations have different effects on both pulmonary phenotype and regulation of outwardly rectified chloride currents. Proc. Natl. Acad. Sci. USA 92: 6832-6836, 1995.
61. GALANT, S. P., L. NORTON, J. HERBST, AND C. WOOD. Impaired beta adrenergic receptor binding and function in cystic fibrosis neutrophils. J. Clin. Invest. 68: 253-258, 1981.
62. GALLOWAY, C., G. DEAN, G. RUDNICK, AND I. MELLMAN. Acidification of macrophage and fibroblast endocytic vesicles. Proc. Natl. Acad. Sci. USA 80: 3334-3338, 1983.
63. GASSER, K. W., J. DIDOMENICO, AND U. HOPFER. Secretagogues activate chloride transport pathways in pancreatic zymogen granules. Am. J. Physiol 254 (Gastrointest. Liver Physiol. 17): G93-G99, 1988.
64. GEISOW, M. J., AND W. H. EVANS. pH in the endosome. Exp. Cell Res. 150: 36-46, 1984.
65. GEISOW, M. J., P. D. HART, AND M. R. YOUNG. Temporal changes of lysosome and phagosome pH during phagolysosome formation in macrophages: studies by fluorescence microscopy. J. Cell Biol. 89: 645-652, 1981.
66. + pumps in parallel to a chloride conductance. J. Cell Biol. 97: 1303-1308, 1983.
67. HALM, D. R., AND R. A. FRIZZELL. Intestinal chloride secretion. In: Textbook of Secretory Diarrhea, edited by E. Lebenthal and M. Duffey. New York: Raven, 1990, p. 47-58.
68. HANEY, P. M., J. W. SLOT, R. C. PIPER, D. E. JAMES, AND M. MUECKLER. Intracellular targeting of the insulin-regulatable glucose transporter (GLUT4) is isoform specific and independent of cell type. J. Cell Biol. 114: 689-699, 1991.
69. HANRAHAN, J. W., J. A. TABCHARANI, AND R. GRYGORCZYK. Patch clamp studies of apical membrane chloride channels. In: Cystic Fibrosis Current Topics, edited by J. A. Dodge, D. J. H. Brock, and J. H. Widdicombe. Chichester, UK: Wiley, 1993, p. 93-137.
70. HARIKUMAR, P., AND J. P. REEVES. The lysosomal pump is electrogenic. J. Biol. Chem. 258: 10403-10410, 1983.
71. HARPER, J. F. Stimulus-secretion coupling: second messenger-regulated exocytosis. Adv. Second Messenger Phosphoprotein Res. 22: 193-318, 1988.
72. HOWARD, M., T. JILLING, M. DUVALL, AND R. A. FRIZZELL. cAMP-regulated trafficking of epitope-tagged CFTR. Kidney Int. 49:1642-1648, 1996.
73. HUDSON, A. W., M. RUIZ, AND M. J. BIRNBAUM. Isoform-specific subcellular targeting of glucose transporters in mouse fibroblasts. J. Cell Biol. 116: 785-797, 1992.
74. HUG, M. J., I. E. THIELE, AND R. GREGER. The role of exocytosis in the activation of the chloride conductance in Chinese hamster ovary cells (CHO) stably expressing CFTR. Eur. J. Physiol. 434: 779-784, 1997.
75. JILLING, T., AND K. L. KIRK. Cyclic AMP and chloride-dependent regulation of the apical constitutive secretory pathway in colonic epithelial cells. J. Biol. Chem. 271: 4381-4387, 1996.
76. JOHNSON, R. G., S. CARTY, AND A. SCARPA. A model of biogenic amine accumulation into chromaffin granules and ghosts based on coupling to the electrochemical proton gradient. Federation Proc. 41: 2746-2754, 1982.
77. KARTNER, N., J. W. HANRAHAN, J. T. JENSEN, A. L. NAISMITH, S. SUN, C. A. ACKERLY, E. F. REYES, L.-C. TSUI, J. M. ROMMENS, C. E. BEAR, AND J. R. RIORDAN. Expression of the cystic fibrosis gene in non-epithelial cells produces a regulated anion conductance. Cell 64: 681-691, 1991.
78. KATO, Y., AND R. G. SPIRO. Characterization of a thyroid sulfo-transferase responsible for the 3-O-sulfation of terminal β-D-galactosyl residues in N-linked carbohydrate units. J. Biol. Chem. 264: 3364-3371, 1989.
79. KOLLBERG, H. Disturbed energy metabolism and intracellular acidosis are important for the pathogenesis of cystic fibrosis. Lakartidningen 82: 494-496, 1985.
80. KOOMANS, A., M. VON EULER, R. M. MULLER, AND H. GILLIAM. X-ray microanalysis of goblet cells in bronchial epithelium of patients with cystic fibrosis. J. Submicrosc. Cytol. 18: 613-615, 1986.
81. KRIVAN, H. C., V. GINSBURG, AND D. D. ROBERTS. Pseudomonas aeroginosa and Pseudomonas cepacia isolated from cystic fibrosis patients bind specifically to gangliotetraosylceramide (asialo GM1) and gangliotriaosylceramide (asialo GM2). Arch. Biochem. Biophys. 260: 493-496, 1988.
82. KRIVAN, H. C., D. D. ROBERTS, AND V. GINSBURG. Many pulmonary pathogenic bacteria bind specifically to the carbohydrate sequence GalNAcb1-4Gal found in some glycolipids. Proc. Natl. Acad. Sci. USA 85: 6157-6161, 1988.
83. + currents by intracellular domains of the cystic fibrosis transmembrane conductance regulator. FEBS Lett. 400: 341-344, 1997.
84. KURZ, J. B., AND J. P. PERKINS. Cystic fibrosis fibroblasts respond normally to isoproterenol. Pediatr. Res. 15: 1328-1333, 1981.
85. LEHRICH, R., S. G. ALLER, C. R. MARINO, P. WEBSTER, AND J. N. FORREST. Vasoactive intestinal peptide, forskolin, and genistein increase apical CFTR trafficking in the rectal gland of the spiny dogfish, Squalus acanthias. Acute regulation of CFTR trafficking in an intact epithelium. J. Clin. Invest. 101: 737-745, 1998.
86. LI, M., J. D. McCANN, C. M. LIEDTKE, A. C. NAIRN, P. GREENGARD, AND M. J. WELSH. Cyclic AMP-dependent protein kinases open chloride channels in normal but not in cystic fibrosis airway epithelium. Nature 331: 358-360, 1988.
87. LUKACS, G. L., X.-B. CHANG, N. KARTNER, O. D. ROTSTEIN, J. R. RIORDAN, AND S. GRINSTEIN. The cystic fibrosis transmembrane conductance regulator is present and functional in endosomes. J. Biol. Chem. 267: 14568-14572, 1992.
88. LUKACS, G. L., G. SEGAL, N. KARTNER, S. GRINSTEIN, AND F. ZHANG. Constitutive internalizatlon of cystic fibrosis transmembrane conductance regulator occurs via clathrin-dependent endocytosis and is regulated by protein phosphorylation. Biochem. J. 328: 353-361, 1997.
89. MARINO, C. R., L. M. MATOVCIK, F. S. GORELICK, AND J. A. COHN. Localization of the cystic fibrosis transmembrane conductance regulator in pancreas. J. Clin. Invest. 88: 712-716, 1991.
90. MARSHALL, J., K. A. MARTIN, M. PICCIOTTO, S. HOCKFIELD, A. C. NAIRN, AND L. K. KACZMAREK. Identification and localization of a dogfish homolog of human cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 266: 22749-22754, 1991.
91. MASUR, S. K., E. HOLTZMAN, AND R. WALTER. Hormone-stimulated exocytosis in toad urinary bladder. Some possible implications for turnover of surface membranes. J. Cell Biol. 52: 211-219, 1972.
92. MAXFIELD, F. R. Weak bases and ionophores rapidly and reversibly raise the pH of endocytic vesicles in cultured mouse fibroblasts. J. Cell Biol. 95: 676-681, 1982.
93. McPHERSON, M. A., R. L. DORMER, N. A. BRADBURY, J. A. DODGE, AND M. C. GOODCHILD. Defective β-adrenergic secretory responses in submandibular acini from cystic fibrosis patients. Lancet II: 1007-1008, 1986.
94. 2+. In: Cellular and Molecular Basis of Cystic Fibrosis, edited by G. Mastella and P. M. Quinton. San Francisco, CA: San Francisco Press, 1988, p. 343-354.
95. MELLMAN, I., R. FUCHS, AND A. HELENIUS. Acidification of the endocytic and exocytic pathways. Annu. Rev. Biochem. 55: 663-700, 1986.
96. MERGEY, M., M. LEMNAOUAR, D. VEISSIERE, M. PERRICAUDET, D. C. GRUENERT, J. PICARD, J. CAPEAU, H. M. BRAHIMI, AND A. PAUL. CFTR gene transfer corrects defective glycoconjugate secretion in human epithelial tracheal cells. Am. J. Physiol. 269 (Lung Cell. Mol. Physiol 13): L855-L864, 1995.
97. METCHNIKOFF, E. Lectures on the Comparatice Pathology of Inflammation. London: Paul, Kegan, Trench, Trabner, 1893.
98. MILLS, C. L, J. R. DORIN, D. J. DAVIDSON, D. J. PORTEUS, E. W. ALTON, R. L. DORMER, AND M. A. McPHERSON. Decreased beta-adrenergic stimulation of glycoprotein secretion in CF mice submandibular glands: reversal by the methylxanthine, IBMX. Biochem. Biophys. Res. Commun. 215: 674-681, 1995.
99. MOYER, B., J. TABB, E. SCHWIEBERT, W. GUGGINO, G. LANGFORD, AND B. STANTON. Intracellular trafficking of CFTR in living human airway cells using green fluorescent protein (GFP) (Abstract). Proc. N. Am. Cystic Fibrosis Conf. 10th Orlando FL 1996, p. 209.
100. MULBERG, A. E., B. M. TULK, AND M. FORGAC. Modulation of coated vesicle chloride channel activity and acidification by reversible protein kinase A-dependent phosphorylation. J. Biol. Chem. 266: 20590-20593, 1991.
101. + channel in the cardiac atrial cell membrane. Intracellular chloride inhibition of the GTPase activity of GK. J. Gen. Physiol. 99: 665-682, 1992.
102. NEUTRA, M. R., R. J. GRAND, AND J. S. TRIER. Glycoprotein synthesis, transport, and secretion by epithelial cells of human rectal mucosa: normal and cystic fibrosis. Lab. Invest. 36: 535-546, 1977.
103. OHKUMA, S., AND B. POOLE. Fluorescence probe measurements of the intralysosomal pH in living cells and the perturbation of pH by various agents. Proc. Natl. Acad. Sci. USA 75: 3327-3331, 1978.
104. - channel is functional when retained in endoplasmic reticulum of mammalian cells. J. Biol. Chem. 270: 12347-12350, 1995.
105. PAULSON, J. C., J. P. PRIEELS, L. R. GLASGOW, AND R. L. HILL. Sialyl and fucosyltransferases in the biosynthesis of asparaginyl-linked oligosaccharaides in glycoproteins. Mutually exclusive glycosylation by β-galactoside α2,6 sialyltransferase and N-acetyl glucosamine β1,6,3,fucosyltransferase. J. Biol. Chem. 253: 5617-5625, 1978.
106. PETERS, K. W., M. HOWARD, S. C. WATKINS, AND R. A. FRIZZELL. Cell Surface Labeling of M2-901 CFTR in Xenopus Oocytes. Washington, DC: Am. Soc. Cell Biol., 1997.
107. PODBLIEWICZ, B., AND I. MELLMAN. ATP and cytosol requirements for transferrin recycling in intact and disrupted MDCK cells. EMBO J. 9: 3477-3487, 1990.
108. PRIEELS, J.-P., T. BEYERS, AND R. L. HILL. Human milk fucosyl-transferases. Biochem. Soc. Trans. 5: 838-839, 1977.
109. PRINCE, L. S., A. TOUSSON, AND R. B. MARCHASE. Cell surface labelling of CFTR in T84 cells. Am. J. Physiol. 264 (Cell Physiol. 33): C491-C498, 1993.
110. PRINCE, L. S., R. B. J. WORKMAN, AND R. B. MARCHASE. Rapid endocytosis of the cystic fibrosis transmembrane conductance regulator chloride channel. Proc. Natl. Acad. Sci. USA 91: 5192-5196, 1994.
111. PUCHELLE, E., D. GAILLARD, D. PLOTON, J. HINNRASKY, C. FUCHEY, M.-C. BOUTTERIN, J. JACQUOT, D. DREYER, AND A. PAVIRANI. Differential localization of the cystic fibrosis transmembrane conductance regulator in normal and cystic fibrosis airway epithelium. Am. J. Respir. Cell Mol. Biol. 7: 485-491, 1992.
112. QI, J.-J., J. J. FORREST, W. VAN DRIESSCHE, K. PETERS, R. J. BRIDGES, AND R. A. FRIZZELL. cAMP stimulates CFTR trafficking in shark (Squalus acanthias) rectal gland. Bull. Mt. Desert Isl. Biol. Lab. 38: 26-29, 1998.
113. QUINTON, P. M. Cystic fibrosis: a disease in electrolyte transport. FASEB J. 4: 2709-2717, 1990.
114. - impermeability in cystic fibrosis sweat duct epithelium. Am. J. Physiol. 257 (Cell Physiol. 26): C727-C735, 1989.
115. - conductance in both cell membranes of an absorptive epithelium. J. Membr. Biol. 130: 49-62, 1992.
116. REENSTRA, W. W, I. SABOLIC, H.-R. BAE, AND A. S. VERKMAN. Protein kinase A dependent membrane protein phosphorylation and chloride conductance in endosomal vesicles from kidney cortex. Biochemistry 31: 175-181, 1992.
117. RIORDAN, J. R., J. M. ROMMENS, B. S. KEREM, N. ALON, R. ROZMAHEL, Z. GRZELCZAK, J. ZIELENSKI, S. LOK, N. PLAVSIC, J.-L. CHOU, M. L. DRUMM, M. C. IANUZZI, F. S. COLLINS, AND L.-C. TSUI. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245: 1059-1065, 1989.
118. ROMMENS, J. M., S. DHO, C. E. BEAR, N. KARTNER, D. KENNEDY, J. R. RIORDAN, L.-C. TSUI, AND J. K. FOSKETT. cAMP-inducible chloride conductance in mouse fibroblast lines stably expressing the human cystic fibrosis transmembrane conductance regulator. Proc. Natl. Acad. Sci. USA 88: 7500-7504, 1991.
119. ROOS, A., AND W. F. BORON. Intracellular pH. Physiol, Rev. 61: 296-434, 1981.
120. ROOT, K. V., J. F. ENGELHARDT, M. POST, J. W. WILSON, AND R. W. VAN DYKE. CFTR does not alter acidication of L cell endosomes. Biochem. Biophys. Res. Commun. 205: 396-401, 1994.
121. ROSCHER, A. A., A. J. SCHMID, B. HADORN, AND U. N. WIESMANN. Enhanced adenosine 3′:5′-monophosphate response to beta-adrenergic stimulation in cystic fibrosis fibroblasts after removal of conditioned medium. Pediatr. Res. 14: 261-265, 1980.
122. ROSE, M. C. Epithelial mucous glycoproteins and cystic fibrosis. Horm. Metab. Res. 20: 601-608, 1988.
123. ROTTENBERG, H. Proton electrochemical potential gradients in vesicles, organelles, and prokaryotic cells. Methods Enzymol. 172: 63-84, 1989.
124. RUSSELL, J. T., AND R. W. HOLZ. Meaurement of ΔpH and membrane potential in isolated neurosecretory vesicles from bovine neurohypopheses. J. Biol. Chem. 256: 5950-5953, 1981.
125. RYBAK, S. L., F. LANNI, AND R. F. MURPHY. Theoretical considerations on the role of membrane potential in the regulation of endosomal pH. Biophys. J. 73: 674-687, 1997.
126. SAIJAN, U., J. REISMAN, P. DOIG, R. T. IRVIN, G. FORSTNER, AND J. FORSTNER. Binding of non-mucoid Pseudomonas aeroginosa to normal human intestinal mucin and respiratory mucin from patients with cystic fibrosis. J. Clin. Invest. 89: 657-667, 1992.
127. SAIMAN, L., AND A. PRINCE. Pseudomonas aeroginosa pili bind to asialoGM1 which is increased on the surface of cystic fibrosis epithelial cells. J. Clin. Invest. 92: 1875-1880, 1993.
128. SANTOS, G. F., AND W. W. REENSTRA. Activation of the cystic fibrosis transmembrane conductance regulator by cAMP is not correlated with inhibition of endocytosis. Biochim. Biophys. Acta 1195: 96-102, 1994.
129. SATO, K., AND F. SATO. Defective beta adrenergic response of cystic fibrosis sweat glands in vivo and in vitro. J. Clin. Invest 73: 1763-1771, 1984.
130. SCHNEIDER, D. L. ATP-dependent acidification of intact and disrupted lysosomes. J. Biol. Chem. 256: 3858-3864, 1981.
131. SCHOUMACHER, R. A., J. RAM, M. C. IANUZZI, N. A. BRADBURY, R. W. WALLACE, C. T. HON, D. R. KELLY, S. M. SCHMIDT, F. B. GELDER, T. A. RADO, AND R. A. FRIZZELL. A cystic fibrosis pancreatic adenocarcinoma cell line. Proc. Natl. Acad. Sci. USA 87: 4012-4016, 1990.
132. SCHOUMACHER, R. A., R. L. SHOEMAKER, D. R. HALM, E. A. TALLANT, R. W. WALLACE, AND R. A. FRIZZELL. Phosphorylation fails to activate chloride channels from cystic fibrosis airway cells. Nature 330: 752-754, 1987.
133. - channels. Am. J. Physiol. 267 (Cell Physiol. 36): C272-C281, 1994.
134. STONE, D. K., S.-S. XIE, AND E. RACKER. An ATP-dependent proton pump in clathrin coated vesicles. J. Biol. Chem. 258: 4059-4062, 1983.
135. STOORVOGEL, W., H. J. GEUZE, J. M. GRIFFITH, A. L. SCHWARTZ, AND G. J. STROUS. Relations between the intracellular pathways of the receptors for transferrin, asialoglycoprotein, and mannose-6-phosphate in human hepatoma cells. J. Cell Biol. 108: 2137-2148, 1989.
136. TAATJES, D. J., J. ROTH, J. WEINSTEIN, AND J. C. PAULSON. Post-Golgi apparatus localization and regional expression of rat intestinal sialyltransferase detected by immunoelectron microscopy with polypeptide epitope-purified antibody. J. Biol. Chem. 263: 6302-6309, 1988.
137. TAKAHASI, A., S. C. WATKINS, M. HOWARD, AND R. A. FRIZZELL. CFTR-dependent membrane insertion is linked to stimulation of the CFTR chloride conductance. Am. J. Physiol. 271 (Cell Physiol. 40): C1887-C1894, 1996.
138. TALAMO, R. C., B. J. ROSENSTEIN, AND R. W. BERNINGER. Cystic Fibrosis. New York: McGraw-Hill, 1983.
139. TANG, H., M. KAYS, AND A. PRINCE. Role of Pseudomonas aeroginosa pilin in acute pulmonary infection. Infect. Immun, 63: 1278-1285, 1995.
140. THEANDER, T. G., A. KHARZAMI, B. K. PEDERSEN, L. D. CHRISTENSEN, N. TVEDE, L. K. POULSON, N. ODUM, M. SVENSON, AND K. BENDTZEN. Inhibition of human lymphocyte proliferation and cleavage of interleukin-2 by Pseudomonas aeroginosa proteases. Infect. Immun. 56: 1673-1677, 1988.
141. TILLY, B. C., G. M. S. MANCINI, J. BIJMAN, P. G. M. VAN GAGELDONK, C. E. M. T. BEERENS, R. J. BRIDGES, H. R. DE JONGE, AND F. W. VERHEIJEN. Nucleotide-activated chloride channels in lysosomal membranes. Biochem. Biophys. Res. Commun. 187: 254-260, 1992.
142. 2-macroglobulin. Cell 92: 643-651, 1982.
143. VAN DYKE, R. W. Acidification of lysosomes and endosomes. In: Subcellular Biochemistry, edited by J. B. Lloyd and R. W. Mason. New York: Plenum, 1996, p. 331-360.
144. VAN DYKE, R. W. Proton pump-generated electrochemical gradient in rat liver multivesicular bodies. J. Biol Chem, 263: 2603-2611, 1997.
145. VAN DYKE, R. W., AND J. D. BELCHER. Acidification of three types of liver endocytic vesicles: similarities and differences. Am. J. Physiol. 266 (Cell Physiol. 35): C81-C94, 1994.
146. VAN DYKE, R. W., K. V. ROOT, AND R. A. HSI. cAMP and protein kinase A stimulate acidification of rat liver endosomes in the absence of chloride. Biochem. Biophys. Res. Commun. 222: 312-316, 1996.
147. VAN DYKE, R. W., K. V. ROOT, J. H. SCHREIBER, AND J. M. WILSON. Role of CFTR in lysosomal acidification. Biochem. Biophys. Res. Commun, 184: 300-305, 1992.
148. WALLER, R. L., W. J. BRATTIN, AND D. G. DEARBORN. Cytosolic free calcium concentration and intracellular calcium distribution in lymphocytes from cystic fibrosis patients. Life Sci. 35: 775-781, 1984.
149. WEBSTER, P., L. VANACORE, A. C. NAIRN, AND C. R. MARINO. Subcellular localization of CFTR to endosomes in a ductal epithelium. Am. J. Physiol 267 (Cell Physiol. 36): C340-C348, 1994.
150. WELSH, M. J., AND C. M. LIEDTKE. Chloride and potassium channels in cystic fibrosis airway epithelia. Nature 322: 467-470, 1986.
151. WESLEY, A., J. FORSTNER, R. QUERESHI, M. MANTLE, AND G. FORSTNER. Human intestinal mucin in cystic fibrosis. Pediatr. Res. 17: 65-69, 1983.
152. WIDDICOMBE, J. H. Cystic fibrosis and beta-adrenergic response of airway epithelial cell cultures. Am. J. Physiol. 251 (Regulatory Integrating Comp. Physiol 20): R818-R822, 1986.
153. WOODS, D. E., J. A. BASS, W. G. J. JOHANSON, AND D. C. STRAUS. Role of adherance in the pathogenesis of Pseudomonas aeroginosa lung infection in cystic fibrosis patients. Infect. Immun. 30: 694-699, 1980.
154. YAMASHIRO, D. J., S. R. FLUSS, AND F. R. MAXFIELD. Acidification of endocytic vesicles by an ATP-dependent proton pump. J. Cell Biol. 97: 929-934, 1983.
155. YANG, Y., D. C. DEVOR, J. F. ENGELHARDT, S. A. ERNST, T. V. STRONG, F. S. COLLINS, J. A. COHN, R. A. FRIZZELL, AND J. M. WILSON. Molecular basis of defective anion transport in L cells expressing recombinant forms of CFTR. Hum. Mol. Genet. 2: 1253-1261, 1993.
156. YANG, Y., S. JANICH, J. A. COHN, AND J. M. WILSON. 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 90: 9480-9484, 1993.
157. +-ATPases in the secretory pathway of HepG2 cells. J. Biol. Chem. 268: 19092-19100, 1993.
158. ZEUZEM, S., P. FEICK, P. ZUMMERMANN, W. HAASE, R. A. KAHN, AND I. SCHULZ. Intravesicular acidification correlates with binding of ADP-ribosylation factor to microsomal membranes. Proc. Natl. Acad. Sci. USA 89: 6619-6623, 1992.