Terminal glycosylation in cystic fibrosis

Biochimica et Biophysica Acta - Molecular Basis of Disease

Volumn 1455, Issue 2-3, 1999, Pages 241-253

  Scanlin, Thomas F ^a   Glick, Mary Catherine ^a  

^a CHILDREN S HOSPITAL OF PHILADELPHIA   (United States)

Author keywords

Cystic fibrosis; Cystic fibrosis transmembrane regulator; Glycosylation in cystic fibrosis; Terminal glycosylation

Indexed keywords

BINDING PROTEIN; CHLORIDE CHANNEL; GENE PRODUCT; GLYCOCONJUGATE; GLYCOPROTEIN; MEMBRANE PROTEIN; OLIGOSACCHARIDE; TRANSMEMBRANE CONDUCTANCE REGULATOR;

ANIMAL MODEL; CYSTIC FIBROSIS; FUCOSYLATION; GENE MUTATION; GENE THERAPY; GLYCOSYLATION; HAEMOPHILUS INFLUENZAE; HUMAN; INFLAMMATION; LUNG DISEASE; MOUSE; NONHUMAN; PRIORITY JOURNAL; PSEUDOMONAS AERUGINOSA; REVIEW; SIALYLATION;

ALPHA-L-FUCOSIDASE; ANIMALS; CARBOHYDRATE SEQUENCE; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; EPITHELIAL CELLS; FIBROBLASTS; GENE THERAPY; GLYCOSYLATION; HUMANS; LUNG; MAGNETIC RESONANCE SPECTROSCOPY; MOLECULAR SEQUENCE DATA; MUTATION; OLIGOSACCHARIDES; SPECTROMETRY, MASS, FAST ATOM BOMBARDMENT;

BACTERIA (MICROORGANISMS); HAEMOPHILUS INFLUENZAE; PSEUDOMONAS; PSEUDOMONAS AERUGINOSA;

EID: 0032869010     PISSN: 09254439     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0925-4439(99)00059-9     Document Type: Review

References (121)

1. Adam E.C., Mitchell B.S., Schumacher D.U., Grant G., Schumacher U. Pseudomonas aeruginosa II lectin stops human ciliary beating: therapeutic implications of fucose. Am. J. Respir. Crit. Care Med. 55:1997;2102-2104.
2. Alhadeff J.A., Tennant L., O'Brien J.S. Altered isoenzyme patterns of liver α-L-fucosidase in cystic fibrosis. Clin. Genet. 10:1976;63-72.
3. Alhadeff J.A. Glycoproteins and cystic fibrosis. A review. Clin. Genet. 14:1978;189-201.
4. Alhadeff J.A., Watkins P. Differential concanavalin A binding of cystic fibrosis and normal liver α-L-fucosidase. Biochem. Biophys. Res. Commun. 86:1979;787-792.
5. Barasch J., Kiss B., Prince A., Saiman L., Gruenert D., AI-Awqati Q. Defective acidification of intracellular organelles in cystic fibrosis. Nature. 352:1991;70-73.
6. Barasch J., Al-Awqati Q. Defective acidification of the biosynthetic pathway in cystic fibrosis. J. Cell Sci. 17:1993;229-233.
7. Bebok Z., Mazzochi C., King S.A., Hong J.S., Sorscher E.J. The mechanism underlying cystic fibrosis transmembrane conductance regulator transport from the endoplasmic reticulum to the proteasome includes sec61β and a cytosolic, deglycosylated intermediary. J. Biol. Chem. 6:1998;29873-29878.
8. 1 is a receptor for Pseudomonas aeruginosa adherence to regenerating respiratory epithelial cells. Infect. Immun. 64:1996;1582-1588.
9. 2-macroglobulin from patients with cystic fibrosis. Clin. Chim. Acta. 99:1979;31-35.
10. Bertrand F., Hermelin B., Paul A., Picard J. Pericellular glycoconjugates of cultured fibroblasts from control and cystic fibrosis. Int. J. Biochem. 23:1991;51-57.
11. Boat T.F., Kleinerman J.L., Carlson D.M., Maoney W.H., Matthews L.W. Human respiratory tract secretions: mucous glycoproteins secreted by cultured nasal polyp epithelium from subjects with allergic rhinitis and cystic fibrosis. Am. Rev. Respir. Dis. 110:1974;428-441.
12. Boat T.F., Cheng P.W. Biochemistry of airway mucus secretions. Fed. Proc. 39:1980;3067-3074.
13. Bradbury N.A., Jilling T., Berta G., Sorscher E.J., Bridges R.J., Kirk K.L. Regulation of plasma membrane recycling by CFTR. Science. 256:1992;530-531.
14. Bradbury N.A., Cohn J.A., Venglarik C.J., Bridges R.J. Biochemical and biophysical identification of cystic fibrosis transmembrane conductance regulator chloride channels as components of endocytic clathrin-coated vesicles. J. Biol. Chem. 269:1994;8296-8302.
15. 1 and increased Pseudomonas aeruginosa binding by epithelial cells. Am. J. Respir. Crit. Care Med. 19:1998;269-277.
16. Carnoy C., Ramphal R., Scharfman A., Lo-Guidice J.-M., Houdret N., Klein A., Galabert C., Lamblin G., Roussel P. Altered carbohydrate composition of salivary mucins from patients with cystic fibrosis and the adhesion of Pseudomonas aeruginosa. Am. J. Respir. Cell Mol. Biol. 9:1993;323-334.
17. Chace K.V., Leahy D.S., Martin R., Carubelli R., Flux M., Sachdev G.P. Respiratory mucous secretions in patients with cystic fibrosis: relationship between levels of highly sulfated mucin component and severity of disease. Clin. Chim. Acta. 132:1983;143-155.
18. Cheng P.W., Boat T.F., Cranfill K., Yankaskas J.R., Boucher R.C. Increased sulfation of glycoconjugates by cultured nasal epithelial cells from patients with cystic fibrosis. J. Clin. Invest. 84:1989;68-72.
19. Cheng S.H., Gregory R.J., Marshall J., Paul S., Souza D.W., White G.A., O'Riordan C.R., Smith A.E. Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell. 63:1990;827-834.
20. Cheng S.H., Fang S.L., Zabner J., Marshall J., Piraino S., Schiavi S.C., Jefferson D.M., Welsh M.J., Smith A.E. Functional activation of the cystic fibrosis trafficking mutant ΔF508-CFTR by overexpression. Am. J. Physiol. 268:1995;L615-L624.
21. Clamp J.R., Gough M. Study of the oligosaccharide units from mucus glycoproteins of meconium from normal infants and from cases of cystic fibrosis with meconium ileus. Clin. Sci. 57:1979;445-451.
22. Cohen J.C., Morrow S.L., Cork R.J., Delcarpio J.B., Larson J.E. Molecular pathophysiology of cystic fibrosis based on the rescued knockout mouse model. Mol. Genet. Metab. 64:1998;108-118.
23. Collins F.S. Cystic fibrosis: molecular biology and therapeutic implications. Science. 256:1992;774-779.
24. Crystal R.G. Transfer of genes to humans: early lessons and obstacles to success. Science. 270:1995;404-410.
25. Cutting G.R. Genotype defect: its effect on cellular function and phenotypic expression. Semin. Respir. Crit. Care Med. 15:1994;356-361.
26. Davies J.C., Stern M., Dewar A., Caplen N.J., Munkonge F.M., Pitt T., Sorgi F., Huang L., Bush A., Geddes D.M., Alton E.W.F.W. CFTR gene transfer reduces the binding of Pseudomonas aeruginosa to cystic fibrosis respiratory epithelium. Am. J. Respir. Cell Mol. Biol. 16:1997;657-663.
27. Denning G.M., Anderson M.P., Amara J.F., Marshall J., Smith A.E., Welsh M.J. Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature sensitive. Nature. 358:1992;761-764.
28. Denning G.M., Ostedgaard L.W., Welsh M. Abnormal localization of cystic fibrosis transmembrane conductance regulator in primary cultures of cystic fibrosis airway epithelia. J. Biol. Chem. 118:1992;551-559.
29. Devaraj N., Sheykhnazari M., Warren W.S., Bhavanadan V.P. Differential binding of Pseudomonas aeruginosa to normal and cystic fibrosis trachebronchial mucins. Glycobiology. 4:1994;307-316.
30. Diamond G., Scanlin T.F., Zasloff M.A., Bevins C.L. A cross-species analysis of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 266:1991;22761-22769.
31. Dische Z., di Sant'Agnese P., Pallavicini C., Youlos J. Composition of mucoprotein fractions from duodenal fluid of patients with cystic fibrosis of the pancreas and from controls. Pediatrics. 24:1959;74-79.
32. Dosanjh A., Lencer W., Brown D., Ausiello D.A., Stow J.L. Heterologous expression of ΔF508 CFTR results in decreased sialylation of membrane glycoconjugates. Am. J. Physiol. 35:1994;C360-C366.
33. Drumm M.L., Pope H.A., Cliff W.H., Rommens J.M., Marvin S.A., Tsui L.C., Collins F.S., Frizzell R.A., Wilson J.M. Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer. Cell. 62:1990;1227-1233.
34. Drumm M.L., Wilkinson D.J., Smit L.S., Worell R.T., Strong T.V., Prizzell R.A., Dawson D.C., Collins F.S. Chloride conductance expressed by ΔF508 and other mutant CFTRs in Xenopus oocytes. Science. 254:1991;1797-1799.
35. Duthel S., Revol A. Glycan microheterogeneity of α1-antitrypsin in serum and meconium from normal and cystic fibrosis patients by crossed immuno-affinoelectrophoresis with different lectins (Con A, LCA, WGA). Clin. Chim. Acta. 215:1993;173-187.
36. Erbacher P., Bousser M.-T., Raimond J., Monsigny M., Midoux P., Roche A.C. Gene transfer by DNA/glycosylated polylysine complexes into human blood monocyte-derived macrophages. Hum. Gene Ther. 7:1996;721-729.
37. 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. 266:1994;107-109.
38. T.A. Gerken, R. Gupta, Mucus in cystic fibrosis, in: P.B. Davis (Ed.), Cystic Fibrosis, vol. 3, 1993, pp. 53-90.
39. Gibson A., Futter C.E., Maxwell S., Allchin E.H., Shipman M., Kraehenbuhl J.-P., Domingo D., Odorizzi G., Trowbridge I.S., Hopkins C.R. Sorting mechanisms regulating membrane protein traffic in the apical transcytotic pathway of polarized MDCK cells. J. Cell Biol. 143:1998;81-94.
40. Gilboa-Garber N. Pseudomonas aeruginosa lectins. Methods Enzymol. 83:1982;378-385.
41. Gregory R.J., Cheng S.H., Rich D.P., Marshall J., Sucharita P., Hehir K., Ostedgaard L., Klinger K.W., Welsh M.J., Smith A.E. Expression and characterization of the cystic fibrosis transmembrane conductance regulator. Nature. 347:1990;382-386.
42. Gruenert D.C., Basbaum C.B., Welsh M.J., Li M., Finkbeiner W.E., Nadel J.A. Characterization of human tracheal epithelial cells transformed by an origin defective simian virus 40. Proc. Natl. Acad. Sci. USA. 85:1988;5951-5955.
43. Harris A. Intracellular retention of lysosomal enzymes in cystic fibrosis. Clin. Genet. 20:1981;315-319.
44. Harsch A.D., Xu J., Bevins C.L., Glick M.C., Scanlin T.F. Preparation of isolated surface membranes from cystic fibrosis airway epithelial cells. Chest. 101:1992;58s-60s.
45. Hebert D.N., Foellmer B., Helenius A. Glucose trimming and reglucosylation determine glycoprotein association with calnexin in the endoplasmic reticulum. Cell. 81:1995;425-433.
46. Hill W.G., Harper G.S., Rozaklis T., Boucher R.C., Hopwood J.J. Organ-specific over-sulfation of glycosaminoglycans and altered extracellular matrix in a mouse model of cystic fibrosis. Biochem. Mol. Med. 62:1997;113-122.
47. Imundo L., Barasch J., Prince A., Al-Awqati Q. Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface. Proc. Natl. Acad. Sci. USA. 92:1995;3019-3023.
48. Jensen T.J., Loo M.A., Pind S., Williams D.B., Goldberg A.L., Riordan J.R. Multiple proteolytic systems, including the proteasome, contribute to CF processing. Cell. 83:1995;129-135.
49. Jetten A.M., Yankaskas J.R., Stutts M.J., Willumsen N.J., Boucher R.C. Persistence of abnormal chloride conductance regulation in transformed cystic fibrosis epithelia. Science. 244:1989;1472-1475.
50. Jiang X., Hill W.G., Pilewski J.M., Weisz O.A. Glycosylation differences between cystic fibrosis and rescued airway cell line are not CFTR dependent. Am. J. Physiol. 273:1997;L913-L920.
51. Johnson K., Dawson G. Molecular defect in processing α-fucosidase in fucosidosis. Biochem. Biophys. Res. Commun. 133:1985;90-97.
52. Kerem B.S., Buchanan J.A., Durie P., Coorey M.L., Levinson H., Rommens J.M., Buchwald M., Tsui L.-C. Identification of the cystic fibrosis gene: genetic analysis. Science. 245:1989;1073-1080.
53. Khan T.Z., Wagener J.S., Boat T., Martinez J., Accurso F.J., Riches D.W.H. Early pulmonary inflammation in infants with cystic fibrosis. Am. J. Respir. Crit. Care Med. 151:1995;L1076-1082.
54. Kollen W.J.W., Midoux P., Erbacher P., Yip A., Roche A.C., Monsigny M., Glick M.C., Scanlin T.F. Gluconoylated and glycosylated polylysines as vectors for gene transfer into cystic fibrosis airway epithelial cells. Hum. Gene Ther. 7:1996;1577-1586.
55. Kollen W.J.W., Schembri F., Gerwig G.J., Vliegenthart J.F.G., Glick M.C., Scanlin T.F. Enhanced efficiency of lactosylated poly-L-lysine mediated gene transfer into cystic fibrosis airway epithelial cells. Am. J. Respir. Cell Mol. Biol. 20:1999;1081-1086.
56. Kollen W.J.W., Mulberg A.E., Wei X., Sugita M., Raghuram V., Wang J., Foskett J.K., Glick M.C., Scanlin T.F. High efficiency transfer of CFTR cDNA into cystic fibrosis airway cells in culture using lactosylated polylysine as a vector. Hum. Gene Ther. 10:1999;615-622.
57. 2). Arch. Biochem. Biophys. 260:1988;493-496.
58. Kube D., Perez A., Davis P.B. Quantitative fluorescent microscopy reveals altered cell surface glycoconjugated on 9HTEo-cells transfected with the regulatory domain of CFTR or ΔF508 CFTR. Pediatr. Pulmonol. 12:1995;209A.
59. Kuver R., Ramesh N., Lau S., Savard C., Lee S.P., Osborne W.R.A. Constitutive mucin secretion linked to CFTR expression. Biochem. Biophys. Res. Commun. 203:1994;1457-1462.
60. G. Lamblin, J.J. Lafitte, M. Lhermitte, P. Degand, P. Roussel, Mucins from cystic fibrosis sputum, in: G.G. Forstner (Ed.), Mucus Secretions and Cystic Fibrosis, vol. 19 of Modern Problems in Pediatrics, S. Karger, New York, 1967, pp. 153-164.
61. Lamblin G., Boersma A., Klein A., Roussel P. Primary structure determination of five sialylated oligosaccharides derived from bronchial mucus glycoproteins of patients suffering from cystic fibrosis. J. Biol. Chem. 259:1984;9051-9058.
62. Lazatin J.O., Glick M.C., Scanlin T.F. Fucosylation in cystic fibrosis airway epithelial cells. Glycosyl. Dis. 1:1994;263-270.
63. Leibold D.M., Robinson C.A., Scanlin T.F., Glick M.C. Lack of proteolytic processing of α-L-fucosidase in human skin fibroblasts. J. Cell. Physiol. 137:1988;411-420.
64. Li C., Ramjeesingh M., Reyes E., Jensen T., Chang X., Rommens J.M., Bear C.E. The cystic fibrosis mutation (ΔF508) does not influence the chloride channel activity of CFTR. Nat. Genet. 3:1993;311-316.
65. Lippincott-Schwartz J., Yuan L., Tipper C., Amherdt M., Orci L., Klausner R.D. Brefeldin A's effects on endosomes, lysosomes and the TGN suggest a general mechanism for regulating organelle structure and membrane traffic. Cell. 67:1991;601-616.
66. Liu A., Park P.J., Rhim A.D., Weiser J.N., Glick M.C., Scanlin T.F. Functional implications of increased fucosylation of airway epithelial cell surface glycoproteins in CF. Pediatr. Pulmonol. S17:1998;218.
67. Lowe J.B. Selectin ligands, leukocyte trafficking, and fucosyltransferase genes. Kidney Int. 51:1997;1418-1426.
68. Lukacs G.L., Chang X.-B., Kartner N., Rotstein O.D., Riordan J.R., Grinstein S. The cystic fibrosis transmembrane regulator is present and functional in endosomes. J. Biol. Chem. 267:1992;14568-14572.
69. Maler T., Duthie M., Alon N., Riordan J.R. α-L-Fucosidase is quantitatively reduced in cultured lymphoblasts from patients with cystic fibrosis. J. Biol. Chem. 256:1981;1420-1427.
70. Margolies R., Boat T. The carbohydrate content of IgG from patients with cystic fibrosis. Pediatr. Res. 17:1983;931-935.
71. Marshall J., Fang S., Ostedgaard L.S., O'Riordan C.R., Ferrara D., Amara J.F., Hoppe H. IV, Scheule R.K., Welsh M.J., Smith A.E., Cheng S.H. Stoichiometry of recombinant cystic fibrosis transmembrane conductance regulator in epithelial cells and its functional reconstitution into cells in vitro. J. Biol. Chem. 269:1994;2987-2995.
72. Monsigny M., Roche A.-C., Midoux P., Mayer R. Glycoconjugates as carriers for specific delivery of therapeutic drugs and genes. Adv. Drug Deliv. Rev. 14:1994;1-24.
73. Morris M.R., Pereira M.M.C., Hallett M.B., McPherson M.A., Dormer R.L. Cellular location of the most common mutant form of the CF gene proteins ΔF508-CFTR. Biochem. Soc. Trans. 26:1998;S293.
74. Olsen J.C., Johnson L.G., Stutts M.J., Sarkadi B., Yankaskas J.R., Swanstrom R., Boucher R.C. Correction of the apical membrane chloride permeability defect in polarized cystic fibrosis airway epithelia following retroviral-mediated gene transfer. Hum. Gene Ther. 3:1992;253-266.
75. - channel is functional when retained in endoplasmic reticulum of mammalian cells. J. Biol. Chem. 270:1995;12347-12350.
76. Park P.J., Leeflang B., Vliegenthart J.F.G., Rhim A.D., Scanlin T.F., Glick M.C. Primary and immortalized CF airway epithelial cells express a selectin ligand. Pediatr. Pulmonol. Suppl. 14:1997;415.
77. Pearson R.D., Lubin A.H. Increased heparin binding in cystic fibrosis: a reflection of altered glycoprotein biosynthesis. Pediatr. Res. 13:1979;834-840.
78. Pfeifer A.M.A., Mark G.E., Malan-Shibley L. Cooperation of C-raf-I and C-myc protooncogenes in the neoplastic transformation of simian virus 40 large tumor antigen-immortalized human bronchial epithelial cells. Proc. Natl. Acad. Sci. USA. 86:1989;10075-10079.
79. Pind S., Riordan J.R., Williams D.B. Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 269:1994;12784-12788.
80. Prince L.S., Workman J., Marchase R.B. Rapid endocytosis of the cystic fibrosis transmembrane conductance regulator chloride channel. Proc. Natl. Acad. Sci. USA. 91:1994;5192-5196.
81. Redell R.R., Ke Y., Gerwin B.L., McMenamin M.G., Lechner J.F., Su R.T., Brash D.E., Park J.B., Rhim J.S., Harris C.C. Transformation of human bronchial epithelial cells by infection with SV40 or adenovirus-12 SV40 hybrid virus, or transfection via strontium phosphate coprecipitation with a plasmid containing SV40 early region genes. Cancer Res. 48:1988;1904-1909.
82. Riordan J.R., Rommens J.M., Kerem B., Alon M., Rozmahel R., Grzelczak Z., Zielenski J., Lok S., Plavsic N., Chou J.-L., Drumm M.L., Iannuzzi M.C., Collins F.S., Tsui L.-C. Identification of the cystic fibrosis gene: cloning and characterization of the complementary DNA. Science. 245:1989;1066-1073.
83. C. Robinson, T.F. Scanlin, Cystic fibrosis, in: A.P. Fishman (Ed.), Pulmonary Diseases and Disorders, McGraw-Hill, New York, 1997, pp. 803-824.
84. Roelfs R.E., Gibbs G.E., Griffin G.D. The composition of rectal mucus in cystic fibrosis. Am. J. Dis. Child. 113:1967;419-421.
85. Rommens J.M., Iannuzzi M.C., Kerem B., Drumm M.L., Melner B., Dean M., Rozmahel R., Cole J.L., Kennedy D., Hidaka N., Zsiga M., Buchwald M., Riordan J.R., Tsui L.C., Collins F.S. Identification of the cystic fibrosis gene: chromosome walking and jumping. Science. 245:1989;1059-1065.
86. Rose M.C. Epithelial mucous glycoproteins and cystic fibrosis. Horm. Metab. Res. 20:1988;601-608.
87. Rose M.C. Mucins: structure, function, and role in pulmonary diseases. Am. J. Physiol. 263:1992;L413-L429.
88. Rosenfeld M.D., Collins F.S. Gene therapy for cystic fibrosis. Chest. 109:1996;241-252.
89. Rotoli B.M., Bussolati O., Sironi M., Cabrini G., Gazzola G.C. CFTR protein is involved in the efflux of neutral amino acids. Biochem. Biophys. Res. Commun. 204:1994;653-658.
90. Roussel P., Lamblin G., Degand P., Walker-Nasir E., Jeanloz R.W. Heterogeneity of the carbohydrate chains of sulfated bronchial glycoproteins isolated from a patient suffering from cystic fibrosis. J. Biol. Chem. 250:1975;2114-2122.
91. Rubenstein R.C., Egan M.E., Zeitlin P.L. In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing ΔF508-CFTR. J. Clin. Invest. 100:1997;2457-2465.
92. Saiman L., Prince A.S. Pseudomonas aeruginosa pili bind to asialo GM1 which is increased on the surface of cystic fibrosis epithelial cells. J. Clin. Invest. 92:1993;1875-1880.
93. Sarkadi B., Bauzon D., Huckle W.R., Earp H.S., Berry A., Suchindran H., Price E.M., Olsen J.C., Boucher R.C., Scarborough G.A. Biochemical characterization of the cystic fibrosis transmembrane conductance regulator in normal and cystic fibrosis epithelial cells. J. Biol. Chem. 267:1992;2087-2095.
94. Sato S., Ward C.L., Kopito R.R. Cotranslational ubiquitination of cystic fibrosis transmembrane conductance regulator in vitro. J. Biol. Chem. 273:1998;7189-7192.
95. Scanlin T.F., Matacic S.S., Pace M., Santer U.V., Glick M.C. Abnormal distribution of α-L-fucosidase in cystic fibrosis: Increased activity in skin fibroblasts. Biochem. Biophys. Res. Commun. 79:1977;869-875.
96. Scanlin T.F., Matacic S.S., Glick M.C. Abnormal distribution of α-L-fucosidase in cystic fibrosis: decreased activity in serum. Clin. Chim. Acta. 91:1979;197-202.
97. T.F. Scanlin, M.C. Glick, Dynamic and structural aspects of cystic fibrosis fibroblast membranes, in: J.M. Sturgess (Ed.), Perspectives in Cystic Fibrosis Proceedings, 8th International Cystic Fibrosis Congress, Canadian Cystic Fibrosis Foundation, Toronto, 1980, pp. 44-57.
98. Scanlin T.F., Voynow J.A., Thomas E.J., Glick M.C. Glycoproteins in culture medium: a comparison from cystic fibrosis and control skin fibroblasts. Biochemistry. 21:1982;491-497.
99. Scanlin T.F., Wang Y.-M., Glick M.C. Altered fucosylation of membrane glycoproteins from cystic fibrosis fibroblasts. Pediatr. Res. 19:1985;368-374.
100. Scanlin T.F., Liu A., Park P.J., Rhim A.D., Kothari V., Weiser J.N., Glick M.C. Fucosylation and sialylation of cystic fibrosis (CF) airway epithelial cells. Glycobiology. 8:1998;150.
101. Scharfman A., Brussel E.V., Houdret N., Lamblin G., Roussel P. Interactions between glycoconjugates from human respiratory airways and Pseudomonas aeruginosa. J. Respir. Crit. Care Med. 154:1996;5163-5169.
102. Seksek O., Biwersi J., Verkman A.S. Evidence against defective trans-Golgi acidification in cystic fibrosis. J. Biol. Chem. 271:1996;15542-15548.
103. Smith A. Pathogenesis of bacterial bronchitis in cystic fibrosis. Pediatr. Infect. Dis. J. 16:1997;91-96.
104. Thiru S., Devereux G., King A. Abnormal fucosylation of ileal mucus in cystic fibrosis. I. A histochemical study using peroxidase labelled lectins. J. Clin. Pathol. 43:1990;1014-1018.
105. Thomassen M.J., Demko C.A., Wood R.E., Tandler B., Dearborn D.G., Boxerbaum B., Kuchenbrod P.J. Ultrastructure and function of alveolar macrophages from cystic fibrosis patients. Pediatr. Res. 14:1980;715-721.
106. Thomsson K.A., Ingemar C., Karlsson N.G., Karlsson H., Hansson G.C. Different O-glycosylation of respiratory mucin glycopeptides from a patient with cystic fibrosis. Glycoconjugate J. 15:1998;823-833.
107. 1H-NMR spectroscopy. Eur. J. Biochem. 127:1982;7-20.
108. Verma I.M., Somia N. Gene therapy: promises, problems and prospects. Nature. 389:1997;239-242.
109. Wadhwa M.S., Knoll D.L., Young A.P., Rice K.G. Targeted gene delivery with a low molecular weight glycopeptide carrier. Bioconjugate Chem. 6:1995;283-291.
110. Wang Y.M., Hare T.R., Won B., Stowell C.P., Scanlin T.F., Glick M.C., Hard K., van Kuik J.A., Vliegenthart J.F.G. Additional fucosyl residues on membrane glycoproteins but not a secreted glycoprotein from cystic fibrosis fibroblasts. Clin. Chim. Acta. 188:1990;193-210.
111. Ward C.L., Omura S., Kopito R.R. Degradation of CFTR by the ubiquitin-proteasome pathway. Cell. 83:1995;121-127.
112. Wei X., Eisman R., Xu J., Harsch A.D., Mulberg A.E., Bevins C.L., Glick M.C., Scanlin T.F. Turnover of the cystic fibrosis transmembrane conductance regulator (CFTR): slow degradation of wild-type and ΔF508 CFTR in surface membrane preparations of immortalized airway epithelial cells. J. Cell. Physiol. 168:1996;373-384.
113. Weiser J.N., Chong S.T.H., Greenberg D., Fong W. Identification and characterization of a cell envelop protein of Haemophilus influenzae contributing to phase variation in colony opacity and nasopharyngeal colonization. Med. Microbiol. 17:1995;555-564.
114. Welsh M.J., Smith A.E. Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. Cell. 73:1993;1251-1254.
115. M.J. Welsh, L.-C., Tsui, T.F. Boat, A.L. Beaudet, Cystic fibrosis, in: C.L. Scribner, A.L. Beaudet, W.S. Sly, S.B. Fiel, (Eds.), The Metabolic and Molecular Bases of Inherited Disease, 7th edn., McGraw-Hill, New York, 1995, pp. 3799-3876.
116. Wesley A., Forstner J., Qureshi R., Mantle M., Forstner G. Human intestinal mucin in cystic fibrosis. Pediatr. Res. 17:1983;65-69.
117. Weyer P., Barasch J., Al Awqati Q., Ausiello D.A., Brown D. Immunolocalization of two sialyltransferases is altered in polarized LLLC-PK1 epithelial cells expressing ΔF508 CFTR. Pediatr. Pulmonol. 12:1995;238.
118. Yang Y., Janich S., Cohn J.A., Wilson J.M. 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. 90:1993;9480-9484.
119. Zar H., Saiman L., Quittell L., Prince A. Binding of Pseudomonas aeruginosa to respiratory epithelial cells from patients with various mutations in the cystic fibrosis transmembrane regulator. J. Pediatr. 126:1995;230-233.
120. Zeitlin P.L., Lu L., Rhim J., Cutting G., Stetten G., Kiefer K.A., Craig R., Guggino W.B. A cystic fibrosis bronchial epithelial cell line: immortalization by adeno 12-SV40 infection. Am. J. Respir. Cell Mol. Biol. 4:1991;313-319.
121. Zhang Y., Doranz B., Yankaskas J.R., Engelhardt J.F. Genotypic analysis of respiratory mucous sulfation defects in cystic fibrosis. J. Clin. Invest. 96:1995;2997-3004.