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Glycobiology and Medicine pp 17–32Cite as

The Glycosylation of Airway Mucins in Cystic Fibrosis and its Relationship with Lung Infection by Pseudomonas aeruginosa

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Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 535))

Abstract

Cystic fibrosis (CF) is the most common severe genetic disease among Caucasians (1/2500–1/3000 births). It affects the exocrine glands and, in its most typical form, the main symptoms are a chronic pulmonary disease, a pancreatic insufficiency with fat malabsorption, a meconium ileus at birth (in 10% CF neonates) and, later on, cirrhosis and male sterility. The diagnosis is based on the elevation of sweat electrolytes (sweat chloride: ⁥70 mEq/L). In CF, there is a mucus hyper secretion as in chronic bronchitis. However, unlike chronic bronchitis, the CF lung infection is very peculiar and is characterized by infection due to Staphylococcus aureus in early life and, rapidly if not directly, by Pseudomonas aeruginosa which is almost impossible to eradicate and is responsible for most of the morbidity and mortality of the disease (Welsh et al., 1995).

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References

  • Arora, S.K., Ritchings, B.W., Almira, E.C., Lory, S., and Ramphal, R., 1998, The Pseudomonas aeruginosa flagellar cap protein, FliD, is responsible for mucin adhesion, Infect Immun. 66:1000–1007.

    PubMed  CAS  Google Scholar 

  • Barasch, J., and Al-Awqati, Q., 1993, Defective acidification of the biosynthetic pathway in cystic fibrosis, J Cell Sci Suppl. 17:229–233.

    PubMed  CAS  Google Scholar 

  • Barasch, J., Kiss, B., Prince, A., Saiman, L., Gruenert, D., and Al-Awqati, Q., 1991, Defective acidification of intracellular organelles in cystic fibrosis, Nature. 352:70–73.

    Article  PubMed  CAS  Google Scholar 

  • Blackwell, T.S., Stecenko, A.A., and Christman, J.W., 2001, Dysregulated NF-KB activation in cystic fibrosis: evidence for a primary inflammatory disorder, Am J Physiol Lung Cell Mol Physiol. 281:L69–L70.

    PubMed  CAS  Google Scholar 

  • Bonfield, T.L., Panuska, J.R., Konstan, M.W., Hilliard, K.A., Hilliard, J.B., Ghnaim, H., and Berger, M., 1995, Inflammatory cytokines in cystic fibrosis lungs, Am J Respir Crit Care Med. 152:2111–2118.

    PubMed  CAS  Google Scholar 

  • Borchers, M.T., Carty, M.P., and Leikauf, G.D., 1999, Regulation of human airway mucins by acrolein and inflammatory mediators, Am J Physiol. 276:L549–L555.

    PubMed  CAS  Google Scholar 

  • Cantin, A., 1995, Cystic fibrosis lung inflammation: early, sustained, and severe, Am J Respir Crit Care Med. 151:939–941.

    PubMed  CAS  Google Scholar 

  • Carnoy, C., Ramphal, R., Scharfman, A., Houdret, N., Lo-Guidice, J.-M., Klein, A., Galabert, C., Lamblin, G., and Roussel, P., 1993, Altered carbohydrate composition of salivary mucins from patients with cystic fibrosis and the adhesion of Pseudomonas aeruginosa, Am J Respir Cell Mol Biol. 9:323–334.

    PubMed  CAS  Google Scholar 

  • Carnoy, C., Scharfman, A., Van Brussel, E., Lamblin, G., Ramphal, R., and Roussel, P., 1994, Pseudomonas aeruginosa outer membrane adhesins for human respiratory mucus glycoproteins, Infect Immun. 62:1896–1900.

    PubMed  CAS  Google Scholar 

  • Chace, K.V., Flux, M., and Sachdev, G.P., 1985, Comparison of physicochemical properties of purified mucus glycoproteins isolated from respiratory secretions of cystic fibrosis and asthmatic patients, Biochemistry. 24:7334–7341.

    Article  PubMed  CAS  Google Scholar 

  • Cheng, P.W., Boat, T.F., Cranfill, K., Yankaskas, J.R., and Boucher, R.C., 1989, Increased sulfation of glycoconjugates by cultured nasal epithelial cells from patients with cystic fibrosis, J Clin Invest. 84:68–72.

    Article  PubMed  CAS  Google Scholar 

  • Chmiel, J.F., Berger, M., and Konstan, M.W., 2002, The role of inflammation in the pathophysiology of CF lung disease, Clin Rev Allergy Immunol. 23:5–27.

    Article  PubMed  Google Scholar 

  • Couceiro, J.N., Paulson, J.C., and Baum, L.G., 1993, Influenza virus strains selectively recognize sialyloligosac-charides on human respiratory epithelium: the role of the host cell in selection of hemagglutinin receptor specificity, Virus Res. 29:155–165.

    Article  PubMed  CAS  Google Scholar 

  • Cystic Fibrosis Mutation Data Base—http://www.genet.sickkids.on.ca/cftr/201.

    Google Scholar 

  • Dabbagh, K., Takeyama, K., Lee, H.M., Ueki, LE, Lausier, J.A., and Nadel, J.A., 1999, IL-4 induces mucin gene expression and goblet cell metaplasia in vitro and in vivo, J Immunol. 162:6233–6237.

    PubMed  CAS  Google Scholar 

  • Dakin, C.J., Numa, A.H., Wang, H., Morton, J.R., Vertzyas, C.C., and Henry, R.L., 2002, Inflammation, infection, and pulmonary function in infants and young children with cystic fibrosis, Am J Respir Crit Care Med. 165:904–910, 904-10.

    PubMed  Google Scholar 

  • Davril, M., Degroote, S., Humbert, P., Galabert, C., Dumur, V., Lafitte, J.-J., Lamblin, G., and Roussel, P., 1999, The sialylation of bronchial mucins secreted by patients suffering from cystic fibrosis or from chronic bronchitis is related to the severity of airway infection, Glycobiology. 9:311–321.

    Article  PubMed  CAS  Google Scholar 

  • Debailleul, V., Laine, A., Huet, G., Mathon, P., d’Hooghe, M.C., Aubert, J.-P, and Porchet, N., 1998, Human mucin genes MUC2, MUC3, MUC4, MUC5AC, MUC5B, and MUC6 express stable and extremely large mRNAs and exhibit a variable length polymorphism—An improved method to analyze large mRNAs, J Biol Chem. 273:881–890.

    Article  PubMed  CAS  Google Scholar 

  • de Bentzmann, S., Roger, P., Dupuit, F., Bajolet-Laudinat, O., Fuchey, C., Plotkowski, M.C., and Puchelle, E., 1996, Asialo GM1 is a receptor for Pseudomonas aeruginosa adherence to regenerating respiratory epithelial cells, Infect Immun. 64:1582–1588.

    PubMed  Google Scholar 

  • De Graaf, T.W., Van der Stelt, M.E., Anbergen, M.G., and van Dijk, W., 1993, Inflammation-induced expression of sialyl-Lewis X-containing glycan structures on α1-acid glycoprotein (orosomucoid) in human sera, J Exp Med. 177:657–666.

    Article  PubMed  Google Scholar 

  • Degroote, S., Maes, E., Humbert, P., Delmotte, P., Lamblin, G., and Roussel, P., 2003, Sulfated oligosaccharides isolated from the respiratory mucins of a secretor patient suffering from chronic bronchitis, Biochimie. 85:369–379

    Article  PubMed  CAS  Google Scholar 

  • Delmotte, P., Degroote, S., Lafitte, J.-J., Lamblin, G., Perini, J.-M., and Roussel, P., 2002, Tumor necrosis factor alpha increases the expression of glycosyltransferases and sulfotransferases responsible for the biosynthesis of sialylated and/or sulfated Lewis x epitopes in the human bronchial mucosa, J Biol Chem. 277:424–431.

    Article  PubMed  CAS  Google Scholar 

  • Delmotte, P., Degroote, S., Merten, M., Bernigaud, A., Van Seuningen, I., Figarella, C., Roussel, P., and Perini, J.-M, 2001, Influence of culture conditions on the alpha 1,2-fucosyltransferase and MUC gene expression of a transformed cell line MM-39 derived from human tracheal gland cells, Biochimie. 83:749–755.

    Article  PubMed  CAS  Google Scholar 

  • Devaraj, N., Sheykhnazari, M., Warren, W.S., Bhavanandan, V.P., 1994, Differential binding of Pseudomonas aeruginosa to normal and cystic fibrosis tracheobronchial mucins, Glycobiology. 4:307–316.

    Article  PubMed  CAS  Google Scholar 

  • Devidas, S. and Guggino, W.B., 1997, CFTR: domains, structure, and function, Bioenerg Biomembr. 29:443–451.

    Article  CAS  Google Scholar 

  • Dosanjh, A., Lencer, W., Brown, D., Ausiello, D.A., and Stow, J.L., 1994, Heterologous expression of ΔF508 CFTR results in decreased sialylation of membrane glycoconjugates, Am J Physiol. 266:C360–C366.

    PubMed  CAS  Google Scholar 

  • Engelhardt, J.F., Yankaskas, J.R., Ernst, S.A., Yang, Y., Marino, C.R., Boucher, R.C., Cohn, J.A., and Wilson, J.M., 1992, Submucosal glands are the predominant site of CFTR expression in the human bronchus, Nat Genet. 3:240–248.

    Article  Google Scholar 

  • Engelhardt, J.F., Zepeda, M., Cohn, J.A., Yankaskas, J.R., Wilson, J.M., 1994, Expression of the cystic fibrosis gene in adult human lung, J Clin Invest. 93:737–749.

    Article  PubMed  CAS  Google Scholar 

  • Frates, R.C. Jr, Kaizu, T.T., and Last, J.A., 1983, Mucus glycoproteins secreted by respiratory epithelial tissue from cystic fibrosis patients, Pediatr Res. 17:30–34.

    Article  PubMed  CAS  Google Scholar 

  • Glick, M.C., Kothari, V.A., Liu, A., Stoykova, L.I., and Scanlin, T.F., 2001, Activity of fucosyltransferases and altered glycosylation in cystic fibrosis airway epithelial cells, Biochimie. 83:743–747.

    Article  PubMed  CAS  Google Scholar 

  • Jacquot, J., Puchelle, E., Hinnrasky, J., Fuchey, C., Bettinger, C., Spilmont, C., Bonnet, N., Dieterle, A., Dreyer, D, Pavirani, A. et al., 1993, Localization of the cystic fibrosis transmembrane conductance regulator in airway secretory glands, Eur Respir J. 6:169–176.

    PubMed  CAS  Google Scholar 

  • Jeffery, P.K. and Brain, A.P.R., 1988, Surface morphology of human airway mucosa: normal, carcinoma or cystic fibrosis, Scan Microsc. 2:345–351.

    Google Scholar 

  • Karpati, F., Hjelte, F.L., and Wretlind, B., 2000, TNF-alpha and IL-8 in consecutive sputum samples from cystic fibrosis patients during antibiotic treatment, Scand J Infect Dis. 32:75–79.

    Article  PubMed  CAS  Google Scholar 

  • Konstan, M.W., Hilliard, K.A., Norvell, T.M., and Berger, M., 1994, Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung diseases suggest ongoing infection and inflammation, Am J Respir Crit Care Med. 150:448–454.

    PubMed  CAS  Google Scholar 

  • Krivan, H.C., Ginsburg, V., Roberts, D.D., 1988, Pseudomonas aeruginosa and Pseudomonas cepacia isolated from cystic fibrosis patients bind specifically to gangliotetraosylceramide (asialo GM1) and gangliotriao-sylceramide (asialo GM2), Arch Biochem Biophys. 260:493–496.

    Article  PubMed  CAS  Google Scholar 

  • Lamblin, G., Degroote, S., Perini, J.-M., Delmotte, P., Scharfman, A., Davril, M., Lo-Guidice J.-M., Houdret, N., Dumur, V., Klein, A., and Roussel, P., 2001, Human airway mucin glycosylation: a combinatory of carbohydrate determinants which vary in cystic fibrosis, Glycoconj J. 18:661–684.

    Article  PubMed  CAS  Google Scholar 

  • Lamblin, G., Lafitte, J.-J., Lhermitte, M., Degand, P., and Roussel, P., 1977, Mucins from cystic fibrosis sputum, Mod Probl Paediat. 19:153–164.

    CAS  Google Scholar 

  • Levine, S.J., Larivee, P., Logun, C., Angus, C.W., Ognibene, F.P., and Shelhamer, J.H., 1995, Tumor necrosis factor-α induces mucin hypersecretion and MUC-2 gene expression by human airway epithelial cells, Am J Respir Cell Mol Biol. 12:196–204.

    PubMed  CAS  Google Scholar 

  • Lo-Guidice, J.-M., Herz, H., Lamblin, G., Plancke, Y., Roussel, P., and Lhermitte, M., 1997, Structures of sulfated oligosaccharides isolated from the respiratory mucins of a non-secretor (O, Lea+b-) patient suffering from chronic bronchitis, Glycoconj J. 14:113–125.

    Article  PubMed  CAS  Google Scholar 

  • Lo-Guidice, J.-M., Merten, M.D., Lamblin, G., Porchet, N., Houvenaghel, M.-C., Figarella, C., Roussel, P., and Perini, J.-M., 1997, Mucins secreted by a transformed cell line derived from human tracheal gland cells, Biochem J. 326:431–437.

    PubMed  CAS  Google Scholar 

  • Lo-Guidice, J.-M., Wieruszewski, J.-M., Lemoine, J., Verbert, A., Roussel, P., and Lamblin, G., 1994, Sialylation and sulfation of the carbohydrate chains in respiratory mucins from a patient with cystic fibrosis, J Biol Chem. 269:18794–18813.

    PubMed  CAS  Google Scholar 

  • Longphre, M., Li, D., Gallup, M., Drori, L., Ordonez, C.L., Redman, T, Wenzel, S., Bice, D.E., Fahy, J.V., and Basbaum, C., 1999, Allergen-induced IL-9 directly stimulates mucin transcription in epithelial cells, J Clin Invest. 104:1375–1382.

    Article  PubMed  CAS  Google Scholar 

  • Louahed, J., Toda, M., Jen, J., Hamid, Q., Renauld, J.C., Levitt, R.C., and Nicolaides, N.C., 2000, Interleukin-9 upregulates mucus expression in the airways, Am J Respir Cell Mol Biol. 22:649–656.

    PubMed  CAS  Google Scholar 

  • Lukacs, N.W., Strieter, R.M., Chensue, S.W., Widmer, M., and Kunkel, S.L., 1995, TNF-alpha mediates recruitment of neutrophils and eosinophils during airway inflammation, J Immunol. 154:5411–5417.

    PubMed  CAS  Google Scholar 

  • McNamara, N. and Basbaum, C., 2001, Signaling networks controlling mucin production in response to Gram-positive and Gram-negative bacteria, Glycoconj J. 18:715–722.

    Article  PubMed  CAS  Google Scholar 

  • Matsui, H., Davis, C.W., Tarran, R., and Boucher, R.C., 2000, Osmotic water permeabilities of cultured, well-differentiated normal and cystic fibrosis airway epithelia, J Clin Invest. 105:1418–1427.

    Article  Google Scholar 

  • Mohapatra, N.K., Cheng, P.W., Parker, J.C., Paradiso, A.M., Yankaskas, J.R., Boucher, R.C., and Boat, T.F., 1995, Alteration of sulfation of glycoconjugates, but not sulfate transport and intracellular inorganic sulfate content in cystic fibrosis airway epithelial cells, Pediatr Res. 38:42–48.

    Article  PubMed  CAS  Google Scholar 

  • Moniaux, N., Escande, F., Porchet, N., Aubert, J.-P., and Batra, S.K., 2001, Structural organization and classification of the mucin genes, Front Biosci. 6:d1192–1206.

    Article  PubMed  CAS  Google Scholar 

  • Morelle, W., Sutton-Smith, M, Morris, H.R., Davril, M., Roussel, P., and Dell, A., 2001, FAB-MS characterization of sialyl Lewisx determinants on polylactosamine chains of human airway mucins secreted by patients suffering from cystic fibrosis or chronic bronchitis, Glycoconj J. 18:699–708.

    Article  PubMed  CAS  Google Scholar 

  • Oceandy, D., McMorran, B.J., Smith, S.N., Schreiber, R., Kunzelmann, K., Alton, E.W., Hume, D.A., and Wainwright, B.J., 2002, Gene complementation of airway epithelium in the cystic fibrosis mouse is necessary and sufficient to correct the pathogen clearance and inflammatory abnormalities, Hum Mol Genet. 11:1059–1067.

    Article  PubMed  CAS  Google Scholar 

  • Osika, E., Cavaillon, J.-M., Chadelat, K., Boule, M., Fitting, C., Tournier, G., and Clement, A., 1999, Distinct sputum cytokine profiles in cystic fibrosis and other chronic inflammatory airway disease, Eur Respir J. 14:339–346.

    Article  PubMed  CAS  Google Scholar 

  • Pasyk, E.A. and Foskett, J.K., 1997, Cystic fibrosis transmembrane conductance regulator-associated ATP and adenosine 3′-phosphate 5′-phosphosulfate channels in endoplasmic reticulum and plasma membranes, J Biol Chem. 272:7746–7751.

    Article  PubMed  CAS  Google Scholar 

  • Perez-Vilar, J., Randell, S.H., and Boucher, R., 2002, The cys subdomains of human gel-forming mucins are C-mannosylated domains involved in weak protein-protein interactions, Pediatric Pulmonol. Suppl 24:190.

    Google Scholar 

  • Perrais, M., Pigny, P., Copin, M.-C., Aubert, J.-P, and Van Seuningen, I., 2002, Induction of MUC2 and MUC5AC mucins by factors of the epidermal growth factor (EGF) family is mediated by EGF receptor/ Ras/Raf/extracellular signal-regulated kinase cascade and Spl, J Biol Chem. 277:32258–32267.

    Article  PubMed  CAS  Google Scholar 

  • Poschet, J.F., Boucher, J.C., Tatterson, L., Skidmore, J., Van Dyke, R.W., and Deretic, V., 2001, Molecular basis for defective glycosylation and Pseudomonas pathogenesis in cystic fibrosis lung, Proc Natl Acad Sci USA. 98:13972–13977.

    Article  PubMed  CAS  Google Scholar 

  • Ramphal, R. and Arora, S.K., 2001, Recognition of mucin components by Pseudomonas aeruginosa, Glycoconj J. 18:709–713.

    Article  PubMed  CAS  Google Scholar 

  • Ramphal, R., Carnoy, C., Fievre, S., Michalski, J.-C., Houdret, N., Lamblin, G., Strecker, G., and Roussel, P., 1991a, Pseudomonas aeruginosa recognizes carbohydrate chains containing type 1 (Galβ1-3GlcNAc) or type 2 (Galβ1-4GlcNAc) disaccharide units, Infect Immun. 59:700–704.

    PubMed  CAS  Google Scholar 

  • Ramphal, R., Houdret, N., Koo, L., Lamblin, G., and Roussel, P., 1989, Differences in adhesion of Pseudomonas aeruginosa to mucin glycopeptides from sputa of patients with cystic fibrosis and chronic bronchitis, Infect Immun. 57:3066–30671.

    PubMed  CAS  Google Scholar 

  • Ramphal, R., Koo, L., Ishimoto, K.S., Torten, P.A., Lara, J.C., and Lory, S., 1991b, Adhesion of Pseudomonas aeruginosa pilin-deficient mutants to mucin, Infect Immun. 59:1307–1311.

    PubMed  CAS  Google Scholar 

  • Ramphal, R., Small, P.M., Shands, J.W. Jr, Fischlschweiger, W., and Small, P.A. Jr, 1980, Adherence of Pseudomonas aeruginosa to tracheal cells injured by influenza infection or by endotracheal intubation. Infect Immun. 27:614–619.

    PubMed  CAS  Google Scholar 

  • Ramphal, R. and Vishwanath, S., 1987, Why is Pseudomonas the colonizer and why does it persist, Infection. 15:281–287.

    Article  PubMed  CAS  Google Scholar 

  • Rhim, A.D., Stoykova, L., Glick, M.C., and Scanlin, T.F., 2001, Terminal glycosylation in cystic fibrosis (CF): a review emphasizing the airway epithelial cell, Glycoconj J. 8:649–659

    Article  Google Scholar 

  • Riordan, J.R., 1999, Cystic fibrosis as a disease of misprocessing of the cystic fibrosis transmembrane conductance regulator glycoprotein, Am J Hum Genet. 64:1499–1504.

    Article  PubMed  CAS  Google Scholar 

  • Rosenstein, I.J., Yuen, C.T., Stoll, M.S., and Feizi, T, 1992, Differences in the binding specificities of Pseudomonas aeruginosa M35 and Escherichia coli C600 for lipid-linked oligosaccharides with lactose-related core regions, Infect Immun. 60:5078–5084.

    PubMed  CAS  Google Scholar 

  • Roussel, P., Lamblin, G., Degand, P., Walker-Nasir, E., and Jeanloz, R.W., 1975, Heterogeneity of the carbohydrate chains of sulfated bronchial glycoproteins isolated from a patient suffering from cystic fibrosis, J Biol Chem. 250:2114–2122.

    PubMed  CAS  Google Scholar 

  • Saba, S., Soong, G., Greenberg, S., and Prince, A., 2002, Bacterial stimulation of epithelial G-CSF and GM-CSF expression promotes PMN survival in CF airways, Am J Respir Cell Mol Biol. 27:561–567.

    PubMed  CAS  Google Scholar 

  • Saiman, L. and Prince, A., 1993, Pseudomonas aeruginosa pili binds asialo-GM1 which is increased at the surface of cystic fibrosis epithelial cells, J Clin Invest. 92:1875–1880.

    Article  PubMed  CAS  Google Scholar 

  • Sajjan, U., Thanassoulis, G., Cherapanov, V., Lu, A., Sjolin, C., Steer, B., Wu, Y.J., Rotstein, O.D., Kent, G., McKerlie, C., Forstner, J., and Downey, P., 2001, Enhanced susceptibility to pulmonary infection with Burkholderia cepacia in Cftr -/- mice, Infect Immun. 69:5138–5150.

    Article  PubMed  CAS  Google Scholar 

  • Scharfman, A., Arora, S.K., Delmotte, P., Van Brussel, E., Mazurier, J., Ramphai, R., and Roussel, P., 2001, Recognition of Lewis x derivatives present on mucins by flagellar components of Pseudomonas aeruginosa, Infect Immun. 69:5243–5248.

    Article  PubMed  CAS  Google Scholar 

  • Scharfman, A., Degroote, S., Beau, J., Lamblin, G., Roussel, P., and Mazurier, J., 1999, Pseudomonas aeruginosa binds to neoglycoconjugates bearing mucin carbohydrate determinants and predominantly to sialyl-Lewis x conjugates. Glycobiology. 9:757–764.

    Article  PubMed  CAS  Google Scholar 

  • Scharfman, A., Delmotte, P., Beau, J., Lamblin, G., Roussel, P., and Mazurier, J., 2000, Sialyl-Le(x) and sulfo-sialyl-Le(x) determinants are receptors for P. aeruginosa. Glycoconj J. 10:735–740.

    Article  Google Scholar 

  • Seksek, O., Biwersi, J., and Verkman, A.S., 1996, Evidence against defective trans-Golgi acidification in cystic fibrosis, J Biol Chem. 271:15542–15548.

    Article  PubMed  CAS  Google Scholar 

  • Sheth, H.B., Lee, K.K., Wong, W.Y., Srivastava, G., Hindsgaul, O., Hodges, R.S., Paranchych, W., and Irvin, R.T., 1994, The pili of Pseudomonas aeruginosa strains PAK and PAO bind specifically to the carbohydrate sequence beta GalNAc(1–4)betaGal found in glycosphingolipids asialo-GM1 and asialo-GM2, Mol Microbiol. 11:715–23202.

    Article  PubMed  CAS  Google Scholar 

  • Shori, D.K., Genter, T., Hansen, J., Koch, C., Wyatt, H., Kariyawasam, H.H., Knight, R.A., Hodson, M.E., Kalogeridis, A., and Tsanakas, I., 2001, Altered sialyl-and fucosyl-linkage on mucins in cystic fibrosis patients promotes formation of the sialyl-Lewis X determinant on salivary MUC-5B and MUC-7, Pflugers Arch. 443Suppl:S55–S61.

    PubMed  CAS  Google Scholar 

  • Simel, D.L., Mastin, J.P., Pratt, P.C., Wisseman, C.L., Shelburne, J.D., Spock, A., Ingram, P., 1984, Scanning electron microscopic study of the airways in normal children and in patients with cystic fibrosis and other lung diseases, Pediatr Pathol. 2:47–64.

    Article  PubMed  CAS  Google Scholar 

  • van Heeckeren, A., Walenga, R., Konstan, M.W., Bonfield, T., Davis, P.B., and Ferkol, T., 1997, Excessive inflammatory response of cystic fibrosis mice to bronchopulmonary infection with Pseudomonas aeruginosa, J Clin Invest. 100:2810–2815.

    Article  PubMed  CAS  Google Scholar 

  • Welsh, M.J., Tsui, L.-C., Boat, T.F., and Beaudet, A.L., 1995, Cystic fibrosis, in: The metabolic and molecular bases of inherited disease. C.R. Scriver, A.L. Beaudet, W.S. Sly, and D. Valle, eds., (McGraw-Hill Inc.) pp. 3799–3876.

    Google Scholar 

  • Zhang, Y., Doranz, B., Yankaskas, J.R., and Engelhardt, J.F., 1995, Genotypic analysis of respiratory mucous sulfation defects in cystic fibrosis, J Clin Invest. 96:2997–3004.

    Article  PubMed  CAS  Google Scholar 

  • Zielenski, J. and Tsui, L.-C., 1995, Cystic fibrosis: genotypic and phenotypic variations, Annu Rev Genet. 29:777–807.

    Article  PubMed  CAS  Google Scholar 

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  1. Département de Biochimie Faculté de Médecine, Université de Lille, 2 place de Verdun, 59045, Lille, France

    Philippe Roussel & Geneviève Lamblin

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  1. Philippe Roussel
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  1. The Sir Joseph Hotung Centre for Musculoskeletal Diseases St Georges Hospital and Medical School, London, UK

    John S. Axford (Director) (Director)

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Roussel, P., Lamblin, G. (2003). The Glycosylation of Airway Mucins in Cystic Fibrosis and its Relationship with Lung Infection by Pseudomonas aeruginosa . In: Axford, J.S. (eds) Glycobiology and Medicine. Advances in Experimental Medicine and Biology, vol 535. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-0065-0_2

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