Access the full text.
Sign up today, get DeepDyve free for 14 days.
C. Koch, N. Høiby (2000)
Diagnosis and Treatment of Cystic FibrosisRespiration, 67
J. Lo-Guidice, J. Wieruszeski, J. Lemoine, A. Verbert, P. Roussel, G. Lamblin (1994)
Sialylation and sulfation of the carbohydrate chains in respiratory mucins from a patient with cystic fibrosis.The Journal of biological chemistry, 269 29
David Thornton, N. Khan, Ravi Mehrotra, Marj Howard, Enno Veerman, N. Packer, J Sheehan (1999)
Salivary mucin MG1 is comprised almost entirely of different glycosylated forms of the MUC5B gene product.Glycobiology, 9 3
J. Barasch, Q. Al-Awqati (1993)
Defective acidification of the biosynthetic pathway in cystic fibrosisJournal of Cell Science, 1993
J. Garrett, L. Anderson (1991)
Rat sublingual salivary glands: secretory changes on parasympathetic or sympathetic nerve stimulation and a reappraisal of the adrenergic innervation of striated ducts.Archives of oral biology, 36 9
C. Bombieri, L. Gilè, M. Benetazzo, A. Saccomani, M. Luisetti, P. Pignatti (1997)
Genotype-phenotype correlations in Cystic Fibrosis.
J. Barasch, Brian Kiss, A. Prince, L. Saiman, D. Gruenert, Qais AI-Awqati (1991)
Defective acidification of intracellular organelles in cystic fibrosisNature, 352
D. Shori, H. Kariyawasam, R. Knight, M. Hodson, T. Genter, J. Hansen, C. Koch, A. Kalogeridis (2001)
Sulphation of the salivary mucin MG1 (MUC-5B) is not correlated to the degree of its sialylation and is unaffected by cystic fibrosisPflügers Archiv, 443
G. Cacalano, M. Kays, L. Saiman, A. Prince (1992)
Production of the Pseudomonas aeruginosa neuraminidase is increased under hyperosmolar conditions and is regulated by genes involved in alginate expression.The Journal of clinical investigation, 89 6
C. Lenaerts (1997)
Genotype ‐ phenotype correlations: Where are we?Pediatric Pulmonology, 23
L. Joris, I. Dab, P. Quinton (1993)
Elemental composition of human airway surface fluid in healthy and diseased airways.The American review of respiratory disease, 148 6 Pt 1
Ravi Mehrotra, David Thornton, J Sheehan (1998)
Isolation and physical characterization of the MUC7 (MG2) mucin from saliva: evidence for self-association.The Biochemical journal, 334 ( Pt 2)
M. Super (1992)
Milestones in cystic fibrosis.British medical bulletin, 48 4
I. Iontcheva, F. Oppenheim, R. Troxler (1997)
Human Salivary Mucin MG1 Selectively Forms Heterotypic Complexes with Amylase, Proline-rich Proteins, Statherin, and HistatinsJournal of Dental Research, 76
Steve Nelson, Warren Summer (1998)
Innate immunity, cytokines, and pulmonary host defense.Infectious disease clinics of North America, 12 3
Peter Nielsen, E. Bennett, H. Wandall, M. Therkildsen, Jens Hannibal, Henrik Clausen (1997)
Identification of a major human high molecular weight salivary mucin (MG1) as tracheobronchial mucin MUC5B.Glycobiology, 7 3
D. Devor, Ashvani Singh, L. Lambert, A. DeLuca, R. Frizzell, R. Bridges (1999)
Bicarbonate and Chloride Secretion in Calu-3 Human Airway Epithelial CellsThe Journal of General Physiology, 113
R. Ramphal (1999)
The molecular basis of mucin-Pseudomonas interactionsBiochemical Society Transactions, 27
Prerna Sharma, L. Dudus, Peter Nielsen, Henrik Clausen, James Yankaskas, Michael Hollingsworth, John Engelhardt (1998)
MUC5B and MUC7 are differentially expressed in mucous and serous cells of submucosal glands in human bronchial airways.American journal of respiratory cell and molecular biology, 19 1
C. Jiang, Walter Finkbeiner, Jonathan Widdicombe, S. Miller (1997)
Fluid transport across cultures of human tracheal glands is altered in cystic fibrosisThe Journal of Physiology, 501
S. Thiru, G. Devereux, A. King (1990)
Abnormal fucosylation of ileal mucus in cystic fibrosis: I. A histochemical study using peroxidase labelled lectins.Journal of Clinical Pathology, 43
C. Carnoy, R. Ramphal, A. Scharfman, J. Lo-Guidice, N. Houdret, A. Klein, C. Galabert, G. Lamblin, P. Roussel (1993)
Altered carbohydrate composition of salivary mucins from patients with cystic fibrosis and the adhesion of Pseudomonas aeruginosa.American journal of respiratory cell and molecular biology, 9 3
G. Forstner (1995)
Signal transduction, packaging and secretion of mucins.Annual review of physiology, 57
D. Thornton, N. Khan, R. Mehrotra, M. Howard, E. Veerman, N. Packer, J. Sheehan (1999)
Salivary mucin MG 1 is comprised almost entirely of different glycosylated forms of the MUC 5 B gene product
M. Reddy, M. Levine, W. Paranchych (1993)
Low-molecular-mass human salivary mucin, MG2: structure and binding of Pseudomonas aeruginosa.Critical reviews in oral biology and medicine : an official publication of the American Association of Oral Biologists, 4 3-4
J. Zieleński (2000)
Genotype and Phenotype in Cystic FibrosisRespiration, 67
A. King, M. Mcleish, S. Thiru (1990)
Abnormal fucosylation of-ileal mucus in cystic fibrosis: II. A histochemical study using monoclonal antibodies to fucosyl oligosaccharides.Journal of Clinical Pathology, 43
Peter Nielsen, Ulla Mandel, M. Therkildsen, Henrik Clausen (1996)
Differential Expression of Human High-molecular-weight Salivary Mucin (MG1) and Low-molecular-weight Salivary Mucin (MG2)Journal of Dental Research, 75
Destruction of the lungs as a consequence of recurrent infections with microorganisms such as Pseudomonas aeruginosa remains the underlying cause of most morbidity and mortality in cystic fibrosis (CF). We have hypothesized that changes in the glycosylation of key tracheal mucins such as MUC5B and MUC7 might increase the risk of pulmonary disease in CF patients. However, in preference to sputum we have examined the sugar-chains on these mucins in saliva because in the latter not only can the glycoproteins be collected from controls, but they are essentially free from modifications made following bacterial infection in disease. Proteins in ductal or whole-mouth saliva from 20 CF patients with the Δ F-508 CFTR mutation and age-and sex-matched controls were separated by SDS-PAGE and blotted onto nitrocellulose and then probed with labelled lectins of known specificity. Linkage of terminal sialic acid on the blotted mucins was determined using Sambucus nigra agglutinin (detects the 2→6 linkage) and Maackia amurensis agglutinin (the 2→3 linkage). Fucose was detected by Ulex europaeus agglutinin-1 (1→2 linkage) and Aleuria aurantia agglutinin (1→3 linkage). We found that each mucin shows a characteristic glycosylation pattern and in controls most of the sialic acid is 2→6 linked on MG1 (MUC 5B) and 2→3 linked on MG2 (MUC 7). CF is associated with a shift from a 2→6 linkage to a 2→3 linkage on MG1 with some patients showing almost no 2→6 linkage; 2→3 linkage on MG2 is similarly increased in disease in some individuals. The expression of fucose on these mucins is also raised in CF patients. These shift to a 2→3 linkage of sialic acid, and with increased fucosylation this promotes the formation of sialyl-Lewis X antigen detected on CF mucins in our study. These changes will be tested for their correlation with the severity of lung disease. We gratefully acknowledge support from the European Union Biomed-II Programme.
Pflügers Archiv European Journal of Physiologyl of Physiology – Springer Journals
Published: Nov 28, 2001
Read and print from thousands of top scholarly journals.
Already have an account? Log in
Bookmark this article. You can see your Bookmarks on your DeepDyve Library.
To save an article, log in first, or sign up for a DeepDyve account if you don’t already have one.
Copy and paste the desired citation format or use the link below to download a file formatted for EndNote
Access the full text.
Sign up today, get DeepDyve free for 14 days.
All DeepDyve websites use cookies to improve your online experience. They were placed on your computer when you launched this website. You can change your cookie settings through your browser.