title: Additional fucosyl residues on membrane glycoproteins but not a secreted glycoprotein from cystic fibrosis fibroblasts.
abstract: Glycopeptides derived from peripheral membrane glycoproteins of skin fibroblasts of seven patients with cystic fibrosis (CF) had an increase in fucosyl residues when compared with those of seven age, race and sex matched controls (Pediatr Res 1985;19:368-374). To further define these results, the membrane glycopeptides which bound to immobilized lentil lectin and thereby enriched in fucosyl residues linked alpha 1----6 to N-acetylglucosamine attached to asparagine, were Pronase digested, partially purified and examined by 500-MHz 1H-NMR spectroscopy. The CF derived glycopeptides had two different features when compared to those from Controls (1) an increased number of fucosyl residues linked alpha 1----6 to the N-acetylglucosamine attached to asparagine and (2) fucosyl residues linked alpha 1----3 to a branch N-acetylglucosamine. The glycopeptides from both sources were of the di and triantennary type containing sialic acid linked alpha 2----3 and alpha 2----6 to galactose in an approximate molar ratio of 3:2 and 2:1, from CF and Control, respectively. Glycopeptides derived from a glycoprotein, fibronectin, secreted from CF fibroblasts were also examined by 1H-NMR spectroscopy and showed no evidence of fucosyl residues linked alpha 1----3 to branch N-acetylglucosamine and a lesser percentage of core fucose than found in the peripheral membrane glycopeptides. These results define further the altered fucosylation of the CF peripheral membrane glycoproteins.
authors.affiliation: Department of Pediatrics, School of Medicine, University of Pennsylvania, Philadelphia.
authors.forename: Y M
authors.initials: YM
authors.lastname: Wang
authors.forename: T R
authors.initials: TR
authors.lastname: Hare
authors.forename: B
authors.initials: B
authors.lastname: Won
authors.forename: C P
authors.initials: CP
authors.lastname: Stowell
authors.forename: T F
authors.initials: TF
authors.lastname: Scanlin
authors.forename: M C
authors.initials: MC
authors.lastname: Glick
authors.forename: K
authors.initials: K
authors.lastname: Hård
authors.forename: J A
authors.initials: JA
authors.lastname: van Kuik
authors.forename: J F
authors.initials: JF
authors.lastname: Vliegenthart
mesh_terms: D002240:Carbohydrate Sequence; D002846:Chromatography, Affinity; D003550:Cystic Fibrosis / Q000378:metabolism* / Q000473:pathology; D005347:Fibroblasts / Q000378:metabolism*; D005353:Fibronectins / Q000378:metabolism; D005643:Fucose / Q000378:metabolism*; D006020:Glycopeptides / Q000302:isolation & purification; D006801:Humans; D006882:Hydroxyapatites; D009682:Magnetic Resonance Spectroscopy / Q000379:methods; D008562:Membrane Glycoproteins / Q000302:isolation & purification / Q000378:metabolism*; D008969:Molecular Sequence Data; D009844:Oligosaccharides / Q000378:metabolism
chemical_list: D005353:Fibronectins; D006020:Glycopeptides; D006882:Hydroxyapatites; D008562:Membrane Glycoproteins; D009844:Oligosaccharides; D005643:Fucose