title: Glycan microheterogeneity of alpha 1-antitrypsin in serum and meconium from normal and cystic fibrosis patients by crossed immunoaffinoelectrophoresis with different lectins (Con A, LCA, WGA).
abstract: In order to test whether abnormalities of glycosylation occur in cystic fibrosis (CF), the glycan microheterogeneity of alpha 1-antitrypsin (alpha 1-AT) was studied in serum and meconium from normal individuals and patients with cystic fibrosis, by crossed immunoaffinoelectrophoresis (CIAE) using free Concanavalin A (Con A), Lens culinaris lectin (LCA) and wheat germ agglutinin (WGA). Three main results emerged from this study: (1) modification of glycosylation in serum alpha 1-AT from patients with cystic fibrosis were only significant with free Con A and WGA; this probably results from a reduced synthesis of the bi-antennary side-chains or by their increased catabolism. (2) Differences in isoforms found in alpha 1-AT from normal individuals and patients with CF using free Con A, LCA, were more pronounced in the meconium than in the serum; this may provide a useful test in diagnosis of cystic fibrosis. (3) There was parallelism between the behaviour of alpha 1-AT in serum and meconium from patients with CF using LCA, Con A; this may be explained by different types or levels of disfunction affecting a glycosylation mechanism.
authors.affiliation: Biochemistry Laboratory, Centre Hospitalier Lyon-Sud, Pierre-Bénite, France.
authors.forename: S
authors.initials: S
authors.lastname: Duthel
authors.forename: A
authors.initials: A
authors.lastname: Revol
mesh_terms: D000328:Adult; D003208:Concanavalin A; D003550:Cystic Fibrosis / Q000378:metabolism*; D006031:Glycosylation; D006801:Humans; D007123:Immunoelectrophoresis, Two-Dimensional / Q000379:methods*; D007231:Infant, Newborn; D037102:Lectins; D008470:Meconium / Q000737:chemistry*; D037121:Plant Lectins; D011134:Polysaccharides / Q000032:analysis / Q000378:metabolism*; D014909:Wheat Germ Agglutinins; D000515:alpha 1-Antitrypsin / Q000032:analysis / Q000378:metabolism*
chemical_list: D037102:Lectins; D037121:Plant Lectins; D011134:Polysaccharides; D014909:Wheat Germ Agglutinins; D000515:alpha 1-Antitrypsin; C033619:lentil lectin; D003208:Concanavalin A