title: Identification of urinary oligosaccharides by matrix-assisted laser desorption ionization time-of-flight mass spectrometry.
abstract: A new method of urinary oligosaccharides identification by matrix-assisted laser desorption time-of-flight mass spectrometry is presented. The method involves three steps: coupling of the urinary oligosaccharides with 8-aminonaphthalene-1,3,6-trisulfonic acid; fast purification over a porous graphite carbon extraction column; and mass spectrometric analysis. Identification of urinary oligosaccharides is based on the patterns and values of the pseudomolecular ions observed. We report here the patterns in urines from patients with Pompe disease, alpha and beta mannosidoses, galacto-sialidosis, and GM1 gangliosidosis. The protocols described here allowed facile and sensitive identification of the pathognomonic oligosacchariduria present in lysosomal diseases and can be extended to any pathological oligosacchariduria.
authors.affiliation: 1 Centre Hospitalier Régional Universitaire de Lille, Laboratoire de Biochimie et de Biologie Moléculaire, Hôpital Calmette, Bd du Professeur Jules Leclercq, 59047 Lille Cedex, France. klein@lille.inserm.fr
authors.forename: A
authors.initials: A
authors.lastname: Klein
authors.forename: A
authors.initials: A
authors.lastname: Lebreton
authors.forename: J
authors.initials: J
authors.lastname: Lemoine
authors.forename: J M
authors.initials: JM
authors.lastname: Périni
authors.forename: P
authors.initials: P
authors.lastname: Roussel
authors.forename: J C
authors.initials: JC
authors.lastname: Michalski
mesh_terms: D000328:Adult; D002240:Carbohydrate Sequence; D004591:Electrophoresis, Polyacrylamide Gel; D005456:Fluorescent Dyes; D016537:Gangliosidosis, GM1 / Q000652:urine; D006009:Glycogen Storage Disease Type II / Q000652:urine; D006801:Humans; D007231:Infant, Newborn; D008969:Molecular Sequence Data; D009281:Naphthalenes; D009844:Oligosaccharides / Q000652:urine*; D019032:Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization; D008363:alpha-Mannosidosis / Q000652:urine
chemical_list: D005456:Fluorescent Dyes; D009281:Naphthalenes; D009844:Oligosaccharides; C046465:8-amino-1,3,6-naphthalenetrisulfonic acid