title: Urinary oligosaccharide excretion and severity of galactosialidosis and sialidosis.
abstract: Urinary oligosaccharides of galactosialidosis and sialidosis patients were investigated by the Bio-Gel P-4 column chromatography in order to delineate each clinical subtype biochemically. The levels of urinary oligosaccharides (nmol/mg creatinine) in late infantile-type galactosialidosis patients with early onset and severe clinical manifestations were 2.5 times the levels in juvenile/adult-type patients. Galactosyl oligosaccharides which had not been reported in the analysis of galactosialidosis urine were identified in the urine of patients of both subtypes. The structures of the urinary oligosaccharides in late infantile-type patients were the same as those in the juvenile/adult-type patients. The levels of urinary oligosaccharides in the infantile-type sialidosis patients were 3.5 times the levels in late-type patients who were less severely affected. The structures of oligosaccharides in the urine of infantile-type patients were the same as those in the urine of late-type patients, but more high-molecular weight sialyl oligosaccharides were excreted in the urine of infantile-type patients than in the urine of late-type patients. The structures of 7 different kinds of sialyl oligosaccharides that have not been previously reported were identified in the urine of both galactosialidosis and sialidosis patients. Sialidosis patients excreted relatively more sialyl oligosaccharides than did galactosialidosis patients.
authors.affiliation: Department of Pediatrics, Gifu University School of Medicine, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Takahashi
authors.forename: Y
authors.initials: Y
authors.lastname: Nakamura
authors.forename: S
authors.initials: S
authors.lastname: Yamaguchi
authors.forename: T
authors.initials: T
authors.lastname: Orii
mesh_terms: D000293:Adolescent; D000328:Adult; D002236:Carbohydrate Conformation; D002240:Carbohydrate Sequence; D002648:Child; D002675:Child, Preschool; D006801:Humans; D016464:Lysosomal Storage Diseases / Q000652:urine*; D008969:Molecular Sequence Data; D009439:Neuraminidase / Q000172:deficiency; D009844:Oligosaccharides / Q000737:chemistry / Q000652:urine*; D001616:beta-Galactosidase / Q000172:deficiency
chemical_list: D009844:Oligosaccharides; D009439:Neuraminidase; D001616:beta-Galactosidase