[Chemistry of urinary mannosides excreted in mannosidosis].
Mannose-rich oligosaccharides have been isolated from urines of 5 patients with mannosidosis. Their compositon and structure were determined. Three of them have been previously described by Norden et al: alpha p-Manp-(1 leads to 3) beta-d-Manp-(1 leads to 4) d-GlcNAcp; alpha-p-Manp-(1 leads to 2), alpha-d-Manp-(1 leads to 3) beta-d-Manp-(1 leads to 4) d-GlcNAc and alpha-d-Manp-(1 leads to 2) alpha-d-Manp-(1 leads to 2) alpha-d-Manp-(1 leads to 3) beta-d-Manp-(1 leads to 4) d-GlcNAcp, but the four others are new entities: alpha-d-Manp-(1 leads to 3) (alpha-d-Manp-(1 leads to 2) alpha-d-Manp-(1 leads to 2) alpha-d-Manp-(1 leads to 6) beta-d-Manp-(1 leads to 4) GlcNAcp; alpha-d-Manp-(1 leads to 2) alpha-d-Manp-(1 leads to 3) (alpha-d-Manp-(1 leads to 2) alpha-d-Manp-(1 leads to 6) beta-d-Manp-(1 leads to 4) GlcNAcp; alpha-d-Manp-(1 leads to 2), alpha-d-Man-(1 leads to 3) (alpha-d-Manp-(1 leads to 6) beta-d-Manp-(1 leads to 4) GlcNAp and alpha-d-Manp-(1 leads to 2) alpha-d-Manp-(1 leads to 3) (alpha-d-Manp-(1 leads to 6) beta-d-Manp-(1 leads to 4) GlcNAcp. These structures are related to the glycans of "oligomannosidic type" present in numerous glycoproteins. All possess a N-acetylglucosamine residue in terminal reducing position and reinforce the hypothesis of Kobata et al. and Montreuil et al. that catabolism of glycans N-glucosidically linked to the protein moiety begins by the aciton of a beta-endo-N-acetylglucosaminidase.
D000117:Acetylglucosamine / Q000032:analysis; D002239:Carbohydrate Metabolism, Inborn Errors / Q000652:urine*; D002849:Chromatography, Gas; D002854:Chromatography, Paper; D005643:Fucose / Q000032:analysis; D006023:Glycoproteins / Q000652:urine; D006027:Glycosides / Q000652:urine*; D006801:Humans; D008358:Mannose / Q000032:analysis; D008362:Mannosides / Q000652:urine*; D009844:Oligosaccharides / Q000032:analysis
D006023:Glycoproteins; D006027:Glycosides; D008362:Mannosides; D009844:Oligosaccharides; D005643:Fucose; D008358:Mannose; D000117:Acetylglucosamine
Etude chimique des 'mannosides urinaires excrétés au cours de la mannosidose