Structural analysis of the major caprine beta-mannosidosis urinary oligosaccharides.
Urinary oligosaccharides were studied in beta-mannosidosis, a newly identified, inherited glycoprotein catabolic disorder associated with severe neonatal neurological deficits, widespread lysosomal storage vacuoles and a deficiency of plasma and tissue beta-mannosidase. A preliminary analysis of the oligosaccharides was obtained by gel-permeation chromatography and mass chromatography. The major urinary oligosaccharides were then isolated by gel-permeation chromatography, DEAE-Sephadex column chromatography and preparative paper chromatography, and were analyzed by carbohydrate composition analysis, methylation studies, mass spectrometry and glycosidase digestion. As a result of these studies, beta-mannosyl-(1 leads to 4)-N-acetylglucosamine and beta-mannosyl-(1 leads to 4)-beta-N-acetylglucosaminyl-(1 leads to 4)-N-acetylglucosamine were identified as the major abnormal oligosaccharides. Galactosaminyl-(alpha 1 leads to 3)-[fucosyl-(alpha 1 leads to 2)]-galactose was also found in affected goat urine, while lactose was present in the urine of both control and affected goats.
D000818:Animals; D002239:Carbohydrate Metabolism, Inborn Errors / Q000652:urine*; D002850:Chromatography, Gel; D006041:Goats; D008361:Mannosidases / Q000172:deficiency*; D013058:Mass Spectrometry; D009844:Oligosaccharides / Q000652:urine*; D044902:beta-Mannosidase
D009844:Oligosaccharides; D008361:Mannosidases; D044902:beta-Mannosidase