Mannosidosis: isolation and comparison of mannose-containing oligosaccharides from gingiva and urine.
Excessive gingival hyperplasia with storage of mannose-rich oligosaccharides appears to be a unique feature present in a 31-year-old mannosidosis patient. Fractionation and analysis of the gingiva established the presence of (Man)2GlcNAc (2.2 mumol/g), (Man)3GlcNAc (3.5 mumol/g), (Man)4GlcNAc (2.8 mumol/g) and higher oligomers (Man)5GlcNAc--(Man)8GlcNAc (0.5 mumol/g); (Man, mannose; GlcNAc, N-acetylglucosamine). Eight characteristic oligosaccharides were isolated from the patient's urine by thin-layer chromatography. The most abundant was (Man)2GlcNAc (161--558 mumol/l); decreasing amounts of higher homologues up to a dekasaccharide, (Man)9GlcNAc (1--4 mumol/l) were also present. In contrast to urine, in which the trisaccharide was predominant, tetrasaccharides and pentasaccharides were more abundant in gingiva.
D000117:Acetylglucosamine / Q000032:analysis; D000328:Adult; D002239:Carbohydrate Metabolism, Inborn Errors / Q000378:metabolism*; D002855:Chromatography, Thin Layer; D005881:Gingiva / Q000032:analysis*; D006801:Humans; D008297:Male; D008358:Mannose / Q000032:analysis*; D008361:Mannosidases / Q000172:deficiency*; D009844:Oligosaccharides / Q000302:isolation & purification* / Q000652:urine
D009844:Oligosaccharides; D008361:Mannosidases; D008358:Mannose; D000117:Acetylglucosamine