Mannosidosis: Isolation and Comparison of Mannose‐Containing Oligosaccharides from Gingiva and Urine

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Abstract
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 μmol/g), (Man)3GlcNAc (3.5 μmol/g), (Man)4GlcNAc (2.8 μmol/g) and higher oligomers (Man)5GlcNAc–(Man)8GlcNAc (0.5 μmol/g); (Man, mannose; GlcNAc, N‐acetylglucosamine). Eight characteristic oligosaccltarides were isolated from the patient's urine by thin‐layer chromatography. The most abundant was (Man)2GlcNAc (161–558 μmol/l); decreasing amounts of higher homologues up to a dekasaccharide. (Man)9GlcNAC (1–4 μmol/1) were also present. In contrast to urine, in which the trisaccharide was predominant, tetrasaccharides and pentasaccharides were more abundant in gingiva.