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Mannosidosis: Isolation and Comparison of Mannose‐Containing Oligosaccharides from Gingiva and Urine
Author(s) -
DANIEL Peter F.,
DEFEUDIS Donna F.,
LOTT Ira T.
Publication year - 1981
Publication title -
european journal of biochemistry
Language(s) - English
Resource type - Journals
eISSN - 1432-1033
pISSN - 0014-2956
DOI - 10.1111/j.1432-1033.1981.tb05141.x
Subject(s) - mannose , urine , oligosaccharide , fractionation , trisaccharide , chemistry , biochemistry , chromatography
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) 2 GlcNAc (2.2 μmol/g), (Man) 3 GlcNAc (3.5 μmol/g), (Man) 4 GlcNAc (2.8 μmol/g) and higher oligomers (Man) 5 GlcNAc–(Man) 8 GlcNAc (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) 2 GlcNAc (161–558 μmol/l); decreasing amounts of higher homologues up to a dekasaccharide. (Man) 9 GlcNAC (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.

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