Abstract
Abstract The urine from three patients with mannosidosis was found to contain abnormally high amounts of mannose-containing oligosaccharides. The highest yield (123 to 430 mg per liter of urine) was obtained of a trisaccharide, which was isolated and characterized. The isolation procedure included ultrafiltration, gel chromatography on Sephadex G-25, purification by preparative zone electrophoresis, and preparative paper chromatography. Sugar analysis showed that the trisaccharide consisted of 2 d-mannose moieties and 1 2-acetamido-2-deoxy-d-glucose residue. The linkages and sequential order of the sugar units were established by methylation analysis of the reduced trisaccharide. The partially methylated sugars obtained on hydrolysis of the methylated trisaccharide alditol were analyzed by gas-liquid chromatography and mass spectrometry. The anomeric configurations were determined by chromium trioxide oxidation followed by methylation analysis. Additional proof for the anomeric natures of the sugar units was obtained by enzymatic hydrolysis studies and by inhibition experiments using a precipitin system with concanavalin A. As a result of these studies, the structure of the trisaccharide could be established as α-d-mannopyranoside-(1→3)-β-d-mannopyranoside-(1→4)-2-acetamido-2-deoxy-d-glucose. The oligosaccharide is most probably a degradation product derived from the inner core of glycoprotein chains.
Highlights
A Perkin-Elmer 270 gas-liquid chromatography-mass spectrometry instrument fitted with a column packed with UC W-98 on Chromosorb Q was used at 190”
Urines collected from the three patients after fasting were fractionated on a Sephadcx G-25 column (5 x 130 cm, eluted with distilled water at 4“)
Sugar analysis shon-ed that RL 0.82 consisted of 2 molts of n-mannose and 1 mole of 2-acetamido-2-deoxy-n-glucose
Summary
NORDI?,N~ .&ND ARNE LUNDBLAD From the Institute of Medical Chemistry, University of Uppsala, Uppsala, Sweden. SIGFRID SVENSSOX From the Department of Organic Chemistry, University of Stockholm, Stockholm, S’tceden. PER-ARNE ~~CKERMAN From the Department of Clinical Chemistry, University Hospital, Luwl, kceden. SEPPO AUTIO From the Department of Pediatrics, University Central Hospital, Helsindci, Finlad
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