Quantitative analysis of disaccharides in the urine ofβ-mannosidosis patients

1 March 1990

journal article
research article
Published by Wiley in Journal of Inherited Metabolic Disease

Vol. 13 (2) , 187-194
https://doi.org/10.1007/bf01799685

Abstract

Until recently, there have not been any confirmed reports ofβ-mannosidase deficiency in man. We have now analysed urine from two patients with confirmedβ-mannosidase deficiency and have found Man-β(1–4)GlcNAc concentrations of 65 and 73 mg/mmol creatinine. These levels are at least 12 times higher than those found in patients with other lysosomal storage diseases. The method we report for this analysis requires only 0.1 ml of urine and uses a gas chromatograph, area ratio, calibration curve for the quantitative analysis.

Keywords

This publication has 9 references indexed in Scilit:

Fast atom bombardment/collisional activation mass spectrometry of β-D-mannosyl-(1→4)-β-D-N-acetylglucosaminyl (1→N) urea and related compounds
Rapid Communications in Mass Spectrometry, 1988
Structure of a new hexasaccharide from the coaggregation polysaccharide of Streptococcus sanguis 34
Carbohydrate Research, 1987
Human β-Mannosidase Deficiency
New England Journal of Medicine, 1986
Determination, by methylation analysis, of the glycosyl-linkage compositions of microgram quantities of complex carbohydrates
Carbohydrate Research, 1983
Isolation and characterisation of 4‐O‐β‐D‐mannopyranosyl‐2‐acetamido‐2‐deoxy‐D‐glucose from the urine of a patient with mucolipidosis II and its occurrence in normal and pathological urine
FEBS Letters, 1982
Permethylation of polysaccharides by a modified Hakomori method
Carbohydrate Research, 1982
Quantitative metabolic profiling of urinary organic acids by gas chromatography-mass spectrometry. Comparison of isolation methods
Analytical Chemistry, 1975
Determination of aminosugar linkages in glycolipids by methylation
Archives of Biochemistry and Biophysics, 1973
[1] Study of the carbohydrates of glycoproteins
Published by Elsevier ,1972