Analysis of urinary oligosaccharides in lysosomal storage disorders by capillary high-performance anion-exchange chromatography-mass spectrometry.

Abstract

Many lysosomal storage diseases are characterized by an increased urinary excretion of glycoconjugates and oligosaccharides that are characteristic for the underlying enzymatic defect. Here, we have used capillary high-performance anion-exchange chromatography (HPAEC) hyphenated to mass spectrometry to analyze free oligosaccharides from urine samples of patients suffering from the lysosomal storage disorders fucosidosis, α-mannosidosis, G(M1)-gangliosidosis, G(M2)-gangliosidosis, and sialidosis. Glycan fingerprints were registered, and the patterns of accumulated oligosaccharides were found to reflect the specific blockages of the catabolic pathway. Our analytical approach allowed structural analysis of the excreted oligosaccharides and revealed several previously unpublished oligosaccharides. In conclusion, using online coupling of HPAEC with mass spectrometric detection, our study provides characteristic urinary oligosaccharide fingerprints with diagnostic potential for lysosomal storage disorders.

• Authors: Cees Bruggink, Ben J H M Poorthuis, André M Deelder, Manfred Wuhrer
• Journal: Analytical and bioanalytical chemistry (Anal Bioanal Chem) Vol. 403 Issue 6 Pg. 1671-83 (Jun 2012) ISSN: 1618-2650 [Electronic] Germany
• PMID: 22526647 (Publication Type: Journal Article)

Chemical References

• Anion Exchange Resins
• Oligosaccharides

Topics

• Anion Exchange Resins
• Chromatography, High Pressure Liquid (methods)
• Chromatography, Ion Exchange (methods)
• Humans
• Lysosomal Storage Diseases (urine)
• Mass Spectrometry (methods)
• Oligosaccharides (urine)