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

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...

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Autores principales: Bruggink, Cees, Poorthuis, Ben J. H. M., Deelder, André M., Wuhrer, Manfred
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer-Verlag 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3354319/
https://www.ncbi.nlm.nih.gov/pubmed/22526647
http://dx.doi.org/10.1007/s00216-012-5968-9
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author Bruggink, Cees
Poorthuis, Ben J. H. M.
Deelder, André M.
Wuhrer, Manfred
author_facet Bruggink, Cees
Poorthuis, Ben J. H. M.
Deelder, André M.
Wuhrer, Manfred
author_sort Bruggink, Cees
collection PubMed
description 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 Our analytical approach allowed structural analysis of the excreted oligosaccharides and revealed several previously unpublished oligosaccharides.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. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1007/s00216-012-5968-9) contains supplementary material, which is available to authorized users.
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spelling pubmed-33543192012-05-31 Analysis of urinary oligosaccharides in lysosomal storage disorders by capillary high-performance anion-exchange chromatography–mass spectrometry Bruggink, Cees Poorthuis, Ben J. H. M. Deelder, André M. Wuhrer, Manfred Anal Bioanal Chem Original Paper 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. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1007/s00216-012-5968-9) contains supplementary material, which is available to authorized users. Springer-Verlag 2012-04-20 2012 /pmc/articles/PMC3354319/ /pubmed/22526647 http://dx.doi.org/10.1007/s00216-012-5968-9 Text en © The Author(s) 2012 https://creativecommons.org/licenses/by/4.0/This article is distributed under the terms of the Creative Commons Attribution License which permits any use, distribution and reproduction in any medium, provided the original author(s) and source are credited.
spellingShingle Original Paper
Bruggink, Cees
Poorthuis, Ben J. H. M.
Deelder, André M.
Wuhrer, Manfred
Analysis of urinary oligosaccharides in lysosomal storage disorders by capillary high-performance anion-exchange chromatography–mass spectrometry
title Analysis of urinary oligosaccharides in lysosomal storage disorders by capillary high-performance anion-exchange chromatography–mass spectrometry
title_full Analysis of urinary oligosaccharides in lysosomal storage disorders by capillary high-performance anion-exchange chromatography–mass spectrometry
title_fullStr Analysis of urinary oligosaccharides in lysosomal storage disorders by capillary high-performance anion-exchange chromatography–mass spectrometry
title_full_unstemmed Analysis of urinary oligosaccharides in lysosomal storage disorders by capillary high-performance anion-exchange chromatography–mass spectrometry
title_short Analysis of urinary oligosaccharides in lysosomal storage disorders by capillary high-performance anion-exchange chromatography–mass spectrometry
title_sort analysis of urinary oligosaccharides in lysosomal storage disorders by capillary high-performance anion-exchange chromatography–mass spectrometry
topic Original Paper
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3354319/
https://www.ncbi.nlm.nih.gov/pubmed/22526647
http://dx.doi.org/10.1007/s00216-012-5968-9
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