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https://hdl.handle.net/2440/23266
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Type: | Journal article |
Title: | Characterization of sulfated oligosaccharides in mucopolysaccharidosis type IIIA by electrospray ionization mass spectrometry |
Author: | Mason, K. Meikle, P. Hopwood, J. Fuller, M. |
Citation: | Analytical Chemistry, 2006; 78(13):4534-4542 |
Publisher: | Amer Chemical Soc |
Issue Date: | 2006 |
ISSN: | 0003-2700 1520-6882 |
Abstract: | Heparan sulfate is a linear glycosaminoglycan with considerable structural diversity that binds a myriad of growth factors and proteins that play pivotal roles in a variety of biological processes. We have investigated the structural complexity of partially degraded fragments of heparan sulfate in mucopolysaccharidosis type IIIA in which there is a defect in heparan sulfate catabolism. Mono- to hexadecasaccharides were isolated from the urine of a mucopolysaccharidosis IIIA patient and shown to have non-reducing end glucosamine N-sulfate residues, reflecting the catabolic deficiency in heparan N-sulfatase (sulfamidase) activity. The use of nitrous acid digestion (pH 1.5) combined with separation by reverse-phase high-performance liquid chromatography and analysis by electrospray ionization-mass spectrometry identified multiple forms of these oligosaccharides with some N-acetylated glucosamine residues and one to three sulfates per disaccharide. Furthermore, we demonstrated that each oligosaccharide existed in multiple sulfated forms. Many structural isomers were present, suggesting a complex mixture of oligosaccharides present in the urine as a consequence of a defect in heparan sulfate degradation. |
Keywords: | CHO Cells Animals Cricetulus Mucopolysaccharidosis III Sulfates Oligosaccharides Chromatography, High Pressure Liquid Spectrometry, Mass, Electrospray Ionization Cricetinae |
DOI: | 10.1021/ac052083d |
Published version: | http://dx.doi.org/10.1021/ac052083d |
Appears in Collections: | Aurora harvest 2 Paediatrics publications |
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