Please use this identifier to cite or link to this item: http://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
RMID: 0020060889
DOI: 10.1021/ac052083d
Appears in Collections:Paediatrics publications

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