Journal article

Determination of oligosaccharides in Pompe disease by electrospray ionization tandem mass spectrometry

T Rozaklis, SL Ramsay, PD Whitfield, E Ranieri, JJ Hopwood, PJ Meikle

Clinical Chemistry | AMER ASSOC CLINICAL CHEMISTRY | Published : 2002

Abstract

BACKGROUND: The development of therapies for lysosomal storage disorders has created a need for biochemical markers to monitor the efficacy of therapy and methods to quantify these markers in biologic samples. In Pompe disease, the concentration of a tetrasaccharide, consisting of four glucose residues, is reputedly increased in urine and plasma, but faster and more sensitive methods are required for the analysis of this, and other oligosaccharides, from biologic fluids. METHODS: We optimized the derivatization of storage oligosaccharides with 1-phenyl-3-methyl-5-pyrazolone for the measurement, by electrospray ionization tandem mass spectrometry, of oligosaccharide concentrations in urine (n..

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