Identification of urinary oligosaccharides by matrix-assisted laser desorption ionization time-of-flight mass spectrometry

A new method of urinary oligosaccharides identification by matrix-assisted laser desorption time-of-flight mass spectrometry is presented. The method involves three steps: coupling of the urinary oligosaccharides with 8-amino naphthalene-1,3,6-trisulfonic acid; fast purification over a porous graphit e carbon extraction column; and mass spectrometric analysis. Identification of urinary oligosaccharides is based on the patterns and values of the pse udomolecular ions observed. We report here the patterns in urines from pati ents with Pompe disease, alpha and beta mannosidoses, galacto-sialidosis, a nd GM1 gangliosidosis. The protocols described here allowed facile and sens itive identification of the pathognomonic oligosacchariduria present in lys osomal diseases and can be extended to any pathological oligosacchariduria.