Determination of a mannose-containing trisaccharide in the urine of patients and heterozygotes of mannosidosis

A method employing gas-liquid chromatography and combined gas-liquid chromatography mass spectrometry was developed for the quantitative determination of α-D-mannopyranoside-(1 → 3)-β-D-mannopyranoside-(1 → 4)-2-acetamido-2-deoxy-D-glucose in urines. The concentration of this trisaccharide varied between 123 and 469 mg per litre of urine in the patients with mannosidosis but was not detected in twenty normals and two heterozygotes.