Isolation and characterization of major urinary oligosaccharides excreted by a patient with type 3 GM1 gangliosidosis.

Abstract

Two major oligosaccharides were isolated from the urine of a patient with type 3 GM1 gangliosidosis. From structural studies including compositional sugar analysis, fast-atom bombardment mass spectrometry, direct-inlet chemical ionization mass spectrometry, methylation analysis, chromium trioxide oxidation, and proton magnetic resonance spectroscopy, their structures were deduced to be as follows: [formula: see text] Both oligosaccharides have beta-linked galactose at the non-reducing ends. Oligosaccharide 1 is one of the most common urinary oligosaccharides found in type 1 and type 2 GM1 gangliosidosis. Oligosaccharide 2, lacto-N-difucohexaose II, has not been described in the urine of GM1 gangliosidosis patients. Excretion of oligosaccharide 1 in the type 3 patient was much less than that of a type 2 patient. Thin-layer chromatographic analysis revealed that the excretion of oligosaccharides with higher molecular weight than that of oligosaccharide 1 (octasaccharide) in the type 3 patient was much less than that of a type 2 patient, raising the possibility that the mutant beta-galactosidase of type 3 GM1 gangliosidosis can still act to some extent on higher molecular weight oligosaccharides containing beta-linked galactose at the non-reducing end.

• Authors: S Tsuji, T Ariga, S Ando, Y Tanaka, K Kon, T Yahagi, K Ohta, T Miyatake
• Journal: Journal of biochemistry (J Biochem) Vol. 109 Issue 5 Pg. 722-7 (May 1991) ISSN: 0021-924X [Print] England
• PMID: 1917896 (Publication Type: Case Reports, Journal Article)

Chemical References

• Oligosaccharides

Topics

• Carbohydrate Conformation
• Carbohydrate Sequence
• Gangliosidoses (classification, urine)
• Humans
• Magnetic Resonance Spectroscopy
• Male
• Middle Aged
• Molecular Sequence Data
• Oligosaccharides (chemistry, urine)
• Spectrometry, Mass, Fast Atom Bombardment