Isolation and characterization of major urinary oligosaccharides excreted by a patient with type 3 GM1 gangliosidosis.
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.
Department of Neurology, Niigata University.
S
S
Tsuji
T
T
Ariga
S
S
Ando
Y
Y
Tanaka
K
K
Kon
T
T
Yahagi
K
K
Ohta
T
T
Miyatake
D002236:Carbohydrate Conformation; D002240:Carbohydrate Sequence; D005733:Gangliosidoses / Q000145:classification / Q000652:urine*; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D008875:Middle Aged; D008969:Molecular Sequence Data; D009844:Oligosaccharides / Q000737:chemistry / Q000652:urine*; D016339:Spectrometry, Mass, Fast Atom Bombardment
D009844:Oligosaccharides