Different oligosaccharides accumulate in the brain and urine of a cat with alpha-mannosidosis: structure determination of five brain-derived and seventeen urinary oligosaccharides.
Five brain-derived and 17 urinary oligomannose-type oligosaccharides were isolated by ion-exchange chromatography on Mono Q or Dowex, followed by HPLC on Lichrosorb-NH2 from a Persian cat suffering from alpha-mannosidosis. The structures of the carbohydrate chains were determined by 500- or 600-MHz 1H-NMR spectroscopy. Different oligosaccharide patterns were found in brain and urine. 99% of the urinary oligosaccharides possess an alpha(1-6)-linked mannose residue attached to beta-mannose, whereas only 5% of the brain-derived oligosaccharides contain such a residue. Furthermore, of the urinary carbohydrate chains 71% end with Man beta 1-4GlcNAc beta 1-4GlcNAc and 29% end with Man beta 1-4GlcNAc, whereas the corresponding amounts are 23% and 77%, respectively, for the brain-derived oligosaccharides.
Hård
K
K
Department of Bio-Organic Chemistry, Utrecht University, The Netherlands.
Mekking
A
A
Kamerling
J P
JP
Dacremont
G A
GA
Vliegenthart
J F
JF
D000818:Animals; D001921:Brain / Q000378:metabolism*; D002240:Carbohydrate Sequence; D002415:Cats; D005591:Chemical Fractionation; D002851:Chromatography, High Pressure Liquid; D009682:Magnetic Resonance Spectroscopy; D008969:Molecular Sequence Data; D009844:Oligosaccharides / Q000737:chemistry* / Q000378:metabolism / Q000652:urine; D011522:Protons; D008363:alpha-Mannosidosis / Q000378:metabolism* / Q000652:urine
D009844:Oligosaccharides; D011522:Protons