Structural analysis of the major urinary oligosaccharides in feline alpha-mannosidosis.
Two homologous series of urinary oligosaccharides were identified by h.p.l.c. and fast-atom-bombardment mass spectrometry in feline alpha-mannosidosis. The predominant series has the composition Man2-8GlcNAc2 and a minor series the composition Man2-9GlcNAc. The structure of the most abundant oligosaccharide, which accounts for over 80% of the urinary oligosaccharide, was shown to be alpha-D-Manp(1----3)[alpha-D-Manp-(1----6)]beta-D-Manp -(1----4)-beta-D-GlcpNA c-(1----4)-D-GlcNAc by gas chromatography and mass spectrometry. Such a structure is consistent with the incomplete catabolism of complex N-linked glycans due to a deficiency of alpha-D-mannosidase in tissue lacking an endohexosaminidase activity.
Abraham
D
D
Daniel
P
P
Dell
A
A
Oates
J
J
Sidebotham
R
R
Winchester
B
B
D000818:Animals; D002415:Cats; D002849:Chromatography, Gas; D002850:Chromatography, Gel; D002851:Chromatography, High Pressure Liquid; D002855:Chromatography, Thin Layer; D008745:Methylation; D009844:Oligosaccharides / Q000652:urine*; D008363:alpha-Mannosidosis / Q000652:urine*
D009844:Oligosaccharides