Abraham D, Daniel P, Dell A, Oates J, Sidebotham R, Winchester B
Biochem J. 1986 Feb 1;233(3):899-904. doi: 10.1042/bj2330899.
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.
通过高效液相色谱法（h.p.l.c.）和快原子轰击质谱法，在猫α-甘露糖苷贮积症中鉴定出两个同源的尿寡糖系列。主要系列的组成为Man2 - 8GlcNAc2，次要系列的组成为Man2 - 9GlcNAc。通过气相色谱法和质谱法表明，占尿寡糖80%以上的最丰富寡糖的结构为α-D-甘露糖基(1→3)[α-D-甘露糖基-(1→6)]β-D-甘露糖基-(1→4)-β-D-葡糖胺基-(1→4)-D-葡糖胺。这种结构与由于缺乏内切己糖胺酶活性的组织中α-D-甘露糖苷酶缺乏导致的复杂N-连接聚糖不完全分解代谢一致。
