The accumulation of oligosaccharides in tissues and body fluids of cats with alpha-mannosidosis

被引:18
作者
Warren, Christopher D. [1 ]
Azaroff, Lenore S.
Bugge, Birgitte
Jeanloz, Roger W.
Daniel, Peter F.
Alroy, Joseph
机构
[1] Massachusetts Gen Hosp, Lab Carbohydrate Res, Boston, MA 02114 USA
[2] Eunice Kennedy Shriver Ctr Mental Retardat Inc, Waltham, MA 02254 USA
[3] Tufts Univ, Sch Med & Vet Med, Dept Pathol, Boston, MA 02111 USA
关键词
D O I
10.1016/0008-6215(88)80089-2
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Oligosaccharides were extracted from tissues and body fluids of five kittens with alpha-mannosidosis, three being from the same litter. The kittens were all of different ages at death and were compared to normal and heterozygote cats. The oligosaccharides were analyzed by high-pressure liquid chromatography after per-benzoylation and were identified by comparison with compounds of known structure. This provided a detailed picture of the distribution of oligosaccharides in each tissue, and a method for quantitation of the total oligosaccharides. With the exception of the youngest animal (death at day 2), the oligosaccharide elution profiles were broadly similar for all tissues and fluids, and were typical of feline a-mannosidosis. In contrast, concentrations of total oligosaccharides diverged widely from one source to another, from a high of 17.3 mu mol/g to a low of 0.04 mu mol/g. The results are interpreted in the context of glycoprotein catabolism.
引用
收藏
页码:325 / 338
页数:14
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