BIOCHEMICAL-STUDIES ON A CASE OF FELINE MANNOSIDOSIS
BIOCHEMICAL-STUDIES ON A CASE OF FELINE MANNOSIDOSIS
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DOI：
10.1042/bj1890467
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发表时间：
1980-01-01
影响因子：
4.1
通讯作者：
BLAKEMORE, WF
中科院分区：
文献类型：
--
作者：
BURDITT, LJ;CHOTAI, K;BLAKEMORE, WF
Evidence is presented for the biochemical diagnosis of the 1st case of feline mannosidosis. A marked deficiency of acidic .alpha.-D-mannosidase (EC 3.2.1.24) in the brain, kidney and liver and excessive excretion of mannose-rich oligosaccharides in the urine were found in a kitten suffering from a nervous disorder. Residual acidic .alpha.-D-mannosidase, ranging from 2-5.5% of the normal activity, was observed in the tissues of the affected kitten. It had similar kinetic and physicochemical properties to the normal activity. The amount of mannose in the urine of the affected kitten was 19-fold greater than in a comparable control, and the molar ratio of mannose to N-acetylglucosamine was .apprx. 6:1. High concentrations of neutral oligosaccharides were detected in the urine. The predominant oligosaccharide appeared to be a hexasaccharide. The biochemical features of bovine, feline and human mannosidosis are compared. Feline mannosidosis may be a useful animal model for studying the human disease.
