Hirani S, Winchester B G, Patrick A D
Clin Chim Acta. 1977 Dec 1;81(2):135-44. doi: 10.1016/0009-8981(77)90005-5.
A differential assay based on their difference in thermal stability has been used to measure the acidic and true intermediate alpha-mannosidases in the plasma of cntrols and individuals homozygous or heterozygous for mannosidosis. The intermediate activity was found to be independent of age, sex or mannosidosis genotype. The acidic alpha-mannosidase did not vary significantly with age or between sexes for groups of the same age. The concentrations of acidic and intermediate alpha-mannosidase showed a positive correlation for adults but not for children. The ratio of acidic to true intermediate alpha-mannosidase might therefore be a useful secondary test for the detection of adult heterozygotes for mannosidosis.
基于热稳定性差异的鉴别分析已被用于测量对照组以及甘露糖苷贮积症纯合子或杂合子个体血浆中的酸性和真正的中间α-甘露糖苷酶。发现中间活性与年龄、性别或甘露糖苷贮积症基因型无关。对于同一年龄组,酸性α-甘露糖苷酶在年龄或性别之间没有显著差异。酸性和中间α-甘露糖苷酶的浓度在成年人中呈正相关,但在儿童中并非如此。因此,酸性与真正的中间α-甘露糖苷酶的比值可能是检测成年甘露糖苷贮积症杂合子的有用辅助检测方法。