α-L-岩藻糖苷酶在囊性纤维化中的异常分布：血清中活性降低。

Abnormal distribution of alpha-L-fucosidase in cystic fibrosis: decreased activity in serum.

作者信息

Scanlin T F, Matacic S S, Glick M C

出版信息

Clin Chim Acta. 1979 Jan 15;91(2):197-202. doi: 10.1016/0009-8981(79)90457-1.

Abstract

The activity of alpha-L-fucosidase is decreased in the serum of cystic fibrosis patients when compared to age-matched controls. This result, combined with the elevated activity in skin fibroblasts, supports the concept of an abnormal intracellular and extracellular distribution of alpha-L-fucosidase in cystic fibrosis.

摘要

与年龄匹配的对照组相比，囊性纤维化患者血清中的α-L-岩藻糖苷酶活性降低。这一结果，再加上皮肤成纤维细胞中活性升高，支持了囊性纤维化中α-L-岩藻糖苷酶在细胞内和细胞外分布异常的概念。