变异型囊性纤维化肝脏α-L-岩藻糖苷酶的纯化与特性分析

Purification and characterization of altered cystic fibrosis liver alpha-L-fucosidase.

作者信息

Alhadeff J A, Watkins P, Freeze H

出版信息

Clin Genet. 1978 May;13(5):417-24. doi: 10.1111/j.1399-0004.1978.tb04141.x.

DOI:10.1111/j.1399-0004.1978.tb04141.x

Abstract

alpha-L-Fucosidase (E.C.3.2.4.51) from two cystic fibrosis livers has been purified and characterized. Purification was accomplished by an affinity chromatographic procedure previously used for normal liver alpha-L-fucosidase. Characterization of the two cystic fibrosis alpha-L-fucosidases indicated that they were very similar to normal liver alpha-L-fucosidase with regard to pH optima profiles, Michaelis constants (Km's), subunit structure and antigenicity. However, gas liquid chromatographic analysis revealed altered carbohydrate compositions for both the cystic fibrosis alpha-L-fucosidases. The three major sugars found in normal purified liver alpha-L-fucosidase (mannose, N-acetylglucosamine and sialic acid) were reduced in the cystic fibrosis alpha-L-fucosidases, on average, to 51%, 44% and 32%, respectively, of their normal amounts.

摘要

已对来自两个囊性纤维化肝脏的α-L-岩藻糖苷酶（E.C.3.2.4.51）进行了纯化和特性鉴定。纯化是通过先前用于正常肝脏α-L-岩藻糖苷酶的亲和色谱法完成的。对这两种囊性纤维化α-L-岩藻糖苷酶的特性鉴定表明，它们在最适pH曲线、米氏常数（Km值）、亚基结构和抗原性方面与正常肝脏α-L-岩藻糖苷酶非常相似。然而，气相色谱分析显示，两种囊性纤维化α-L-岩藻糖苷酶的碳水化合物组成发生了变化。正常纯化肝脏α-L-岩藻糖苷酶中发现的三种主要糖类（甘露糖、N-乙酰葡糖胺和唾液酸）在囊性纤维化α-L-岩藻糖苷酶中平均分别降至其正常含量的51%、44%和32%。