戈谢病的动物和细胞模型。I. 葡萄糖脑苷脂硫代类似物对酶促水解的抗性。

Animal and cellular models of Gaucher's disease. I. Refractoriness of thio analogs of glucocerebroside to enzymatic hydrolysis.

作者信息

Quirk J M, Weis A L, Shapiro D, Brady R O

出版信息

Biotechnol Appl Biochem. 1986 Feb;8(1):96-100.

Abstract

In order to develop a nonmetabolizable analog of glucocerebroside to investigate the distribution and accumulation of this lipid in model systems, thiohemiacetal derivatives were synthesized and their susceptibility to enzymatic hydrolysis by purified human placental glucocerebrosidase was examined. Sulfur analogs were found to be completely refractory to the activity of this enzyme, indicating their potential use in animal and isolated cell models and possibly for the preparation of affinity chromatography columns for the isolation of glucocerebrosidase.

摘要

为了开发一种不可代谢的葡糖脑苷脂类似物，以研究这种脂质在模型系统中的分布和积累，合成了硫代半缩醛衍生物，并检测了它们对纯化的人胎盘葡糖脑苷脂酶的酶促水解敏感性。发现硫类似物对该酶的活性完全不敏感，这表明它们在动物和分离细胞模型中具有潜在用途，并且可能用于制备用于分离葡糖脑苷脂酶的亲和层析柱。