人脾脏中“酸性”β-葡萄糖苷酶与葡糖脑苷脂酶的同一性
Identity of 'acid' beta-glucosidase and glucocerebrosidase in human spleen.
作者信息
Ho M W
出版信息
Biochem J. 1973 Nov;136(3):721-9. doi: 10.1042/bj1360721.
Abstract
- Glucocerebrosidase, in association with a membrane-bound ;acid' beta-glucosidase, was separated from a soluble ;neutral' beta-glucosidase that had no activity towards glucocerebroside as substrate. 2. Glucocerebrosidase, as well as ;acid' beta-glucosidase activity depended upon the association of factor P (a heat-stable, soluble, acidic glycoprotein) with factor C (a heat-labile membrane-bound protein). 3. Factor C was solubilized under certain conditions. 4. Solubilized factor C, as well as membrane-bound factor C, could be alternatively stimulated by sodium taurocholate to give both glucocerebrosidase and ;acid' beta-glucosidase activities. 5. Membrane-bound factor C reacted optimally with factor P whereas solubilized factor C was preferentially stimulated by taurocholate. 6. Factor P-dependent glucocerebrosidase activity differed in kinetic properties from the taurocholate-stimulated enzyme activity. 7. The results are discussed in the light of (a) identity of glucocerebrosidase and ;acid' beta-glucosidase, (b) application in clinical diagnosis, (c) physiological significance of the enzyme system, and (d) polygenic inheritance in adult Gaucher's disease.
摘要
- 葡糖脑苷脂酶与一种膜结合的“酸性”β-葡糖苷酶相关联，它从一种对葡糖脑苷脂无底物活性的可溶性“中性”β-葡糖苷酶中分离出来。2. 葡糖脑苷脂酶以及“酸性”β-葡糖苷酶活性取决于因子P（一种热稳定的可溶性酸性糖蛋白）与因子C（一种热不稳定的膜结合蛋白）的结合。3. 因子C在某些条件下可溶解。4. 溶解的因子C以及膜结合的因子C可被牛磺胆酸钠交替刺激，以产生葡糖脑苷脂酶和“酸性”β-葡糖苷酶活性。5. 膜结合的因子C与因子P反应最佳，而溶解的因子C则优先被牛磺胆酸钠刺激。6. 因子P依赖的葡糖脑苷脂酶活性在动力学性质上与牛磺胆酸钠刺激的酶活性不同。7. 根据（a）葡糖脑苷脂酶和“酸性”β-葡糖苷酶的同一性、（b）在临床诊断中的应用、（c）酶系统的生理意义以及（d）成人戈谢病的多基因遗传来讨论这些结果。
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