Datta S C, Radin N S
Lipids. 1986 Nov;21(11):702-9. doi: 10.1007/BF02537244.
The concentration of beta-glucosidase-stimulating proteins (called cohydrolase here) was measured in mouse liver and brain by immunoassay. Factors that might influence the levels of cohydrolase were examined. Injecting mice with an inactivator of glucosidase (conduritol B epoxide) rapidly produced elevations in liver glucosylceramide (the enzyme's substrate) and in liver and brain cohydrolase. Injection of glucosylceramide emulsified with Myrj 52 produced the same two effects in liver but not in brain. The increases in cohydrolase level induced by the enzyme inhibitor persisted in both organs for at least seven days, reaching 61-70% above the normal level. Injection of emulsified galactocerebroside, sphingomyelin and mixed glucosphingolipids but not of ceramide also produced rises in cohydrolase level. An increase in cohydrolase level resulted from injection of phenylhydrazine, which produces hemolysis and consequently an increased workload for the glucosidase of liver. When the enzyme inhibitor and/or larger amounts of glucosylceramide emulsion were injected (750 mg/kg body weight), increases in liver weight of 13 to 37% appeared within one day. The increased weight was characterized by increases in the weights of protein, total lipid and DNA and a very high increase in glucosylceramide level. These procedures have produced a rapidly developing model version of Gaucher disease in mice. Injected glucocerebroside also induced an elevated level of glucosidase activity.
通过免疫测定法测定了小鼠肝脏和大脑中β-葡萄糖苷酶刺激蛋白（此处称为共水解酶）的浓度。研究了可能影响共水解酶水平的因素。给小鼠注射葡萄糖苷酶的灭活剂（conduritol B环氧化物）后，肝脏葡萄糖神经酰胺（该酶的底物）以及肝脏和大脑中的共水解酶迅速升高。注射用Myrj 52乳化的葡萄糖神经酰胺在肝脏中产生了相同的两种效果，但在大脑中没有。酶抑制剂诱导的共水解酶水平升高在两个器官中至少持续七天，比正常水平高出61-70%。注射乳化的半乳糖脑苷脂、鞘磷脂和混合糖鞘脂而非神经酰胺也会导致共水解酶水平升高。注射苯肼会导致共水解酶水平升高，苯肼会引起溶血，从而增加肝脏葡萄糖苷酶的工作量。当注射酶抑制剂和/或大量葡萄糖神经酰胺乳剂（750 mg/kg体重）时，一天内肝脏重量增加13%至37%。体重增加的特征是蛋白质、总脂质和DNA的重量增加，以及葡萄糖神经酰胺水平的极高增加。这些程序在小鼠中产生了一种快速发展的戈谢病模型版本。注射的葡萄糖脑苷脂也诱导了葡萄糖苷酶活性水平的升高。
