Activator proteins for the catabolism of glycosphingolipids.
Activator proteins for the catabolism of glycosphingolipids.
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鞘糖脂分解代谢的激活蛋白。
DOI：
10.1007/978-1-4684-1200-0_18
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发表时间：
1984
影响因子：
--
通讯作者：
Li,SC
中科院分区：
文献类型：
--
作者：
Li,YT;Li,SC
在18971年Büchner发现葡萄糖的酒精发酵可以通过酵母汁（酵母的无细胞提取物）进行之后，Harden和Young 2在1904年报告说，通过添加煮沸的过滤酵母汁，可以大大增加酵母汁对葡萄糖的发酵，尽管煮沸的酵母汁本身不能建立发酵。这是第一次证明酶促反应需要“激活剂”（辅酶）。辅酶的生物学重要性已得到充分认识，因为大多数水溶性维生素是辅酶的组分。直到最近，生物化学家才意识到鞘糖脂的水解可能需要辅因子。通过许多实验室的工作，人们已经充分认识到，鞘糖脂中糖链的水解不能单独通过酶的作用进行;水解需要激活蛋白的存在。在本章中，我们将简要概述与鞘糖脂代谢相关的激活蛋白，并以我们对AB型GM 2-神经节苷脂沉积症的化学病理学研究为例，说明激活蛋白的生物学意义
After Büchner’s discovery in 18971that the alcoholic fermentation of glucose could be carried out by yeast-juice (cell free extract of yeast), Harden and Young2reported in 1904 that the fermentation of glucose by yeast-juice was greatly increased by the addition of boiled filtered yeast-juice, although the boiled yeast-juice itself was incapable of setting up fermentation. This was the first demonstration of the requirement of an “activator” (co-enzyme) for an enzymic reaction. The biological importance of co-enzymes has been well appreciated, since most water soluble vitamins are components of co-enzymes. Until recently, it had not occurred to biochemists that hydrolysis of glycosphingolipids may require a co-factor. Through the work of many laboratories, it has been well recognized that the hydrolysis of sugar chains in glycosphingolipids cannot proceed by the action of enzymes alone; the hydrolysis requires the presence of an activator protein. In this chapter we would like to give a brief overview on activator proteins related to the catabolism of glycosphingolipids and to use our studies on chemical pathology of Type-AB GM2-gangliosidosis as an example to illustrate the biological importance of the activator proteins