MOLECULAR PATHOBIOLOGY OF MYELINATION

这项关于髓鞘形成的研究探讨了这样一种假设， 髓鞘的细胞内加工和装配在震颤中是有缺陷的， 变种人 本论文是通过确定短期利率来展开的 主要蛋白质和脂质在中枢神经系统中的生物合成 突变体及其掺入髓鞘和其他细胞的速率 亚细胞膜 我们会继续详细研究 这种突变体检查细胞内蛋白质通过的步骤， 髓鞘膜的组装过程。 主要的重点将是 研究髓鞘蛋白的分布和细胞内加工， 两种类型：内在的（蛋白脂质蛋白[PLP]和髓鞘相关的 糖蛋白[MAG]）和外周（四种低分子量髓鞘 碱性蛋白[MBP]）。 髓鞘蛋白的细胞内加工 在少突胶质细胞中进行翻译后修饰 内膜将在震颤的CNS中进行检查（以及它们在 PNS中的施万细胞），并参考寡糖加工， 酰化反应。

This research on myelinogenesis explores the hypothesis that the intracellular processing and assembly of myelin is defective in the quaking mutant. This thesis has been developed by determining the short-term rates of biosynthesis of the major proteins and lipids in the CNS of quaking mutant and the rates of their incorporation into myelin and other subcellular membranes. We shall proceed with further detailed studies of this mutant examining the steps of intracellular protein passage in the process of assemply of the myelin membrane. The main thrust will be to examine the distribution and intracellular processing of myelin protein of two types: intrinsic (the proteolipid protein [PLP] and myelin-associated glycoprotein [MAG]) and peripheral (the four low molecular weight myelin basic proteins [MBPs]). The intracellular processing of myelin proteins subject to post-translational modification in oligodendroglial endomembranes will be examined in quaking CNS (and their analogues in the Schwann cell in PNS) with reference to oligosaccharide processing and acylation.