MOLECULAR PATHOBIOLOGY OF MYELINATION

髓鞘形成的分子病理学

• 批准号: 3394688
• 负责人: EDWARD L. HOGAN
• 金额: $ 13.93万
• 依托单位: MEDICAL UNIVERSITY OF SOUTH CAROLINA
• 依托单位国家: 美国
• 财政年份: 1976
• 资助国家: 美国
• 起止时间: 1976-05-01 至 1986-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/3394688
• 关键词: acylation; atomic absorption spectrometry; brain metabolism; electron microscopy; electrophoresis; genetic strain; genetic translation; immunochemistry; lipid biosynthesis; membrane reconstitution /synthesis; molecular pathology; mutant; myelin; myelination; myelinopathy; neuroanatomy; neurochemistry; oligodendroglia; oligosaccharides; peripheral nervous system; proteolipids; radioimmunoassay

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.

这项关于髓鞘发生的研究探索了一种假设，即 震颤时髓鞘的细胞内处理和组装存在缺陷 变种人。这篇论文是通过确定短期利率来展开的 大鼠中枢神经系统主要蛋白质和脂类的生物合成 突变体及其与髓鞘和其他物质结合的速率 亚细胞膜。我们会进一步详细研究 该突变体检测细胞内蛋白传递的步骤 髓鞘膜的组装过程。主要的推动力将是 髓鞘蛋白在细胞内的分布和加工 两种类型：固有的(蛋白脂蛋白[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[Mbps])。髓鞘蛋白的胞内加工 少突胶质细胞的翻译后修饰 内膜将在震颤的中枢神经系统中进行检查(及其类似物在 PNS中的Schwann细胞)与寡糖处理和 酰化反应。