OLIGODENDROGLIA: MYELINATION AND DEMYELINATION

在中枢神经系统中，少突胶质细胞精心制作了大量的 形成多层髓鞘， 来增强神经冲动的跳跃式传导。 除了 少突胶质细胞负责合成这种膜， 在动物的一生中保持它。 在髓磷脂高峰期 生产，据计算，这种细胞类型可以合成更多的 超过自身重量三倍的髓鞘。 疾病中 以脱髓鞘为特征的少突胶质细胞可能是靶细胞， 其表面组分可能特别重要。 我们相信通过 研究少突胶质细胞血浆中的蛋白质和糖蛋白 膜，以及它们在正常和病变中的遗传调节 我们也许能够理解脱髓鞘的机制， 在分子水平上的过程。 我们已经确定并正在净化两个 质膜中的主要糖蛋白，通常不存在于 髓鞘，但存在于患者的受影响的髓鞘和膜中 异染性脑白质营养不良 单克隆和兔抗血清 将针对这些纯化的糖蛋白产生，用作工具， 确定糖蛋白是否是少突胶质细胞的表面标记， 以纯化大量的糖蛋白用于化学分析。 我们漫长 长期的目标是研究这两种糖蛋白的调节， 在正常状态和脱髓鞘过程中的少突胶质细胞。 在其他研究中，我们计划修改我们的差异平板法， 从新生大鼠脑中获得少突胶质细胞， 个脑袋 一旦获得小鼠少突胶质细胞， 通过免疫荧光、电子显微镜和掺入研究， 结果与大鼠少突胶质细胞和 牛脑 然后，少突胶质细胞将准备从震颤， Jimpy突变小鼠品系（异常髓鞘形成或 髓鞘化不足），以直接比较表面抗原、形态学和 正常细胞的功能。 从这些突变体中纯化的少突胶质细胞 到目前为止还没有对小鼠进行研究。 我们的长期目标是研究基因 在发育过程中和疾病中在分子水平上表达。

In the central nervous system, oligodendroglia elaborate extensive amounts of membranes to form the multilamellar myelin sheath which enfolds segments of axons to enhance saltatory conduction of nerve impulses. In addition to being responsible for the synthesis of this membrane, oligodendroglia also maintain it during the lifetime of the animal. During the peak of myelin production, it has been calculated that this cell type may synthesize more than three times its own weight in myelin membranes each day. In diseases chracterized by demyelination, oligodendroglia may be the target cell, and its surface components may be of particular importance. We believe that by studying the proteins and glycoproteins in oligodendroglial plasma membranes, and their genetic regulation in both the normal and diseased state, we may be able to understand the mechanism of the demyelinating process at the molecular level. We have identified and are purifying two major glycoproteins in plasma membranes that are not normally present in myelin, but are present in affected myelin and membranes from a patient with metachromatic leucodystrophy. Both monoclonal and rabbit antiserum will be produced against these purified glycoproteins for use as tools to determine if the glycoproteins are surface markers for oligodendroglia and to purify larger amounts of glycoproteins for chemical analysis. Our long term goals are to study the regulation of these two glycoproteins in oligodendroglia in the normal state and during demyelination. In other studies we plan to modify our differential plating method for obtaining oligodendroglia from neonatal rat brain to obtain them from mouse brain. Once mouse oligodendroglia are obtained, they will be characterized by immunofluorescence, electron microscopy, and incorporation studies, and the results compared with those oligodendroglia obtained from rat and bovine brain. Then oligodendroglia will be prepared from the Quaking and the Jimpy mutant mouse strains (models of abnormal myelin formation or of hypomyelination) to directly compare surface antigens, morphology, and function with normal cells. Purified oligodendroglia from these mutant mice have not been studied thus far. Our long term goals are to study gene expression at the molecular level during development and in disease.