成髓鞘细胞研究和应用进展教学教材演示幻灯片.pptx

成髓鞘细胞研究和应用进展安逸生活 pk 艰苦卓绝创业高原如此艰苦的环境：勇于担当，发挥引领作用以苦为乐，人生需要有一股精神特朗普：我拒绝政治正确， 我只做正确的事情精英政治2016.10.11. 中英脱髓鞘伦敦会议要点一些基本概念与影像学检查儿童脱髓鞘疾病临床分类成髓鞘细胞分类神经修复的路径各种细胞近期研究一览Year 1977~ 2016 40年 demyelination remyelination Pubmed文献量趋势图髓鞘构成成分CNS 髓鞘中60 ~ 70% 为水分固体成分中脂类占70%, 蛋白质占30%构成髓鞘蛋白质主要两种成分：蛋白脂蛋白( PLP) 和髓鞘碱性蛋白(MBP)髓鞘相关糖蛋白(MAG) 髓鞘少突胶质细胞糖蛋白(MOG)Wolfram 蛋白脂DM 准大小蛋白其他微量蛋白髓鞘功能提供轴突与周围组织电绝缘，避免干扰相邻轴突之间轴突与其他结构之间加快动作电位传递：“跳跃式传导”机制引导受损轴突：再生中枢神经系统(CNS)有髓神经纤维与周围(PNS)相似结构有髓鞘和郎飞结髓鞘外无基膜（神经膜）相邻神经纤维有时融合（箭头）儿童发育髓鞘化过程主要集中在：出生后18个月内（1.5年）1岁半接近成人oligodendrocyte少突胶质细胞：大多数细胞5-10年成熟，存活50年。每年减少1-300 OLSThe final stage of oligodendrocyte development is myelination. Unlike the peripheral nervous system where there is a strict size-dependent bias for myelination (only axons 1 um diameter enjoy the privilege), in the CNS axons as small as 300 nm are sometimes myelinated. Nevertheless, CNS myelination is also influenced by axonal size. Surprisingly, cultured oligodendrocytes will begin myelinating even synthetic axon-like tubes lacking the usual neuron-glia signaling cues. In this type of ‘blind’ myelination, only tubes with a diameter of ≥ 400 nm were myelinated, suggesting this mode of myelination may be particularly important for larger axons.Moreover, once differentiating, it appears that oligodendrocytes only have a very narrow window of opportunity to select which adjacent axons to myelinate (~5 h in the developing zebrafish and ~12 h in myelinating cultures of rodent derived cells), irrespective of the total number of sheaths being made. This implies that the signaling pathways mediating this process are likely to be relatively robust. Although in vitro studies have implicated several key pathways, to date no one single molecule has been shown to be indispensable for myelination of CNS axons in vivo, highlighting the great degree of redundancy in the control of this vital process.Despite this, recent in vivo studies have revealed a great deal about how these various signaling pathways converge to control