The importance of myelin in the regulation of axonal mitochondria

脊椎动物有髓轴突通过敬礼传导快速传播电脉冲。结旁轴-胶质连接在有髓轴突分子结构域的组织和维持中起着至关重要的作用。脑苷脂磺基转移酶（CST）缺陷小鼠显示CNS和PNS中的结旁轴神经胶质连接的破坏。我们报道，轴突线粒体在节点的郎维叶大，肿胀，轴突运输相关的蛋白水平下降，在PNS的CST缺陷小鼠。我们还发现小脑浦肯野神经元内有频繁的局灶性轴突损伤。

Vertebrate myelinated axons propagate electrical impulse rapidly by salutatory conduction. Paranodal axo-glial junctions play a crucial role in the organization and maintenance of molecular domains in myelinated axons. Cerebroside sulfotransferase(CST) deficient mice show disruption of the paranodal axo-glial junction both in the CNS and the PNS. We reported that axonal mitochondria at the nodes of Ranvier were large and swollen, and that axonal transport related protein levels decreased in the PNS of CST-deficient mice. We also found that the finding of frequent focal axonal swellings in the cerebellar Purkinje neurons.