The importance of myelin in the regulation of axonal mitochondria

脊椎动物髓鞘轴突通过有益传导快速传播电脉冲。副神经节轴胶质连接在髓系轴突分子结构域的组织和维持中起着至关重要的作用。脑苷硫转移酶（CST）缺陷小鼠在中枢神经系统和PNS中均表现出神经节旁轴胶质连接的破坏。我们报道了cst缺陷小鼠PNS的Ranvier淋巴结轴突线粒体大而肿胀，轴突运输相关蛋白水平下降。我们还发现在小脑浦肯野神经元中发现频繁的局灶性轴突肿胀。

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.