视神经疾病与能量代谢及轴浆转运的关系

发布时间：2018-03-02 13:09

本文选题：视神经疾病　切入点：青光眼　出处：《生物化学与生物物理进展》2016年02期 　论文类型：期刊论文

【摘要】：神经细胞的特化之一是其轴突,长度可达胞体直径的几百甚至几千倍.轴浆转运维系着胞体和轴突终末之间大量的物质交流,保证神经细胞发挥正常功能.轴浆转运障碍可以导致神经细胞功能受损直至凋亡.在一些视神经疾病中,轴浆转运功能的改变是最早出现的症状,因此也可能成为治疗的潜在靶点.在青光眼和视神经缺血的动物模型中,轴浆转运功能的下降是最早出现的变化之一.而Leber's遗传性视神经病变(LHON)和常染色体显性视神经萎缩(ADOA)是已知线粒体功能障碍引起的视神经疾病.不难想象,长距离轴浆转运功能对能量代谢尤其敏感,因此在LHON和ADOA中可能也有不同程度的下降,但似乎并没有受到足够关注.本文首先回顾了微管和马达蛋白在轴浆转运中的作用,比较分析以上所述几种疾病的发病机制、临床表现及治疗手段,试图发现它们之间的共同特点以及这些特点与能量代谢、轴浆转运之间的潜在关系,为其治疗提供新的思路.
[Abstract]:One of the specializations of nerve cells is their axons, which can be hundreds or even thousands of times the diameter of the cell body. The axon transport maintains a large amount of material communication between the cell body and the axon terminal. In some optic nerve diseases, the change of axonal transport function is the earliest symptom. So it could also be a potential target for treatment. In animal models of glaucoma and optic nerve ischemia, The decline in axonal transport is one of the earliest changes, and Leber's hereditary optic neuropathy (LHON) and autosomal dominant optic atrophy (ADOAA) are known to cause optic nerve diseases caused by mitochondrial dysfunction. Long-distance axonal transport is particularly sensitive to energy metabolism, so it may also decrease in varying degrees in LHON and ADOA, but it does not seem to receive enough attention. This paper reviews the role of microtubules and motor proteins in axonal transport. By comparing and analyzing the pathogenesis, clinical manifestations and treatment methods of the above mentioned diseases, we try to find out their common characteristics and their potential relationship with energy metabolism and axonal transport, so as to provide a new idea for the treatment of these diseases.
【作者单位】：上海交通大学生物医学工程学院;复旦大学附属眼耳鼻喉科医院;
【分类号】：R774.6

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