线粒体动力学与视网膜神经节细胞

线粒体动力学是指细胞中的线粒体不断地分裂、融合、移动、运输和线粒体自噬等,这些动态的过程在调节线粒体的形态与功能中发挥关键作用,并对细胞的存活、代谢、功能等有重要影响.视网膜神经节细胞(RGCs)作为视网膜中一类特殊且重要的神经元,对线粒体的动力学改变特别敏感.有关常染色体显性遗传性视神经萎缩疾病的研究发现,控制线粒体融合的相关基因与RGCs功能密切相关.实验性青光眼模型提示,眼压升高引起RGCs的线粒体分裂增多,改变调节线粒体融合基因的表达,最终诱导RGCs的凋亡;线粒体在RGCs中的正常运输和分布对于RGCs轴突的功能至关重要.以上遗传性和实验性视神经病变的研究表明,线粒体动力学在调节RGCs的生存中发挥着核心作用,通过调控线粒体动力学来保护RGCs可能是一个非常有前景的治疗策略.本文将对线粒体动力学的主要内容和RGCs中的线粒体动力学进行阐述.

The role of mitochondrial dynamics in retinal ganglion cells

Mitochondrial dynamics refers to the constant fission,fusion,docking,transportation,and mitophagy of mitochondria within cells.These dynamic processes are not only critical to regulating mitochondrial morphology and function,but also playing key roles in cell survival,metabolism and function.Retinal ganglion cells (RGCs) are special and important neurons in retina and are exquisitely sensitive to disturbances in mitochondrial dynamics.Recent studies on autosomal dominant optic atrophy have demonstrated that genes which encode a mitochondrial protein involved in mitochondrial fusion are closely associated with the function of RGCs.In experimental glaucoma models,increased intraocular pressure induced mitochondrial fission,changes of optic atrophy 1 (OPAl) mRNA and protein expression,and resulted in RGCs death.The normal transportation and distribution of mitochondria in RGCs are critical for the function of axons.The studies on inherited and experimental optic neuropathies suggest that mitochondrial dynamics plays a central role in regulating the survival of RGCs.Manipulating mitochondrial dynamics has emerged as a promising strategy in RGCs protection.This paper reviewed the main content of mitochondrial dynamics and the role of mitochondrial dynamics in RGCs.