Mandal Amrita, Drerup Catherine M
Unit on Neuronal Cell Biology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, United States.
Front Cell Neurosci. 2019 Aug 9;13:373. doi: 10.3389/fncel.2019.00373. eCollection 2019.
The complex and elaborate architecture of a neuron poses a great challenge to the cellular machinery which localizes proteins and organelles, such as mitochondria, to necessary locations. Proper mitochondrial localization in neurons is particularly important as this organelle provides energy and metabolites essential to form and maintain functional neural connections. Consequently, maintenance of a healthy pool of mitochondria and removal of damaged organelles are essential for neuronal homeostasis. Long distance transport of the organelle itself as well as components necessary for maintaining mitochondria in distal compartments are important for a constant supply of healthy mitochondria at the right time and place. Accordingly, many neurodegenerative diseases have been associated with mitochondrial abnormalities. Here, we review our current understanding on transport-dependent mechanisms that regulate mitochondrial replenishment. We focus on axonal transport and import of mRNAs and proteins destined for mitochondria as well as mitochondrial fusion and fission to maintain mitochondrial homeostasis in distal compartments of the neuron.
神经元复杂精细的结构对细胞机制构成了巨大挑战,这种细胞机制负责将蛋白质和细胞器(如线粒体)定位到必要位置。线粒体在神经元中的正确定位尤为重要,因为该细胞器提供形成和维持功能性神经连接所必需的能量和代谢物。因此,维持健康的线粒体库以及清除受损细胞器对于神经元的稳态至关重要。细胞器本身的长距离运输以及维持远端区室中线粒体所需的成分对于在正确的时间和地点持续供应健康的线粒体很重要。相应地,许多神经退行性疾病都与线粒体异常有关。在这里,我们综述了目前对调节线粒体补充的运输依赖性机制的理解。我们重点关注轴突运输以及运往线粒体的mRNA和蛋白质的导入,以及线粒体的融合和裂变,以维持神经元远端区室中的线粒体稳态。