CNRS UMR 8256, Biological Adaptation and Ageing, Paris, 75005, France.
Sorbonne Universités, UPMC, Institut de Biologie Paris-Seine, Paris, 75005, France.
Sci Rep. 2016 Sep 8;6:32777. doi: 10.1038/srep32777.
In chronic neurodegenerative syndromes, neurons progressively die through a generalized retraction pattern triggering retrograde axonal degeneration toward the cell bodies, which molecular mechanisms remain elusive. Recent observations suggest that direct activation of pro-apoptotic signaling in axons triggers local degenerative events associated with early alteration of axonal mitochondrial dynamics. This raises the question of the role of mitochondrial dynamics on both axonal vulnerability stress and their implication in the spreading of damages toward unchallenged parts of the neuron. Here, using microfluidic chambers, we assessed the consequences of interfering with OPA1 and DRP1 proteins on axonal degeneration induced by local application of rotenone. We found that pharmacological inhibition of mitochondrial fission prevented axonal damage induced by rotenone, in low glucose conditions. While alteration of mitochondrial dynamics per se did not lead to spontaneous axonal degeneration, it dramatically enhanced axonal vulnerability to rotenone, which had no effect in normal glucose conditions, and promoted retrograde spreading of axonal degeneration toward the cell body. Altogether, our results suggest a mitochondrial priming effect in axons as a key process of axonal degeneration. In the context of neurodegenerative diseases, like Parkinson's and Alzheimer's, mitochondria fragmentation could hasten neuronal death and initiate spatial dispersion of locally induced degenerative events.
在慢性神经退行性综合征中,神经元通过广泛的回缩模式逐渐死亡,引发向细胞体的逆行轴突变性,但其分子机制仍不清楚。最近的观察表明,轴突中促凋亡信号的直接激活触发了与轴突线粒体动力学早期改变相关的局部退行性事件。这就提出了线粒体动力学在轴突易损性应激及其在损伤向神经元未受挑战部分传播中的作用的问题。在这里,我们使用微流控室评估了干扰 OPA1 和 DRP1 蛋白对局部应用鱼藤酮诱导的轴突变性的影响。我们发现,在低糖条件下,线粒体分裂的药理学抑制可防止鱼藤酮诱导的轴突损伤。虽然线粒体动力学的改变本身不会导致自发的轴突变性,但它大大增强了轴突对鱼藤酮的易损性,而在正常葡萄糖条件下,鱼藤酮没有影响,并促进了轴突变性向细胞体的逆行传播。总之,我们的结果表明,线粒体在轴突中的引发效应是轴突变性的一个关键过程。在帕金森病和阿尔茨海默病等神经退行性疾病的情况下,线粒体碎片化可能会加速神经元死亡并引发局部诱导的退行性事件的空间扩散。