Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, S10 2HQ, UK.
Sheffield Institute for Translational Neuroscience (SITraN), Department of Neuroscience, University of Sheffield, Sheffield, S10 2HQ, UK.
Neurosci Lett. 2019 Sep 25;710:132933. doi: 10.1016/j.neulet.2017.06.052. Epub 2017 Jun 30.
Mitochondria are unique organelles that are essential for a variety of cellular processes including energy metabolism, calcium homeostasis, lipid biosynthesis, and apoptosis. Mitochondrial dysfunction is a prevalent feature of many neurodegenerative diseases including motor neuron disorders such as amyotrophic lateral sclerosis (ALS). Disruption of mitochondrial structure, dynamics, bioenergetics and calcium buffering has been extensively reported in ALS patients and model systems and has been suggested to be directly involved in disease pathogenesis. Here we review the alterations in mitochondrial parameters in ALS and examine the common pathways to dysfunction.
线粒体是一种独特的细胞器，对多种细胞过程至关重要，包括能量代谢、钙稳态、脂质生物合成和细胞凋亡。线粒体功能障碍是许多神经退行性疾病的一个普遍特征，包括运动神经元疾病，如肌萎缩侧索硬化症（ALS）。在 ALS 患者和模型系统中广泛报道了线粒体结构、动力学、生物能量学和钙缓冲的破坏，并被认为直接参与疾病的发病机制。在这里，我们回顾了 ALS 中线粒体参数的变化，并研究了导致功能障碍的常见途径。
