School of Biomedical Sciences, The University of Queensland, St. Lucia, QLD, Australia.
University of Queensland Centre for Clinical Research, The University of Queensland, Herston, QLD, Australia.
FASEB J. 2020 Jun;34(6):7225-7233. doi: 10.1096/fj.201901781. Epub 2020 Apr 19.
Amyotrophic lateral sclerosis (ALS) is a fatal and rapidly progressing motor neuron disease without effective treatment. Although the precise mechanisms leading to ALS are yet to be determined, there is now increasing evidence implicating the defective energy metabolism and components of the innate immune complement system in the onset and progression of its motor phenotypes. This review will survey the mechanisms by which the energy metabolism and the complement system are altered during the disease progression of ALS and how it can contribute to disease. Furthermore, it will also examine how complement activation can modify the energy metabolism in metabolic disorders, in order to highlight how the complement system and energy metabolism may be linked in ALS.
肌萎缩侧索硬化症（ALS）是一种致命的、快速进展的运动神经元疾病，目前尚无有效治疗方法。虽然导致 ALS 的精确机制尚未确定，但越来越多的证据表明，能量代谢缺陷和先天免疫补体系统成分与运动表型的发生和进展有关。本综述将调查在 ALS 疾病进展过程中能量代谢和补体系统改变的机制，以及它如何导致疾病。此外，还将研究补体激活如何改变代谢紊乱中的能量代谢，以强调补体系统和能量代谢如何在 ALS 中相关联。
