ALS is a human neurodegenerative disorder that induces a progressive paralysis of voluntary muscles due to motor neuron loss. The causes are unknown, and there is no curative treatment available. Mitochondrial dysfunction is a hallmark of ALS pathology; however, it is currently unknown whether it is a cause or a consequence of disease progression. Recent evidence indicates that glial mitochondrial function changes to cope with energy demands and critically influences neuronal death and disease progression. Aberrant glial cells detected in the spinal cord of diseased animals are characterized by increased proliferation rate and reduced mitochondrial bioenergetics. These features can be compared with cancer cell behavior of adapting to nutrient microenvironment by altering energy metabolism, a concept known as metabolic reprogramming. We focus on data that suggest that aberrant glial cells in ALS undergo metabolic reprogramming and profound changes in glial mitochondrial activity, which are associated with motor neuron death in ALS. This review article emphasizes on the association between metabolic reprogramming and glial reactivity, bringing new paradigms from the area of cancer research into neurodegenerative diseases. Targeting glial mitochondrial function and metabolic reprogramming may result in promising therapeutic strategies for ALS.
Neuroimmunomodulation

中文翻译：

---

肌萎缩侧索硬化症中的胶质代谢重编程

ALS 是一种人类神经退行性疾病，由于运动神经元丢失导致随意肌进行性麻痹。原因不明，也没有有效的治疗方法。线粒体功能障碍是 ALS 病理学的标志；然而，目前尚不清楚它是疾病进展的原因还是结果。最近的证据表明，神经胶质线粒体功能发生变化以应对能量需求，并严重影响神经元死亡和疾病进展。在患病动物的脊髓中检测到的异常神经胶质细胞的特征是增殖率增加和线粒体生物能学降低。这些特征可以与癌细胞通过改变能量代谢来适应营养微环境的行为进行比较，这一概念被称为代谢重编程。我们关注的数据表明，ALS 中的异常神经胶质细胞经历了代谢重编程和神经胶质线粒体活动的深刻变化，这与 ALS 中的运动神经元死亡有关。这篇综述文章强调了代谢重编程和神经胶质反应性之间的关联，将癌症研究领域的新范式引入神经退行性疾病。靶向神经胶质线粒体功能和代谢重编程可能会为 ALS 带来有希望的治疗策略。将癌症研究领域的新范式引入神经退行性疾病。靶向神经胶质线粒体功能和代谢重编程可能会为 ALS 带来有希望的治疗策略。将癌症研究领域的新范式引入神经退行性疾病。靶向神经胶质线粒体功能和代谢重编程可能会为 ALS 带来有希望的治疗策略。
神经免疫调节