Department of Veterinary Physiology, College of Veterinary Medicine, Research Institute for Veterinary Science, BK21 Four Future Veterinary Medicine Leading Education & Research Center, Seoul National University, Seoul 08826, Republic of Korea.
Department of Veterinary Physiology, College of Veterinary Medicine, Research Institute for Veterinary Science, BK21 Four Future Veterinary Medicine Leading Education & Research Center, Seoul National University, Seoul 08826, Republic of Korea.
Neurobiol Dis. 2021 May;152:105301. doi: 10.1016/j.nbd.2021.105301. Epub 2021 Feb 17.
Neurons are particularly vulnerable to mitochondrial dysfunction due to high energy demand and an inability to proliferate. Therefore, dysfunctional mitochondria cause various neuropathologies. Mitochondrial damage induces maintenance pathways to repair or eliminate damaged organelles. This mitochondrial quality control (MQC) system maintains appropriate morphology, localization, and removal/replacement of mitochondria to sustain brain homeostasis and counter progression of neurological disorders. Glucocorticoid release is an essential response to stressors for adaptation; however, it often culminates in maladaptation if neurons are exposed to chronic and severe stress. Long-term exposure to high levels of glucocorticoids induces mitochondrial dysfunction via genomic and nongenomic mechanisms. Glucocorticoids induce abnormal mitochondrial morphology and dysregulate fusion and fission. Moreover, mitochondrial trafficking is arrested by glucocorticoids and dysfunctional mitochondria are subsequently accumulated around the soma. These alterations lead to energy deficiency, particularly for synaptic transmission that requires large amounts of energy. Glucocorticoids also impair mitochondrial clearance by preventing mitophagy of damaged organelle and suppress mitochondrial biogenesis, resulting in the reduced number of healthy mitochondria. Failure to maintain MQC degrades brain function and contributes to neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease, and Huntington's disease. However, mechanisms of glucocorticoid action on the regulation of MQC during chronic stress conditions are not well understood. The present review discusses pathways involved in the impairment of MQC and the clinical significance of high glucocorticoid blood levels for neurodegenerative diseases.
神经元由于高能量需求和不能增殖而特别容易受到线粒体功能障碍的影响。因此,功能失调的线粒体导致各种神经病理学。线粒体损伤诱导维持途径来修复或消除受损的细胞器。这个线粒体质量控制(MQC)系统维持适当的形态、定位和线粒体的去除/替换,以维持大脑的内稳态并阻止神经退行性疾病的进展。糖皮质激素的释放是对适应压力的必要反应;然而,如果神经元暴露于慢性和严重的应激下,它通常会导致适应不良。长期暴露于高水平的糖皮质激素会通过基因组和非基因组机制诱导线粒体功能障碍。糖皮质激素诱导异常的线粒体形态并扰乱融合和裂变。此外,线粒体运输被糖皮质激素阻断,随后功能失调的线粒体在胞体周围积累。这些改变导致能量缺乏,特别是对于需要大量能量的突触传递。糖皮质激素还通过阻止受损细胞器的自噬和抑制线粒体生物发生来损害线粒体清除,导致健康线粒体数量减少。未能维持 MQC 会降低大脑功能,并导致神经退行性疾病,包括阿尔茨海默病、帕金森病和亨廷顿病。然而,在慢性应激条件下,糖皮质激素对 MQC 调节的作用机制尚不清楚。本综述讨论了 MQC 受损的途径以及高糖皮质激素血水平对神经退行性疾病的临床意义。