Haroon Suraiya, Vermulst Marc
Division of Human Genetics, Children's Hospital of Philadelphia, Philadelphia, PA, United States.
Leonard Davis School of Gerontology, University of Southern California, Los Angeles, California, United States.
Bio Protoc. 2019 Jul 5;9(13):e3288. doi: 10.21769/BioProtoc.3288.
Mitochondria generate 90% of the energy required to sustain life. As a result, loss of mitochondrial function compromises almost every facet of human physiology. Accordingly, most mitochondrial diseases tend to present themselves as complex, multi-systemic disorders that can be difficult to diagnose. Depending on the severity of the mitochondrial dysfunction, the pathology can range from mild discomfort to severe epilepsy, blindness and paralysis. To develop therapies to these diseases, it will be important to optimize experimental techniques that can reliably quantify mitochondrial function, particularly in live cells or intact organisms. Here, we describe how a Seahorse XF24 Analyzer can be used to measure both basal and maximal respiration in the nematode , and how this data can be interpreted to evaluate mitochondrial function.
线粒体产生维持生命所需能量的90%。因此，线粒体功能丧失几乎会损害人体生理的各个方面。相应地，大多数线粒体疾病往往表现为复杂的多系统疾病，难以诊断。根据线粒体功能障碍的严重程度，病理表现可从轻微不适到严重癫痫、失明和瘫痪。要开发针对这些疾病的疗法，优化能够可靠量化线粒体功能的实验技术非常重要，尤其是在活细胞或完整生物体中。在这里，我们描述了如何使用海马XF24分析仪来测量线虫的基础呼吸和最大呼吸，以及如何解释这些数据以评估线粒体功能。
