重症肌无力（MG）是世界公认难治疾病，以往研究主要集中在抑制免疫反应和神经肌肉接头，而对需要线粒体供能才能收缩的肌细胞损伤病理机制研究较少。课题组既往发现MG患者肌细胞线粒体数目减少，超微结构受损，经健脾益气中药治疗后损害得到修复，提示中医药疗效可能与改善神经肌肉接头和肌细胞收缩能量供应有关。而线粒体是氧化磷酸化供能场所，调节其生物合成不同信号通路中PGC-1α极有可能处于核心位置。因此，本课题在既往获广东省科技进步一等奖基础上，采用复制动物EAMG模型，观察健脾益气法对MG大鼠骨骼肌线粒体能量代谢（线粒体呼吸功能、活性氧生成速率、膜电位、ATP合成酶活性）、生物合成重要调节因子及其调控信号通路（PGC-1α、NRF-1、Tfam、p38MAPK、AMPK）、线粒体融合与分裂基因（Mfn1、Mfn2、OPA1、Drp1、Fis1）mRNA及蛋白表达影响，为阐明健脾益气方药作用机制提供依据。

Myasthenia gravis is recognized as refractory disease in the world. However, previous research mainly focused on suppressing the immune reaction and the neuromuscsular junction, the study of pathomechanism of muscle cellular injury which needs energy supported by mitochondria is short. Our research group found that MG patients have phenomenon with reduced number of muscle mitochondria and ultra-structure injury, the injury has been repaired after administration of prescription of strengthening spleen and invigorating Qi,which suggests that its effects maybe related to the improving energy supply of the neuromuscsular junction and muscle cell. Mitochondria are cell powered sites, PGC-1αplays the main role in different signaling pathways which regulates mitochondrial biosynthesis.Based on research which was awarded first prize of Guangdong province science and technology progress award, research group uses duplicate model of EAMG, to observe the effect of strengthening spleen and invigorating Qi therapy on MG rats muscle mitochondria energy metabolism(Mitochondrial respiratory function, generation rate of reactive oxygen, membrane potential, ATP synthase activity ),the important regulation factor of biosynthesis, regulating and controlling signaling pathway(PGC-1α、NRF-1、Tfam、p38MAPK、AMPK), mitochondrial fusion and fission gene(Mfn1、Mfn2、OPA1、Drp1、Fis1) mRNA and protein expression, the research provides scientific evidence for illuminating effect mechanism of strengthening spleen and invigorating Qi medicine.