常染色体显性遗传性多囊肾病中的能量代谢改变

Energy metabolism changes in autosomal dominant polycystic kidney disease

常染色体显性遗传性多囊肾病(autosomal dominant polycystic kidney disease,ADPKD)发生代谢重编程,有氧糖酵解明显上调,为囊肿衬里上皮细胞的增殖提供充足的能量及生产底物。多囊蛋白能通过直接和间接的方式调节线粒体功能,功能蛋白的丢失使线粒体形态、功能发生改变,存在明显的氧化应激。本文对ADPKD的能量代谢改变特征、线粒体功能障碍的特征及发生机制进行综述和讨论,并总结干预ADPKD能量代谢的潜在临床靶点。

In autosomal dominant polycystic kidney disease(ADPKD),metabolic reprogramming occurs and aerobic glycolysis is significantly upregulated,providing sufficient energy and substrates for proliferation.Polycystins can regulate mitochondrial function directly and indirectly.The loss of functional proteins changes mitochondrial morphology and function,resulting in significant oxidative stress.Here,we reviewed and discussed the characteristics and mechanisms of energy metabolic changes and mitochondrial dysfunction in ADPKD.We also summarized the potential clinical targets for the intervention of energy metabolism in ADPKD.