Bratisl Lek Listy. 2022;123(9):659-671. doi: 10.4149/BLL_2022_106.
Mitochondria are intracellular organelles involved in a number of key biologic processes in the cell, including energy production, redox signaling, calcium homeostasis, inflammation, senescence, innate immune response, and mitophagy. Mitochondrial cytopathies include a heterogeneous group of diseases that are characterized by impaired oxidative phosphorylation, leading to multi-organ involvement and progressive clinical deterioration. Mitochondrial cytopathies can result from mitochondrial or nuclear DNA mutations. Mitochondrial defects play an important role in the pathogenesis of nephropathies as tubular syndromes, interstitial nephritis, focal and segmental glomerulosclerosis and diabetic nephropathy. The role of mitochondria in a pathogenesis of nephrotoxicity and kidney carcinogenesis is also discussed (Tab. 2, Fig. 7, Ref. 100). Keywords: mitochondrial nephropathy, interstitial nephritis, glomerulosclerosis, diabetic nephropathy, nephrotoxicity, mitochondrial cytopathies.
线粒体是细胞内参与许多关键生物过程的细胞器，包括能量产生、氧化还原信号、钙稳态、炎症、衰老、先天免疫反应和线粒体自噬。线粒体细胞病包括一组异质性疾病，其特征是氧化磷酸化受损，导致多器官受累和进行性临床恶化。线粒体细胞病可由线粒体或核 DNA 突变引起。线粒体缺陷在肾小管综合征、间质性肾炎、局灶节段性肾小球硬化和糖尿病肾病等肾脏病的发病机制中起着重要作用。线粒体在肾毒性和肾癌发生中的作用也在讨论中（表 2，图 7，参考文献 100）。关键词：线粒体肾病、间质性肾炎、肾小球硬化、糖尿病肾病、肾毒性、线粒体细胞病。
