Mitochondria is a unique signaling hub, which carries a variety of roles in addition to the main function of providing energy to the cell. Mitochondria provide ATP to cell metabolism during aerobic respiration, which is a key process for highly energy-consuming tissues, including nephron cells. Impairments in energy production in the renal epithelium immediately lead to kidney dysfunction. However, many other mitochondrial functions are critical to the organism and in particular to the kidneys. Mitochondria synthesize a number of metabolites, such as steroids, heme, and FeS-containing proteins. One of the most important functions of mitochondria is the regulation of redox balance in the cell, which violation leads to oxidative stress; many renal pathologies are associated with the increased production of reactive oxygen species. Furthermore, the participation of mitochondria in cell differentiation and dedifferentiation was shown, which is critical for the kidney, whose regenerative potential is based in particular…