Mitochondrial Nephrology

Mitochondria are intracellular organelles involved in several key biological processes in the cell, including energy production, redox signaling, homeostasis, inflammation, aging, innate immune response, and mitophagy. Mitochondrial cytopathies comprise a heterogeneous group of diseases characterized by impaired oxidative phosphorylation, leading to multiorgan involvement and progressive clinical deterioration. They are the result of mutations in mitochondrial or nuclear DNA. It is true that a disease caused by the mother (there is also information about the possibility of damage by the father) accompanied by an early onset of damage, the organ of which, with the involvement of other organs, must lead to a suspected diagnosis of mitochondrial diseases. Mitofunctional defects play an important role in the pathogenesis of diseases such as tubular diseases, interstitial nephritis, focal and segmental glomerulosclerosis, and diabetic nephritis. In addition, participation in the pathogenesis of nephrotoxicity and carcinogenesis. In mitochondrial nephrology, the focus is on improving diagnostics and subsequently influencing therapeutic procedures in patients with primary as well as secondary mitochondrial nephropathies, respectively a disorder based on mitochondrial dysfunction. The perspective of MITO nephropathies is targeted therapy of damaged MITOs including CoQ10 treatment, MITO-targeted nanocarriers, molecular hydrogen, platelet-rich plasma, Nrf2 activators, and MITO transplantation.