[Diseases caused by mitochondrial dysfunction].

The morphological description of mitochondria has been known for nearly two hundred years. Mitochondria are found in all human cells, and a thorough understanding of their function in healthy and pathological conditions was a slow progress. In recent decades, the understanding of this extensive network has accelerated, both in the theoretical field and in clinical practice. Our aim was to review the biogenesis of mitochondria and the diseases caused by their dysfunction, based on the current state of the art. We review the literature to describe the major areas of mitochondrial function, such as ATP production, which is crucial for the energy supply of the body, and the importance of the pyruvate and citric acid cycles, the electron transport chain, oxidative phosphorylation and ROS formation. The function of mitohormesis, which contributes to the body's defences, is described. After a description of mitochondrial dysfunction, we turn to the characterisation of the different mitochondrial pathologies. Having discussed the congenital mitochondrial pathologies, we highlight the fibrosis that severely impairs the function of certain parenchymal organs, heart, liver, kidneys, lungs. We emphasize the importance of cardiac fibrosis, in particular cardiac arrhythmias associated with mitochondrial dysfunction, briefly mentioning the latest therapeutic recommendations. In this context, results are expected from the use of SGLT2 or combined SGLT1/2 inhibitor. The role of this system in type 1 diabetes mellitus and in the development of insulin resistance and type 2 diabetes is mentioned as well. We outline the role of mitochondrial dysfunction in the development of nerodegenerative diseases. The importance of exercise, antioxidant therapy, cardiolipin protection, enhancement of mitochondrial biogenesis, use of sodium-glucose co-transporter inhibitors, and - underlined - the recently introduced hopeful mitochondrial transplantation in the management of these pathologies are highlighted. The mitochondrial system is not only an energy centre but also regulates the function of all our vital organs. We have shown that in the case of mitochondrial dysfunction, the function of our vital organs becomes critical due to the fibrosis that develops. However, we do not address the oncological aspects of mitochondria and mitochondrial dysfunction, as this is beyond the scope of this article. The aim of this work is primarily to explore the multiple interrelationships of this system, to deepen our knowledge and to use it for the better care of our patients. Some believe that mitochondria will determine the future of medicine.