Fig. 1

Overview of the MD in the clinical and pharmacological contexts. The patients with MD are characterized by involvement of different organs and tissues which results on various symptoms and their combinations. Of the most typical clinical signs are neurological, sensory and muscular symptoms. All these symptoms are caused by defective work of mitochondrial respiration as a result of mutations in nDNA or mtDNA. Classical pharmacological methods to compensate inadequate functioning of mitochondria rely on reinforcement of mitochondrial metabolic cascades and reduce of toxic agents such as lactate and ROS. For instance, thiamine, lipoic acid and dichloracetate are shown to activate pyruvate dehydrogenase resulting in decrease of lactate accumulation due to turning pyruvate into another metabolite acetyl-CoA. Succinate, riboflavin, and CoQ10 promote ETC donating electrons or restoring the function of complexes I and II. Some compounds such as idebenone, N-acetylcysteine and lipoic acid have the ability to reduce ROS production or inactivate them