Mitochondrial Function and Therapies

Dear Colleagues,

The activity of the mitochondrial respiratory chain (MRC), which is the major site of energy gener-ation within the cell, is regulated by various mechanisms that ensure energy demands are met. These include chan-ges in gene expression, protein translation, protein complex formation, transmembrane transport, enzymat-ic activities, and metabolite levels. These adaptations promote mitochondrial biogenesis and membrane dynamics, supporting MRC activity. In addition to primary genetic causes in both mito-chondrial DNA (mtDNA) and nuclear DNA which affect 1 in 5000 people, MRC dysfunction can also occur as a secondary consequence of disease pathophysiology, as well as drug toxicity. MRC dysfunction may result from either free-radical-induced oxidative damage or direct impair-ment of the enzyme complexes by the toxic metabolites which accumulate during certain diseas-es. Treatment of MRC dysfunction is extremely challenging and, for all intents and purposes, still in its infancy, with no cure as yet in sight. However, certain MRC disorders such as primary CoQ10 deficiencies can be responsive to specific therapies. Treatment can also involve support-ive therapy aimed at enhancing mitochondrial function as well as ameliorating increased reactive oxygen species (ROS) generation, which can be associated with aberrant MRC function. A number of new therapeutic strategies have been developed to enhance either mitochondrial bi-ogenesis or cellular antioxidant status, in addition to bypassing the defective block in the MRC and correcting disease-causing mutations. Furthermore, mitochondrial transfer, the transfer of mitochondria from one cell to another, has recently emerged as a potential therapeutic approach for restoring mitochondrial function in diseased cells. 

The focus of this Special Issue will be to highlight new information on the factors involved in regulating MRC activity and to discuss new treatment strategies that target and are able to ame-liorate mitochondrial dysfunction.

Dr. Iain P. Hargreaves
Guest Editor

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