Perspectives of mitochondrial medicine

Biochemistry (Mosc). 2013 Sep;78(9):979-90. doi: 10.1134/S0006297913090034.

Abstract

Mitochondrial medicine was established more than 50 years ago after discovery of the very first pathology caused by impaired mitochondria. Since then, more than 100 mitochondrial pathologies have been discovered. However, the number may be significantly higher if we interpret the term "mitochondrial medicine" more widely and include in these pathologies not only those determined by the genetic apparatus of the nucleus and mitochondria, but also acquired mitochondrial defects of non-genetic nature. Now the main problems of mitochondriology arise from methodology, this being due to studies of mitochondrial activities under different models and conditions that are far from the functioning of mitochondria in a cell, organ, or organism. Controversial behavior of mitochondria ("friends and foes") to some extent might be explained by their bacterial origin with possible preservation of "egoistic" features peculiar to bacteria. Apparently, for normal mitochondrial functioning it is essential to maintain homeostasis of a number of mitochondrial elements such as mitochondrial DNA structure, membrane potential, and the system of mitochondrial quality control. Abrogation of these elements can cause a number of pathologies that have become subjects of mitochondrial medicine. Some approaches to therapy of mitochondrial pathologies are discussed.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antioxidants / therapeutic use
  • Bacteria / genetics
  • Bacteria / metabolism
  • DNA, Mitochondrial / genetics
  • Humans
  • Membrane Potential, Mitochondrial / physiology
  • Mitochondria / genetics*
  • Mitochondria / metabolism
  • Mitochondrial Diseases / drug therapy
  • Mitochondrial Diseases / genetics

Substances

  • Antioxidants
  • DNA, Mitochondrial