Mitochondrial cardiology

Abstract

The mitochondria belong to the functionally and metabolically most important subcellular organelles, playing a complex multifactorial role in the cell. They are involved in final oxidation of main energy sources with formation of reduced coenzymes, which deliver their electrons to the electron transport chain of the mitochondrial inner membrane. The process of electron transport is realized by some enzymes and other carriers and is associated with the formation of ATP (oxidative phosphorylation pathway). They are also involved in further processes of the cell-they produce the greatest portion of reactive oxygen species, take part in apoptosis and affect many other important cellular functions. A defect of mitochondrial structure and function is the cause of many diseases or disturbances in the whole human organism. Various functional failures due to individual mitochondrial defects are known. Many are genetically dependent or acquired in connection with different pathological events. Genetically dependent defects are often demonstrated in several organs or tissues. The most afflicted organs are those with very extensive energy metabolism-brain, skeletal muscle and heart. Therefore, encephalopathies and myopathies are very often associated with cardiomyopathies, especially in genetic-dependent mitochondrial defects. Acquired mitochondrial defects concern ischemia-reperfusion injury and other events associated with ischemia or anoxia. In this review some of these mitochondrial defects, especially those associated with cardiomyopathy are discussed. © 2008 Springer Netherlands.