Mitochondrial medicine

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Abstract
In the mitochondrion, inherited defects have been identified in the electron transport system by which ATP is formed, as well as in the transport and metabolism of fuels. Clinical findings in diseases due to these defects can be related to abnormal accumulations of metabolic intermediates and inadequate or inefficient ATP generation. In the oxidative process within the mitochondrion, chemical oxidants are generated, which can cause cellular damage. As the body's defences against the oxidants decline, oxidative damage appears to contribute to the ageing process itself as well as to age-related degenerative diseases. Understanding in this area has accelerated with knowledge of the synthesis, structure and function of the mitochondrion and its specific DNA. The frontier is expected to advance rapidly as causal relationships between these diseases and mitochondrial dysfunction, and the potential role of antioxidants in therapy, are better defined.