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Mitochondrial Diseases

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Developmental Neuropathology

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Abstract

It is now widely held that mitochondria originated from the endosymbiosis of a primitive bacterium. If so, significant mutations have taken place in the endosymbiont, since there are not only metabolic similarities, e. g. inhibition of protein synthesis by antibiotics, but also metabolic dissimilarities from present day prokaryontes. Mitochondria possess their own DNA. The mitochondrial chromosome M (the 25th chromosome) is circular and attached to the inner mitochondrial membrane. Its genome is about 200,000 times smaller than the haploid nuclear genome. Only one or a few molecules are present per mitochondrion. The mitochondrial genome contains codes for cytochromes (subunits of cytochrome c oxidase and for the cytochrome bc1 complex), for subunits of the ATPase complex, for mitochondrial RNA and ribosomal proteins (McKusick 1982). The genetic information for the vast majority of other mitochondrial enzymes and proteins is contained in the nuclear genome. These constituents are synthezised in the cytoplasm and transported into the mitochondrion. This includes enzymes of pyruvate utilization, the citric acid cycle or fatty acid oxidation, and others located in the matrix and/or the inner mitochondrial membrane. Even the cytochromes contain translation products of both mitochondrial and nuclear genomes (Rascati and Parsons 1979).

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  1. Zentrum Neurologische Medizin, Abteilung Neuropathologie, Georg-August-Universität Göttingen, Robert-Koch-Straße 40, 3400, Göttingen, Germany

    Reinhard L. Friede

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© 1989 Springer-Verlag Berlin Heidelberg

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Friede, R.L. (1989). Mitochondrial Diseases. In: Developmental Neuropathology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-73697-1_39

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  • DOI: https://doi.org/10.1007/978-3-642-73697-1_39

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