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Age-Linked Changes in the Genotype and Phenotype of Mitochondria

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Frontiers of Cellular Bioenergetics

Abstract

Photophosphorylation and oxidative phosphorylation (OXPHOS) are the main energy-generating systems of higher organisms. During the evolution of eukaryotic cells, the energy (ATP)-producing enzyme complexes related to the production of oxygen—photosynthetic phosphorylation in chloroplasts—as well as to the consumption of oxygen—OXPHOS in mitochondria—became encoded partly by nuclear DNA (nDNA) and partly by extranuclear DNA, the chloroplast DNA and the mitochondrial DNA (mtDNA), respectively. Mammalian mitochondrial DNA (mtDNA) is a molecule of about 16,000 nucleotide pairs (np). It codes for two rRNAs, 22 tRNAs, and 13 proteins, which represent exclusively subunits of the four proton-pumping enzyme complexes of OXPHOS: seven subunits of NADH-dehydrogenase (complex I), one of cytochrome c reductase (complex III), three of cytochrome c oxidase (complex IV), and two of ATP synthase (complex V) (Wallace et al., 1995). Since all eukaryotic cells synthesize ATP also by glycolysis, the enzyme complexes involved in OXPHOS, and thus the extrachromosomal genome (mtDNA), are not essential for the survival of individual cells. In fact, animal cells lacking mtDNA but still containing mitochondria (rho0 cells) (King and Attardi, 1989) or containing pathogenetic mutations of mtDNA (Chomyn et al., 1991) can be kept in culture indefinitely. The life of an animal and the proper function of its organs, however, are inevitably dependent on mitochondrial OXPHOS.

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Authors and Affiliations

  1. Department of Biochemistry and Molecular Biology, University of Bari, 70125, Bari, Italy

    Maria N. Gadaleta, Angela M. S. Lezza & Palmiro Cantatore

  2. Fachbereich Chemie, Philipps-Universität, D-35032, Marburg, Germany

    Bernhard Kadenbach & Annette Reith

  3. Institute of Medical Biochemistry and Chemistry, University of Bari, I-70124, Bari, Italy

    Domenico Boffoli & Sergio Papa

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  1. Maria N. Gadaleta
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  4. Annette Reith
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  6. Domenico Boffoli
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  7. Sergio Papa
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  1. University of Bari, Bari, Italy

    Sergio Papa  & Ferruccio Guerrieri  & 

  2. University of Amsterdam, Amsterdam, The Netherlands

    Joseph M. Tager

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Gadaleta, M.N. et al. (1999). Age-Linked Changes in the Genotype and Phenotype of Mitochondria. In: Papa, S., Guerrieri, F., Tager, J.M. (eds) Frontiers of Cellular Bioenergetics. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-4843-0_28

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