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Cell Metab. 2014 Aug 5;20(2):226-40. doi: 10.1016/j.cmet.2014.07.001. Epub 2014 Jul 31.

Making proteins in the powerhouse.

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Department of Cell and Molecular Biology, Karolinska Institutet, 171 77 Stockholm, Sweden; Röntgen-Ångström-Cluster, Karolinska Institutet Outstation, Centre for Structural Systems Biology, DESY Campus, 22603 Hamburg, Germany; European Molecular Biology Laboratory, Hamburg Unit, 22603 Hamburg, Germany. Electronic address:
Department of Mitochondrial Biology, Max Planck Institute for Biology of Ageing, 509 31 Cologne, Germany; Department of Laboratory Medicine, Karolinska Institutet, 171 77 Stockholm, Sweden. Electronic address:


Understanding regulation of mitochondrial DNA (mtDNA) expression is of considerable interest given that mitochondrial dysfunction is important in human pathology and aging. Similar to the situation in bacteria, there is no compartmentalization between transcription and translation in mitochondria; hence, both processes are likely to have a direct molecular crosstalk. Accumulating evidence suggests that there are important mechanisms for regulation of mammalian mtDNA expression at the posttranscriptional level. Regulation of mRNA maturation, mRNA stability, translational coordination, ribosomal biogenesis, and translation itself all form the basis for controlling oxidative phosphorylation capacity. Consequently, a wide variety of inherited human mitochondrial diseases are caused by mutations of nuclear genes regulating various aspects of mitochondrial translation. Furthermore, mutations of mtDNA, associated with human disease and aging, often affect tRNA genes critical for mitochondrial translation. Recent advances in molecular understanding of mitochondrial translation regulation will most likely provide novel avenues for modulating mitochondrial function for treating human disease.

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