Cell Biology

Energy Makers

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Science  10 Jul 2009:
Vol. 325, Issue 5937, pp. 127
DOI: 10.1126/science.325_127b

Mitochondria are the powerplants of most eukaryotic cells; they generate ATP by oxidative phosphorylation, a process mediated by membrane-bound protein complexes. Some of the subunits of these complexes are encoded in the mitochondrial genome, along with a set of transfer RNA (tRNA) genes. The protein mTERF1 is a mitochondrial transcription termination factor, and a mutation in a mitochondrial tRNA (Leu) gene reduces mTERF1 binding to mitochondrial DNA (mtDNA), leading to encephalomyopathy. Wenz et al. show that mTERF2 regulates oxidative phosphorylation by modulating mitochondrial transcription. Knocking out mTERF2 in mice produced memory mistakes and muscle weakness; these two tissues rely on mitochondrial ATP. Cells from these mice expressed lower levels of oxidative phosphorylation–related proteins and also exhibited decreased mitochondrial transcription. mTERF2 binds to mtDNA as well as other mTERF proteins, suggesting a direct role for it in transcriptional regulation.

Cell Metab. 9, 499 (2009).

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