MTERF3 Regulates Mitochondrial Ribosome Biogenesis in Invertebrates and Mammals

作者：Wredenberg Anna^*; Lagouge Marie; Bratic Ana; Metodiev Metodi D; Spahr Henrik; Mourier Arnaud; Freyer Christoph; Ruzzenente Benedetta; Tain Luke; Groenke Sebastian; Baggio Francesca; Kukat Christian; Kremmer Elisabeth; Wibom Rolf; Polosa Paola Loguercio; Habermann Bianca; Partridge Linda; Park Chan Bae; Larsson Nils Goran

来源：PLoS Genetics, 2013, 9(1): e1003178.

DOI：10.1371/journal.pgen.1003178

Regulation of mitochondrial DNA (mtDNA) expression is critical for the control of oxidative phosphorylation in response to physiological demand, and this regulation is often impaired in disease and aging. We have previously shown that mitochondrial transcription termination factor 3 (MTERF3) is a key regulator that represses mtDNA transcription in the mouse, but its molecular mode of action has remained elusive. Based on the hypothesis that key regulatory mechanisms for mtDNA expression are conserved in metazoans, we analyzed Mterf3 knockout and knockdown flies. We demonstrate here that decreased expression of MTERF3 not only leads to activation of mtDNA transcription, but also impairs assembly of the large mitochondrial ribosomal subunit. This novel function of MTERF3 in mitochondrial ribosomal biogenesis is conserved in the mouse, thus we identify a novel and unexpected role for MTERF3 in coordinating the crosstalk between transcription and translation for the regulation of mammalian mtDNA gene expression.

• 出版日期2013-1
• 单位河北医科大学