MTERF4 regulates translation by targeting the methyltransferase NSUN4 to the mammalian mitochondrial ribosome

  • Cell Metab. 2011 May 4;13(5):527-39. doi: 10.1016/j.cmet.2011.04.002.
Yolanda Cámara  1 ,  Jorge Asin-Cayuela ,  Chan Bae Park ,  Metodi D Metodiev ,  Yonghong Shi ,  Benedetta Ruzzenente ,  Christian Kukat ,  Bianca Habermann ,  Rolf Wibom ,  Kjell Hultenby ,  Thomas Franz ,  Hediye Erdjument-Bromage ,  Paul Tempst ,  B Martin Hallberg ,  Claes M Gustafsson ,  Nils-Göran Larsson
Affiliations
  • 1. Department of Laboratory Medicine, Karolinska Institutet, Stockholm, Sweden.
PMID: 21531335 DOI: 10.1016/j.cmet.2011.04.002
Abstract

Precise control of mitochondrial DNA gene expression is critical for regulation of Oxidative Phosphorylation capacity in mammals. The MTERF protein family plays a key role in this process, and its members have been implicated in regulation of transcription initiation and site-specific transcription termination. We now demonstrate that a member of this family, MTERF4, directly controls mitochondrial ribosomal biogenesis and translation. MTERF4 forms a stoichiometric complex with the ribosomal RNA methyltransferase NSUN4 and is necessary for recruitment of this factor to the large ribosomal subunit. Loss of MTERF4 leads to defective ribosomal assembly and a drastic reduction in translation. Our results thus show that MTERF4 is an important regulator of translation in mammalian mitochondria.