MTERF4 Regulates Translation by Targeting the Methyltransferase NSUN4 to the Mammalian Mitochondrial Ribosome

作者:Camara Yolanda; Asin Cayuela Jorge; Park Chan Bae; Metodiev Metodi B; Shi Yonghong; Ruzzenente Benedetta; Kukat Christian; Habermann Bianca; Wibom Rolf; Hultenby Kjell; Franz Thomas; Erdjument Bromage Hediye; Tempst Paul; Hallberg B Martin; Gustafsson Claes M*; Larsson Nils Goran
来源:Cell Metabolism, 2011, 13(5): 527-539.
DOI:10.1016/j.cmet.2011.04.002

摘要

Precise control of mitochondria! 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.