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Cá,mara, Yolanda,Asin-Cayuela, Jorge,Park, Chan ,Bae,Metodiev, Metodi ,D.,Shi, Yonghong,Ruzzenente, Benedetta,Kukat, Christian,Habermann, Bianca,Wibom, Rolf,Hultenby, Kjell,Franz, Thomas Elsevier 2011 Cell metabolism Vol.13 No.5
<P><B>Summary</B></P><P>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.</P> <P><B>Highlights</B></P><P>► Loss of MTERF4 leads to abolished mitochondrial translation ► MTERF4 forms a complex with the rRNA methyltransferase NSUN4 ► MTERF4 targets NSUN4 to the mitochondrial large ribosomal subunit</P>