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Free keywords:
Animals
Blotting, Northern
Cardiomyopathies
Carrier Proteins/genetics/*metabolism
DNA, Mitochondrial/genetics
*Gene Expression Regulation, Developmental
Immunoprecipitation
Integrases/metabolism
Methyltransferases
Mice
Mice, Knockout
Mitochondria/*genetics/*metabolism
Mitochondrial Diseases/genetics/pathology
Oxidative Phosphorylation
*Protein Biosynthesis
Protein Methyltransferases/*metabolism
RNA, Ribosomal/genetics
Ribosomal Proteins/*physiology
Ribosomes/*metabolism
Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization
Transcription Factors/genetics
Transcription, Genetic
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.
