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MTERF3 is a negative regulator of mammalian mtDNA transcription.

Journal article
Authors Chan Bae Park
Jorge Asin-Cayuela
Yolanda Cámara
Yonghong Shi
Mina Pellegrini
Martina Gaspari
Rolf Wibom
Kjell Hultenby
Hediye Erdjument-Bromage
Paul Tempst
Maria Falkenberg
Claes M Gustafsson
Nils-Göran Larsson
Published in Cell
Volume 130
Issue 2
Pages 273-85
ISSN 0092-8674
Publication year 2007
Published at
Pages 273-85
Language en
Keywords Animals, DNA, Mitochondrial, genetics, Down-Regulation, genetics, Electron Transport, Embryo, Mammalian, metabolism, Embryonic Development, Gene Targeting, Genes, Essential, Hela Cells, Humans, Mice, Mice, Knockout, Mitochondria, pathology, Mitochondrial Proteins, metabolism, Myocardium, pathology, ultrastructure, Organ Specificity, Phenotype, Promoter Regions, Genetic, genetics, Protein Binding, RNA, Messenger, genetics, metabolism, Transcription Factors, metabolism, Transcription, Genetic
Subject categories Chemical Sciences


Regulation of mammalian mtDNA gene expression is critical for altering oxidative phosphorylation capacity in response to physiological demands and disease processes. The basal machinery for initiation of mtDNA transcription has been molecularly defined, but the mechanisms regulating its activity are poorly understood. In this study, we show that MTERF3 is a negative regulator of mtDNA transcription initiation. The MTERF3 gene is essential because homozygous knockout mouse embryos die in midgestation. Tissue-specific inactivation of MTERF3 in the heart causes aberrant mtDNA transcription and severe respiratory chain deficiency. MTERF3 binds the mtDNA promoter region and depletion of MTERF3 increases transcription initiation on both mtDNA strands. This increased transcription initiation leads to decreased expression of critical promoter-distal tRNA genes, which is possibly explained by transcriptional collision on the circular mtDNA molecule. To our knowledge, MTERF3 is the first example of a mitochondrial protein that acts as a specific repressor of mammalian mtDNA transcription initiation in vivo.

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