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Wredenberg, A.* ; Lagouge, M.* ; Bratic, A.* ; Metodiev, M.D.* ; Spåhr, H.* ; Mourier, A.* ; Freyer, C.* ; Ruzzenente, B.* ; Tain, L.* ; Grönke, S.* ; Baggio, F.* ; Kukat, C.* ; Kremmer, E. ; Wibom, R.* ; Polosa, P.L.* ; Habermann, B.* ; Partridge, L.* ; Park, C.B.* ; Larsson, N.G.*

MTERF3 regulates mitochondrial ribosome biogenesis in invertebrates and mammals.

PLoS Genet. 9:e1003178 (2013)
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Regulation of mitochondrial DNA (mtDNA) expression is critical for the control of oxidative phosphorylation in response to physiological demand, and this regulation is often impaired in disease and aging. We have previously shown that mitochondrial transcription termination factor 3 (MTERF3) is a key regulator that represses mtDNA transcription in the mouse, but its molecular mode of action has remained elusive. Based on the hypothesis that key regulatory mechanisms for mtDNA expression are conserved in metazoans, we analyzed Mterf3 knockout and knockdown flies. We demonstrate here that decreased expression of MTERF3 not only leads to activation of mtDNA transcription, but also impairs assembly of the large mitochondrial ribosomal subunit. This novel function of MTERF3 in mitochondrial ribosomal biogenesis is conserved in the mouse, thus we identify a novel and unexpected role for MTERF3 in coordinating the crosstalk between transcription and translation for the regulation of mammalian mtDNA gene expression.
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Publication type Article: Journal article
Document type Scientific Article
Language english
Publication Year 2013
HGF-reported in Year 2013
ISSN (print) / ISBN 1553-7390
e-ISSN 1553-7404
Journal PLoS Genetics
Quellenangaben Volume: 9, Issue: 1, Pages: , Article Number: e1003178 Supplement: ,
Publisher Public Library of Science (PLoS)
Reviewing status Peer reviewed
Institute(s) Institute of Molecular Immunology (IMI)
POF-Topic(s) 30504 - Mechanisms of Genetic and Environmental Influences on Health and Disease
Research field(s) Immune Response and Infection
PSP Element(s) G-501793-001
PubMed ID 23300484
DOI 10.1371/journal.pgen.1003178
Scopus ID 84873489199
Erfassungsdatum 2013-08-01
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