MTERF3 regulates mitochondrial ribosome biogenesis in invertebrates and mammals.
PLoS Genet
; 9(1): e1003178, 2013.
Article
in En
| MEDLINE
| ID: mdl-23300484
ABSTRACT
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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Ribosomes
/
Drosophila Proteins
/
Mitochondrial Proteins
/
Drosophila melanogaster
/
Mitochondria
Type of study:
Prognostic_studies
Limits:
Animals
Language:
En
Journal:
PLoS Genet
Journal subject:
GENETICA
Year:
2013
Type:
Article
Affiliation country:
Germany