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Drosophila melanogaster LRPPRC2 is involved in coordination of mitochondrial translation
Members of the pentatricopeptide repeat domain (PPR) protein family bind RNA and are important for post-transcriptional control of organelle gene expression in unicellular eukaryotes, metazoans and plants. They also have a role in human pathology, as mutations in the leucine-rich PPR-containing (LRP...
Autores principales: | , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4267620/ https://www.ncbi.nlm.nih.gov/pubmed/25428350 http://dx.doi.org/10.1093/nar/gku1132 |
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author | Baggio, Francesca Bratic, Ana Mourier, Arnaud Kauppila, Timo E.S. Tain, Luke S. Kukat, Christian Habermann, Bianca Partridge, Linda Larsson, Nils-Göran |
author_facet | Baggio, Francesca Bratic, Ana Mourier, Arnaud Kauppila, Timo E.S. Tain, Luke S. Kukat, Christian Habermann, Bianca Partridge, Linda Larsson, Nils-Göran |
author_sort | Baggio, Francesca |
collection | PubMed |
description | Members of the pentatricopeptide repeat domain (PPR) protein family bind RNA and are important for post-transcriptional control of organelle gene expression in unicellular eukaryotes, metazoans and plants. They also have a role in human pathology, as mutations in the leucine-rich PPR-containing (LRPPRC) gene cause severe neurodegeneration. We have previously shown that the mammalian LRPPRC protein and its Drosophila melanogaster homolog DmLRPPRC1 (also known as bicoid stability factor) are necessary for mitochondrial translation by controlling stability and polyadenylation of mRNAs. We here report characterization of DmLRPPRC2, a second fruit fly homolog of LRPPRC, and show that it has a predominant mitochondrial localization and interacts with a stem-loop interacting RNA binding protein (DmSLIRP2). Ubiquitous downregulation of DmLrpprc2 expression causes respiratory chain dysfunction, developmental delay and shortened lifespan. Unexpectedly, decreased DmLRPPRC2 expression does not globally affect steady-state levels or polyadenylation of mitochondrial transcripts. However, some mitochondrial transcripts abnormally associate with the mitochondrial ribosomes and some products are dramatically overproduced and other ones decreased, which, in turn, results in severe deficiency of respiratory chain complexes. The function of DmLRPPRC2 thus seems to be to ensure that mitochondrial transcripts are presented to the mitochondrial ribosomes in an orderly fashion to avoid poorly coordinated translation. |
format | Online Article Text |
id | pubmed-4267620 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | Oxford University Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-42676202014-12-23 Drosophila melanogaster LRPPRC2 is involved in coordination of mitochondrial translation Baggio, Francesca Bratic, Ana Mourier, Arnaud Kauppila, Timo E.S. Tain, Luke S. Kukat, Christian Habermann, Bianca Partridge, Linda Larsson, Nils-Göran Nucleic Acids Res RNA Members of the pentatricopeptide repeat domain (PPR) protein family bind RNA and are important for post-transcriptional control of organelle gene expression in unicellular eukaryotes, metazoans and plants. They also have a role in human pathology, as mutations in the leucine-rich PPR-containing (LRPPRC) gene cause severe neurodegeneration. We have previously shown that the mammalian LRPPRC protein and its Drosophila melanogaster homolog DmLRPPRC1 (also known as bicoid stability factor) are necessary for mitochondrial translation by controlling stability and polyadenylation of mRNAs. We here report characterization of DmLRPPRC2, a second fruit fly homolog of LRPPRC, and show that it has a predominant mitochondrial localization and interacts with a stem-loop interacting RNA binding protein (DmSLIRP2). Ubiquitous downregulation of DmLrpprc2 expression causes respiratory chain dysfunction, developmental delay and shortened lifespan. Unexpectedly, decreased DmLRPPRC2 expression does not globally affect steady-state levels or polyadenylation of mitochondrial transcripts. However, some mitochondrial transcripts abnormally associate with the mitochondrial ribosomes and some products are dramatically overproduced and other ones decreased, which, in turn, results in severe deficiency of respiratory chain complexes. The function of DmLRPPRC2 thus seems to be to ensure that mitochondrial transcripts are presented to the mitochondrial ribosomes in an orderly fashion to avoid poorly coordinated translation. Oxford University Press 2014-12-16 2014-11-26 /pmc/articles/PMC4267620/ /pubmed/25428350 http://dx.doi.org/10.1093/nar/gku1132 Text en © The Author(s) 2014. Published by Oxford University Press on behalf of Nucleic Acids Research. http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com |
spellingShingle | RNA Baggio, Francesca Bratic, Ana Mourier, Arnaud Kauppila, Timo E.S. Tain, Luke S. Kukat, Christian Habermann, Bianca Partridge, Linda Larsson, Nils-Göran Drosophila melanogaster LRPPRC2 is involved in coordination of mitochondrial translation |
title |
Drosophila melanogaster LRPPRC2 is involved in coordination of mitochondrial translation |
title_full |
Drosophila melanogaster LRPPRC2 is involved in coordination of mitochondrial translation |
title_fullStr |
Drosophila melanogaster LRPPRC2 is involved in coordination of mitochondrial translation |
title_full_unstemmed |
Drosophila melanogaster LRPPRC2 is involved in coordination of mitochondrial translation |
title_short |
Drosophila melanogaster LRPPRC2 is involved in coordination of mitochondrial translation |
title_sort | drosophila melanogaster lrpprc2 is involved in coordination of mitochondrial translation |
topic | RNA |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4267620/ https://www.ncbi.nlm.nih.gov/pubmed/25428350 http://dx.doi.org/10.1093/nar/gku1132 |
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