ArticleNucleic acids research2014
Drosophila melanogaster LRPPRC2 is involved in coordination of mitochondrial translation.
Article in Nucleic acids research, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed, 34 citations in OpenAlex.
- Slirp2 modulates oogenesis via regulating mitochondrial protein translation.Journal of molecular cell biology · 2026Article
- The mitochondrial mRNA-stabilizing protein SLIRP regulates skeletal muscle mitochondrial structure and respiration by exercise-recoverable mechanisms.Nature communications · 2024Article
- Candida albicans PPR proteins are required for the expression of respiratory Complex I subunits.Genetics · 2024Article
- Bendless is essential for PINK1-Park mediated Mitofusin degradation under mitochondrial stress caused by loss of LRPPRC.PLoS genetics · 2023Article
- The Role of Lonp1 on Mitochondrial Functions during Cardiovascular and Muscular Diseases.Antioxidants (Basel, Switzerland) · 2023Review
- Mitochondria dysfunction impairs Tribolium castaneum wing development during metamorphosis.Communications biology · 2022Article
- The PPR domain of mitochondrial RNA polymerase is an exoribonuclease required for mtDNA replication in Drosophila melanogaster.Nature cell biology · 2022Article
- Types and Functions of Mitoribosome-Specific Ribosomal Proteins across Eukaryotes.International journal of molecular sciences · 2022Review
- An exon junction complex-independent function of Barentsz in neuromuscular synapse growth.EMBO reports · 2022Article
- FARS2 deficiency in Drosophila reveals the developmental delay and seizure manifested by aberrant mitochondrial tRNA metabolism.Nucleic acids research · 2021Article
- Identification of juvenile hormone-induced posttranslational modifications of methoprene tolerant and Krüppel homolog 1 in the yellow fever mosquito, Aedes aegypti.Journal of proteomics · 2021Article
- Inactivation of the mitochondrial protease Afg3l2 results in severely diminished respiratory chain activity and widespread defects in mitochondrial gene expression.PLoS genetics · 2020Article
- Structural basis of mitochondrial translation.eLife · 2020Article
- Elevated expression of PTCD3 correlates with tumor progression and predicts poor prognosis in patients with prostate cancer.Molecular medicine reports · 2018Article
- A novel histochemistry assay to assess and quantify focal cytochrome c oxidase deficiency.The Journal of pathology · 2018Article
- Lon protease inactivation inCell death discovery · 2018Article
- Drosophila protease ClpXP specifically degrades DmLRPPRC1 controlling mitochondrial mRNA and translation.Scientific reports · 2017Article
- The Schizosaccharomyces pombe PPR protein Ppr10 associates with a novel protein Mpa1 and acts as a mitochondrial translational activator.Nucleic acids research · 2017Article
- SLIRP stabilizes LRPPRC via an RRM-PPR protein interface.Nucleic acids research · 2016Article
- Identification of LACTB2, a metallo-β-lactamase protein, as a human mitochondrial endoribonuclease.Nucleic acids research · 2016Article
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Authors and funding
9 authors at 3 institutions in 2 countries.
Funding
Abstract
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.
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