ArticleThe Journal of biological chemistry2008
Role of SUV3 helicase in maintaining mitochondrial homeostasis in human cells.
Article in The Journal of biological chemistry, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 40 papers.
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Who cites it
40 citing papers in PubMed, 58 citations in OpenAlex.
- Mitochondrial double-stranded RNA homeostasis depends on cell-cycle progression.Life science alliance · 2024Article
- Mitochondria and G-quadruplex evolution: an intertwined relationship.Trends in genetics : TIG · 2023Review
- Dimeric assembly of human Suv3 helicase promotes its RNA unwinding function in mitochondrial RNA degradosome for RNA decay.Protein science : a publication of the Protein Society · 2022Article
- Mitochondrial RNA processing defect caused by a SUPV3L1 mutation in two siblings with a novel neurodegenerative syndrome.Journal of inherited metabolic disease · 2022Article
- Interplay between Mitochondrial Metabolism and Cellular Redox State Dictates Cancer Cell Survival.Oxidative medicine and cellular longevity · 2021Review
- Review
- Human mitochondrial degradosome prevents harmful mitochondrial R loops and mitochondrial genome instability.Proceedings of the National Academy of Sciences of the United States of America · 2018Article
- Dedicated surveillance mechanism controls G-quadruplex forming non-coding RNAs in human mitochondria.Nature communications · 2018Article
- Polyadenylation and degradation of structurally abnormal mitochondrial tRNAs in human cells.Nucleic acids research · 2018Article
- PNPase knockout results in mtDNA loss and an altered metabolic gene expression program.PloS one · 2018Article
- Mammalian mitochondrial RNAs are degraded in the mitochondrial intermembrane space by RNASET2.Protein & cell · 2017Article
- Mechanistic and biological considerations of oxidatively damaged DNA for helicase-dependent pathways of nucleic acid metabolism.Free radical biology & medicine · 2017Review
- Mitochondrial transcription in mammalian cells.Frontiers in bioscience (Landmark edition) · 2017Review
- Mitochondrial DNA repair: a novel therapeutic target for heart failure.Heart failure reviews · 2016Review
- Novel subcellular localization of the DNA helicase Twinkle at the kinetochore complex during mitosis in neuronal-like progenitor cells.Histochemistry and cell biology · 2016Article
- SUV3 helicase is required for correct processing of mitochondrial transcripts.Nucleic acids research · 2015Article
- Mitochondrial transcript maturation and its disorders.Journal of inherited metabolic disease · 2015Review
- Borrowing nuclear DNA helicases to protect mitochondrial DNA.International journal of molecular sciences · 2015Review
- Article
- Energizing Genetics and Epi-genetics: Role in the Regulation of Mitochondrial Function.Current genomics · 2014Article
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
Abstract
In yeast mitochondria, RNA degradation takes place through the coordinated activities of ySuv3 helicase and yDss1 exoribonuclease (mtEXO), whereas in bacteria, RNA is degraded via RNaseE, RhlB, PNPase, and enolase. Yeast lacking the Suv3 component of the mtEXO form petits and undergo a toxic accumulation of omega intron RNAs. Mammalian mitochondria resemble their prokaryotic origins by harboring a polyadenylation-dependent RNA degradation mechanism, but whether SUV3 participates in regulating RNA turnover in mammalian mitochondria is unclear. We found that lack of hSUV3 in mammalian cells subsequently yielded an accumulation of shortened polyadenylated mtRNA species and impaired mitochondrial protein synthesis. This suggests that SUV3 may serve in part as a component of an RNA degradosome, resembling its yeast ancestor. Reduction in the expression levels of oxidative phosphorylation components correlated with an increase in reactive oxygen species generation, whereas membrane potential and ATP production were decreased. These cumulative defects led to pleiotropic effects in mitochondria such as decreased mtDNA copy number and a shift in mitochondrial morphology from tubular to granular, which eventually manifests in cellular senescence or cell death. Thus, our results suggest that SUV3 is essential for maintaining proper mitochondrial function, likely through a conserved role in mitochondrial RNA regulation.
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