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Updated: May 13, 2026

Mitochondrial Transformation in Baker's Yeast to Study Translation and Respiratory Complex Assembly
Published on: June 7, 2024
Yeast and human mitochondrial helicases
Roman J Szczesny1, Magdalena A Wojcik, Lukasz S Borowski
1Institute of Genetics and Biotechnology, University of Warsaw, Warsaw, Poland.
Abstract:
Mitochondria are semiautonomous organelles which contain their own genome. Both maintenance and expression of mitochondrial DNA require activity of RNA and DNA helicases. In Saccharomyces cerevisiae the nuclear genome encodes four DExH/D superfamily members (MSS116, SUV3, MRH4, IRC3) that act as helicases and/or RNA chaperones. Their activity is necessary for mitochondrial RNA splicing, degradation, translation and genome maintenance. In humans the ortholog of SUV3 (hSUV3, SUPV3L1) so far is the best described mitochondrial RNA helicase. The enzyme, together with the matrix-localized pool of PNPase (PNPT1), forms an RNA-degrading complex called the mitochondrial degradosome, which localizes to distinct structures (D-foci). Global regulation of mitochondrially encoded genes can be achieved by changing mitochondrial DNA copy number. This way the proteins involved in its replication, like the Twinkle helicase (c10orf2), can indirectly regulate gene expression. Here, we describe yeast and human mitochondrial helicases that are directly involved in mitochondrial RNA metabolism, and present other helicases that participate in mitochondrial DNA replication and maintenance. This article is part of a Special Issue entitled: The Biology of RNA helicases - Modulation for life.
Insights
Mitochondrial RNA helicases are crucial for maintaining and expressing mitochondrial DNA in yeast and humans. These enzymes are involved in RNA splicing, degradation, translation, and genome maintenance.
Area of Science:
- Molecular Biology
- Genetics
- Biochemistry
Background:
- Mitochondria possess their own genome, requiring RNA and DNA helicases for maintenance and expression.
- Saccharomyces cerevisiae has four nuclear-encoded DExH/D superfamily helicases (MSS116, SUV3, MRH4, IRC3) vital for mitochondrial RNA processes.
- Human SUV3 (hSUV3, SUPV3L1) is a key mitochondrial RNA helicase, forming a degradosome complex with PNPase (PNPT1) for RNA degradation.
Purpose of the Study:
- To review yeast and human mitochondrial helicases directly involved in RNA metabolism.
- To present helicases participating in mitochondrial DNA replication and maintenance.
Main Methods:
- Literature review of mitochondrial helicases in yeast and humans.
- Analysis of the roles of specific helicases in mitochondrial RNA metabolism and DNA replication.
Main Results:
- Four DExH/D superfamily helicases in yeast regulate mitochondrial RNA splicing, degradation, translation, and genome maintenance.
- Human hSUV3, along with PNPT1, forms a mitochondrial degradosome involved in RNA degradation.
- Helicases like Twinkle (c10orf2) indirectly regulate gene expression by influencing mitochondrial DNA replication.
Conclusions:
- Mitochondrial helicases play direct and indirect roles in mitochondrial gene expression and genome stability.
- Understanding these helicases is crucial for comprehending mitochondrial biology and potential therapeutic targets.
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