The DOI matches its Crossref record; 26 of the 26 references it cites were found in a registry or an index.
TWINKLE Has 5' −>3' DNA helicase activity and is specifically stimulated by mitochondrial single-stranded DNA-binding protein
Open accessAbstract
Mutations in TWINKLE cause autosomal dominant progressive external ophthalmoplegia, a human disorder associated with multiple deletions in the mitochondrial DNA. TWINKLE displays primary sequence similarity to the phage T7 gene 4 primase-helicase, but no specific enzyme activity has been assigned to the protein. We have purified recombinant TWINKLE to near homogeneity and demonstrate here that TWINKLE is a DNA helicase with 5' to 3' directionality and distinct substrate requirements. The protein needs a stretch of 10 nucleotides of single-stranded DNA on the 5'-side of the duplex to unwind duplex DNA. In addition, helicase activity is not observed unless a short single-stranded 3'-tail is present. The helicase activity has an absolute requirement for hydrolysis of a nucleoside 5'-triphosphate, with UTP being the optimal substrate. DNA unwinding by TWINKLE is specifically stimulated by the mitochondrial single-stranded DNA-binding protein. Our enzymatic characterization strongly supports the notion that TWINKLE is the helicase at the mitochondrial DNA replication fork and provides evidence for a close relationship of the DNA replication machinery in bacteriophages and mammalian mitochondria.
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“Twinkle is a 72 kDA hexamer/heptamer that functions as a 5′ → 3′ helicase for mtDNA replication (Korhonen et al., 2003).”
From Role of the mitochondrial DNA replication machinery in mitochondrial DNA mutagenesis, aging and age-related diseases (2017) · cites this paper as Korhonen et al., 2003
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Cites
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Mitochondrial Dynamics in Mammalian Health and Disease
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Mitochondria in health and disease: perspectives on a new mitochondrial biology
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Maintenance and Expression of Mammalian Mitochondrial DNA
Gustafsson, C. M., Falkenberg, M., & Larsson, N. (2016) · Annual Review of Biochemistry · doi:10.1146/annurev-biochem-060815-014402 · Cited by 688
DNA replication and transcription in mammalian mitochondria.
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Metadata
- Authors
- Korhonen, J. A., Gaspari, M., & Falkenberg, M.
- Affiliations
- Karolinska Institutet (SE)
- Corresponding author
- Falkenberg, Maria
- ORCID
- Falkenberg, Maria
- Venue
- Journal of Biological Chemistry
- Publisher
- Elsevier BV
- Published
- 2003-12
- Volume
- 278
- Issue
- 49
- Pages
- 48627-48632
- ISSN
- 0021-9258 (ISSN-L)
- OpenAlex
- W2077517552
- Cited by
- 298
- References
- 26
- Citations by year
- 2026: 9 · 2025: 14 · 2024: 6 · 2023: 9 · 2022: 14 · 2021: 15 · 2020: 15 · 2019: 12 · 2018: 20 · 2017: 6 · 2016: 16 · 2015: 19 · 2014: 12 · 2013: 13 · 2012: 11
- FWCI
- 2.99
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Journal of Biological Chemistry (publishedVersion, cc-by)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › DNA Repair Mechanisms
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › DNA and Nucleic Acid Chemistry
- Keywords
- Twinkle, DNA helicase, mitochondrial DNA replication, mitochondrial single-stranded DNA-binding protein, chronic progressive external ophthalmoplegia, mitochondrial DNA deletions, single-stranded DNA
- MeSH terms
- Animals, Base Sequence, Cell Line, DNA Helicases, DNA-Binding Proteins, Mitochondria, Recombinant Proteins, DNA Primers, DNA Primase
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