Cited by 122Mitochondrial Function and PathologyDNA Repair MechanismsDNA and Nucleic Acid Chemistry

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Human Heart Mitochondrial DNA Is Organized in Complex Catenated Networks Containing Abundant Four-way Junctions and Replication Forks

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Pohjoismäki, Jaakko L.O., Goffart, Steffi, Tyynismaa, Henna, Willcox, Smaranda, Ide, Tomomi, Kang, Dongchon, Suomalainen, Anu, Karhunen, Pekka J., Griffith, Jack D., Holt, Ian J., Jacobs, Howard T.·2009·Journal of Biological Chemistry

DOI 10.1074/jbc.m109.016600PMC PMC2755869

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Abstract

Analysis of human heart mitochondrial DNA (mtDNA) by electron microscopy and agarose gel electrophoresis revealed a complete absence of the -type replication intermediates seen abundantly in mtDNA from all other tissues. Instead only Y- and X-junctional forms were detected after restriction digestion. Uncut heart mtDNA was organized in tangled complexes of up to 20 or more genome equivalents, which could be resolved to genomic monomers, dimers, and linear fragments by treatment with the decatenating enzyme topoisomerase IV plus the cruciform-cutting T7 endonuclease I. Human and mouse brain also contained a population of such mtDNA forms, which were absent, however, from mouse, rabbit, or pig heart. Overexpression in transgenic mice of two proteins involved in mtDNA replication, namely human mitochondrial transcription factor A or the mouse Twinkle DNA helicase, generated abundant four-way junctions in mtDNA of heart, brain, and skeletal muscle. The organization of mtDNA of human heart as well as of mouse and human brain in complex junctional networks replicating via a presumed non- mechanism is unprecedented in mammals.

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Authors: Pohjoismäki, J. L. O., Goffart, S., Tyynismaa, H., Willcox, S., Ide, T., Kang, D., Suomalainen, A., Karhunen, P. J., Griffith, J. D., Holt, I. J., & Jacobs, H. T.
Affiliations: Tampere University Hospital (FI)
University of Helsinki (FI)
University of North Carolina at Chapel Hill (US)
Kyushu University (JP)
University of Tampere (FI)
MRC Mitochondrial Biology Unit (GB)
Corresponding author: Jacobs, Howard T.
ORCID: Pohjoismäki, Jaakko L.O.
Goffart, Steffi
Tyynismaa, Henna
Willcox, Smaranda
Kang, Dongchon
Suomalainen, Anu
Karhunen, Pekka J.
Griffith, Jack D.
Holt, Ian J.
Jacobs, Howard T.
Venue: Journal of Biological Chemistry
Publisher: Elsevier BV
Published: 2009-08
Volume: 284
Issue: 32
Pages: 21446-21457
ISSN: 0021-9258 (ISSN-L)
DOI: 10.1074/jbc.m109.016600
PMC: PMC2755869
OpenAlex: W2014114000
Cited by: 122
References: 70
Citations by year: 2026: 1 · 2025: 1 · 2024: 9 · 2023: 4 · 2022: 8 · 2021: 6 · 2020: 8 · 2019: 10 · 2018: 10 · 2017: 4 · 2016: 8 · 2015: 10 · 2014: 5 · 2013: 9 · 2012: 10
FWCI: 3.89
Type: journal article
Language: en
Open access: hybrid
doi.org
Licence: cc-by
www.elsevier.com/tdm/userlicense/1.0/ (tdm)
creativecommons.org/licenses/by/4.0/ (vor, 4089 d)
Locations: Journal of Biological Chemistry (publishedVersion, cc-by)
PubMed Central (publishedVersion, cc-by-nc)
PubMed (publishedVersion)
Carolina Digital Repository (University of North Carolina at Chapel Hill) (submittedVersion)
UCL Discovery (University College London) (submittedVersion)
UNC Libraries
Funders: Foundation for Cardiovascular Research
European Commission
Academy of Finland
Suomen Kulttuurirahasto
Sydäntutkimussäätiö
Tays
National Institutes of Health
Medical Research Council
National Institute of General Medical Sciences (GM-31819, R01GM031819)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › DNA Repair Mechanisms
Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › DNA and Nucleic Acid Chemistry
Keywords: mitochondrial DNA, heart, four-way junction, replication fork, topoisomerase IV, T7 endonuclease I, TFAM, Twinkle helicase, mitochondrial DNA replication, brain, skeletal muscle, species differences
MeSH terms: Adult, Aged, Animals, Brain, Child, Preschool, DNA, Mitochondrial, Female, Heart, Humans, Male, Mice, Transgenic, Middle Aged, Nucleic Acid Conformation, Rabbits, Species Specificity, Swine, Tissue Distribution, Mice

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Human Heart Mitochondrial DNA Is Organized in Complex Catenated Networks Containing Abundant Four-way Junctions and Replication Forks

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