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Separating and Segregating the Human Mitochondrial Genome

Journal article
Authors Thomas J. Nicholls
Claes M Gustafsson
Published in Trends in Biochemical Sciences
Volume 43
Issue 11
Pages 869-881
ISSN 0968-0004
Publication year 2018
Published at Institute of Biomedicine, Department of Medical Biochemistry and Cell Biology
Pages 869-881
Language en
Keywords DNA-polymerase gamma, optic atrophy, human mtdna, in-vitro, superresolution fluorescence, nucleoid organization, binding-properties, accessory subunit, topoisomerase-i, inner membrane
Subject categories Cell and Molecular Biology, Biochemistry and Molecular Biology


Cells contain thousands of copies of the mitochondrial genome. These genomes are distributed within the tubular mitochondrial network, which is itself spread across the cytosol of the cell. Mitochondrial DNA (mtDNA) replication occurs throughout the cell cycle and ensures that cells maintain a sufficient number of mtDNA copies. At replication termination the genomes must be resolved and segregated within the mitochondrial network. Defects in mtDNA replication and segregation are a cause of human mitochondrial disease associated with failure of cellular energy production. This review focuses upon recent developments on how mitochondrial genomes are physically separated at the end of DNA replication, and how these genomes are subsequently segregated and distributed around the mitochondrial network.

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