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Mitochondrial DNA replication in mammalian cells: overview of the pathway

Review article
Authors Maria Falkenberg
Published in Essays in Biochemistry
Volume 62
Issue 3
Pages 287-296
ISSN 0071-1365
Publication year 2018
Published at Institute of Biomedicine, Department of Medical Biochemistry and Cell Biology
Pages 287-296
Language en
Links dx.doi.org/10.1042/ebc20170100
Keywords mouse l-cells, polymerase-gamma, transcription factor, d-loop, embryonic, lethality, accessory subunit, escherichia-coli, binding protein, rna-polymerase, knockout mice
Subject categories Biochemistry

Abstract

Mammalian mitochondria contain multiple copies of a circular, double-stranded DNA genome and a dedicated DNA replication machinery is required for its maintenance. Many disease-causing mutations affect mitochondrial replication factors and a detailed understanding of the replication process may help to explain the pathogenic mechanisms underlying a number of mitochondrial diseases. We here give a brief overview of DNA replication in mammalian mitochondria, describing our current understanding of this process and some unanswered questions remaining.

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