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Maintenance and Expression of Mammalian Mitochondrial DNA

Journal article
Authors Claes M Gustafsson
Maria Falkenberg
N. G. Larsson
Published in Annual Review of Biochemistry
Volume 85
Pages 133-160
ISSN 0066-4154
Publication year 2016
Published at Institute of Biomedicine, Department of Medical Biochemistry and Cell Biology
Pages 133-160
Language en
Keywords mitochondria, mtDNA, transcription, replication, respiratory chain, polymerase, mouse l-cells, circular daughter molecules, termination factor mterf, base excision-repair, pol-gamma-b, transcription factor, polymerase-gamma, rna-polymerase, in-vitro, accessory subunit
Subject categories Biochemistry


Mammalian mitochondrial DNA (mtDNA) encodes 13 proteins that are essential for the function of the oxidative phosphorylation system, which is composed of four respiratory-chain complexes and adenosine triphosphate (ATP) synthase. Remarkably, the maintenance and expression of mtDNA depend on the mitochondrial import of hundreds of nuclear-encoded proteins that control genome maintenance, replication, transcription, RNA maturation, and mitochondrial translation. The importance of this complex regulatory system is underscored by the identification of numerous mutations of nuclear genes that impair mtDNA maintenance and expression at different levels, causing human mitochondrial diseases with pleiotropic clinical manifestations. The basic scientific understanding of the mechanisms controlling mtDNA function has progressed considerably during the past few years, thanks to advances in biochemistry, genetics, and structural biology. The challenges for the future will be to understand how mtDNA maintenance and expression are regulated and to what extent direct intramitochondrial cross talk between different processes, such as transcription and translation, is important.

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