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Maintenance and Expression of Mammalian Mitochondrial DNA

Artikel i vetenskaplig tidskrift
Författare Claes M Gustafsson
Maria Falkenberg
N. G. Larsson
Publicerad i Annual Review of Biochemistry
Volym 85
Sidor 133-160
ISSN 0066-4154
Publiceringsår 2016
Publicerad vid Institutionen för biomedicin, avdelningen för medicinsk kemi och cellbiologi
Sidor 133-160
Språk en
Länkar dx.doi.org/10.1146/annurev-biochem-...
Ämnesord 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
Ämneskategorier Biokemi

Sammanfattning

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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