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Complementation between polymerase- and exonuclease-deficient mitochondrial DNA polymerase mutants in genomically engineered flies

Journal article
Authors A. Bratic
T. E. S. Kauppila
Bertil Macao
S. Grönke
Triinu Siibak
J. B. Stewart
F. Baggio
J. Dols
L. Partridge
Maria Falkenberg
A. Wredenberg
N. G. Larsson
Published in Nature Communications
Volume 6
Pages Article number: 8808
ISSN 2041-1723
Publication year 2015
Published at Institute of Biomedicine, Department of Medical Biochemistry and Cell Biology
Pages Article number: 8808
Language en
Subject categories Cell and Molecular Biology


Replication errors are the main cause of mitochondrial DNA (mtDNA) mutations and a compelling approach to decrease mutation levels would therefore be to increase the fidelity of the catalytic subunit (POLγA) of the mtDNA polymerase. Here we genomically engineer the tamas locus, encoding fly POLγA, and introduce alleles expressing exonuclease- (exo-) and polymerase-deficient (pol-) POLγA versions. The exo- mutant leads to accumulation of point mutations and linear deletions of mtDNA, whereas pol- mutants cause mtDNA depletion. The mutant tamas alleles are developmentally lethal but can complement each other in trans resulting in viable flies with clonally expanded mtDNA mutations. Reconstitution of human mtDNA replication in vitro confirms that replication is a highly dynamic process where POLγA goes on and off the template to allow complementation during proofreading and elongation. The created fly models are valuable tools to study germ line transmission of mtDNA and the pathophysiology of POLγA mutation disease. © 2015 Macmillan Publishers Limited. All rights reserved.

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