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Human Molecular Genetics Advance Access first published online on July 31, 2007
This version published online on August 30, 2007
Human Molecular Genetics, doi:10.1093/hmg/ddm182
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© The Author 2007. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/2.0/uk/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

The yeast Holliday junction resolvase, Cce1, can restore wild-type mitochondrial DNA to human cells carrying rearranged mitochondrial DNA

Hiroshi Sembongi, Miriam Di Re, Monika Bokori-Brown and Ian J. Holt*

MRC-Dunn Human Nutrition Unit, Hills Road, Cambridge, CB2 OXY, UK

* Corresponding author: email holt{at}mrc-dunn.cam.ac.uk Tel. + 44 12 23 2 5 28 40 Fax. + 44 12 23 2 5 28 45

Received May 9, 2007; Revised June 12, 2007; Accepted July 6, 2007

Rearrangements of mitochondrial DNA are a well-recognised cause of human disease; deletions are more frequent, but duplications are more readily transmitted to offspring. In theory partial duplications of mitochondrial DNA can be resolved to partially deleted and wild-type molecules, via homologous recombination. Therefore, the yeast CCE1 gene, encoding a Holliday junction resolvase, was introduced into cells carrying partially duplicated or partially triplicated mitochondrial DNA. Some cell lines carrying the CCE1 gene had substantial amounts of wild-type mitochondrial DNA suggesting that the enzyme can mediate intramolecular recombination in human mitochondria. However, high levels of expression of CCE1 frequently led to mitochondrial DNA loss, and so it is necessary to strictly regulate the expression of CCE1 in human cells to ensure the selection and maintenance of wild-type mitochondrial DNA.

This paper has been versioned to remove data intended for review purposes only.


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A. Malena, E. Loro, M. Di Re, I. J. Holt, and L. Vergani
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