Journal Article

The yeast Holliday junction resolvase, <i>CCE1</i>, 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

in Human Molecular Genetics

Volume 16, issue 19, pages 2306-2314
Published in print October 2007 | ISSN: 0964-6906
Published online July 2007 | e-ISSN: 1460-2083 | DOI: http://dx.doi.org/10.1093/hmg/ddm182
The yeast Holliday junction resolvase, CCE1, can restore wild-type mitochondrial DNA to human cells carrying rearranged mitochondrial DNA

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

Journal Article.  4951 words.  Illustrated.

Subjects: Genetics and Genomics

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