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1: Science. 2008 Jul 25;321(5888):575-7.
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Manipulating the metazoan mitochondrial genome with targeted restriction enzymes.

Xu H, DeLuca SZ, O'Farrell PH.

Department of Biochemistry and Biophysics, University of California at San Francisco, San Francisco, CA 94158-2200, USA.

High copy number and random segregation confound genetic analysis of the mitochondrial genome. We developed an efficient selection for heritable mitochondrial genome (mtDNA) mutations in Drosophila, thereby enhancing a metazoan model for study of mitochondrial genetics and mutations causing human mitochondrial disease. Targeting a restriction enzyme to mitochondria in the germline compromised fertility, but escaper progeny carried homoplasmic mtDNA mutations lacking the cleavage site. Among mutations eliminating a site in the cytochrome c oxidase gene, mt:CoI(A302T) was healthy, mt:CoI(R301L) was male sterile but otherwise healthy, and mt:CoI(R301S) exhibited a wide range of defects, including growth retardation, neurodegeneration, muscular atrophy, male sterility, and reduced life span. Thus, germline expression of mitochondrial restriction enzymes creates a powerful selection and has allowed direct isolation of mitochondrial mutants in a metazoan.

Publication Types:
PMID: 18653897 [PubMed - indexed for MEDLINE]