The inability to construct animal models of human diseases caused by mutated mitochondrial DNA (mtDNA) has been a major obstacle to investigating pathogenetic mechanisms associated with specific mtDNA mutations. Mice carrying a large-scale deletion in mtDNA have now been produced. They display some of the key features of the human disorder, but there are surprising differences.
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Shoubridge, E. A debut for mito-mouse. Nat Genet 26, 132–134 (2000). https://doi.org/10.1038/79832
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DOI: https://doi.org/10.1038/79832
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