Reverse genetic studies of mitochondrial DNA-based diseases using a mouse model
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概要
- 論文の詳細を見る
In the situation that it would not be able to produce model animals for mitochondrial diseases caused by mitochondrial DNA (mtDNA) with pathogenic mutations, we succeeded in generating mice with pathogenic deletion mutant mtDNA (ΔmtDNA), named “mito-mice”, by direct introduction of mitochondria with ΔmtDNA into mouse zygotes. In the mito-mice, accumulation of ΔmtDNA induced mitochondrial respiration defects in various tissues, resulting in mitochondrial disease phenotypes, such as low body weight, lactic acidosis, ischemia, myopathy, heart block, deafness, male infertility, and renal failure. Thus, mito-mice are the first model animal for mtDNA-based diseases, and the mice could be valuable for understanding precise pathogeneses and testing therapies of mitochondrial diseases. In the present review, we summarized reverse genetic studies using the mito-mice.(Communicated by Takao SEKIYA, M.J.A.)
- 日本学士院の論文
著者
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Hayashi Jun-ichi
Graduate School Of Life And Environmental Sciences University Of Tsukuba
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Nakada Kazuto
Graduate School Of Life And Environmental Sciences Univ. Of Tsukuba
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SATO Akitsugu
Graduate School of Life and Environmental Sciences, University of Tsukuba
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NAKADA Kazuto
Graduate School of Life and Environmental Sciences, University of Tsukuba
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