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Mitochondrial DNA with a Large-Scale Deletion Causes Two Distinct Mitochondrial Disease Phenotypes in Mice
http://hdl.handle.net/2241/119861
http://hdl.handle.net/2241/11986125c0b506-b90a-4a12-aa6f-086034cb60d2
名前 / ファイル | ライセンス | アクション |
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G3_3-9.pdf (1.9 MB)
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Item type | Journal Article(1) | |||||
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公開日 | 2013-10-21 | |||||
タイトル | ||||||
タイトル | Mitochondrial DNA with a Large-Scale Deletion Causes Two Distinct Mitochondrial Disease Phenotypes in Mice | |||||
言語 | ||||||
言語 | eng | |||||
資源タイプ | ||||||
資源 | http://purl.org/coar/resource_type/c_6501 | |||||
タイプ | journal article | |||||
著者 |
Katada, Shun
× Katada, Shun× Mito, Takayuki× Ogasawara, Emi× Hayashi, Jun-Ichi× Nakada, Kazuto |
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著者別名 |
林, 純一
× 林, 純一× 中田, 和人 |
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抄録 | ||||||
内容記述タイプ | Abstract | |||||
内容記述 | Studies in patients have suggested that the clinical phenotypes of some mitochondrial diseases might transit from one disease to another (e.g., Pearson syndrome [PS] to Kearns-Sayre syndrome) in single individuals carrying mitochondrial (mt) DNA with a common deletion (∆mtDNA), but there is no direct experimental evidence for this. To determine whether ∆mtDNA has the pathologic potential to induce multiple mitochondrial disease phenotypes, we used trans-mitochondrial mice with a heteroplasmic state of wild-type mtDNA and ∆mtDNA (mito-mice∆). Late-stage embryos carrying ≥50% ∆mtDNA showed abnormal hematopoiesis and iron metabolism in livers that were partly similar to PS (PS-like phenotypes), although they did not express sideroblastic anemia that is a typical symptom of PS. More than half of the neonates with PS-like phenotypes died by 1 month after birth, whereas the rest showed a decrease of ∆mtDNA load in the affected tissues, peripheral blood and liver, and they recovered from PS-like phenotypes. The proportion of ∆mtDNA in various tissues of the surviving mito-mice∆ increased with time, and Kearns-Sayre syndrome−like phenotypes were expressed when the proportion of ∆mtDNA in various tissues reached >70–80%. Our model mouse study clearly showed that a single ∆mtDNA was responsible for at least two distinct disease phenotypes at different ages and suggested that the level and dynamics of ∆mtDNA load in affected tissues would be important for the onset and transition of mitochondrial disease phenotypes in mice. | |||||
書誌情報 |
G3: Genes, Genomes, Genetics 巻 3, 号 9, p. 1545-1552, 発行日 2013-09 |
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ISSN | ||||||
収録物識別子タイプ | ISSN | |||||
収録物識別子 | 2160-1836 | |||||
PubMed番号 | ||||||
識別子タイプ | PMID | |||||
関連識別子 | 23853091 | |||||
DOI | ||||||
識別子タイプ | DOI | |||||
関連識別子 | 10.1534/g3.113.007245 | |||||
権利 | ||||||
権利情報 | © 2013 Katada et al. This is an open-access article distributed under the terms of the Creative Commons Attribution Unported License (http://creativecommons.org/licenses/ by/3.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. | |||||
著者版フラグ | ||||||
値 | publisher | |||||
出版者 | ||||||
出版者 | Genetics Society of America | |||||
URI | ||||||
識別子 | http://hdl.handle.net/2241/119861 | |||||
識別子タイプ | HDL |