{"created":"2021-03-01T07:07:54.245649+00:00","id":29416,"links":{},"metadata":{"_buckets":{"deposit":"9b8c8c74-943b-4223-b2b6-df0f279897f8"},"_deposit":{"id":"29416","owners":[],"pid":{"revision_id":0,"type":"depid","value":"29416"},"status":"published"},"_oai":{"id":"oai:tsukuba.repo.nii.ac.jp:00029416","sets":["160:849","160:854","3:62:5589:2551"]},"item_5_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2013-09","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"9","bibliographicPageEnd":"1552","bibliographicPageStart":"1545","bibliographicVolumeNumber":"3","bibliographic_titles":[{"bibliographic_title":"G3: Genes, Genomes, Genetics"}]}]},"item_5_creator_3":{"attribute_name":"著者別名","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"林, 純一"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"中田, 和人"}],"nameIdentifiers":[{},{},{}]}]},"item_5_description_4":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"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.","subitem_description_type":"Abstract"}]},"item_5_identifier_34":{"attribute_name":"URI","attribute_value_mlt":[{"subitem_identifier_type":"HDL","subitem_identifier_uri":"http://hdl.handle.net/2241/119861"}]},"item_5_publisher_27":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"Genetics Society of America"}]},"item_5_relation_10":{"attribute_name":"PubMed番号","attribute_value_mlt":[{"subitem_relation_type_id":{"subitem_relation_type_id_text":"23853091","subitem_relation_type_select":"PMID"}}]},"item_5_relation_11":{"attribute_name":"DOI","attribute_value_mlt":[{"subitem_relation_type_id":{"subitem_relation_type_id_text":"10.1534/g3.113.007245","subitem_relation_type_select":"DOI"}}]},"item_5_rights_12":{"attribute_name":"権利","attribute_value_mlt":[{"subitem_rights":"© 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."}]},"item_5_select_15":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_select_item":"publisher"}]},"item_5_source_id_7":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"2160-1836","subitem_source_identifier_type":"ISSN"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"Katada, Shun"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Mito, Takayuki"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Ogasawara, Emi"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Hayashi, Jun-Ichi"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Nakada, Kazuto"}],"nameIdentifiers":[{}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2014-05-09"}],"displaytype":"detail","filename":"G3_3-9.pdf","filesize":[{"value":"1.9 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"G3_3-9.pdf","url":"https://tsukuba.repo.nii.ac.jp/record/29416/files/G3_3-9.pdf"},"version_id":"9eb75a21-012f-40b9-b88f-4421596e60ce"}]},"item_language":{"attribute_name":"言語","attribute_value_mlt":[{"subitem_language":"eng"}]},"item_resource_type":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"resourcetype":"journal article","resourceuri":"http://purl.org/coar/resource_type/c_6501"}]},"item_title":"Mitochondrial DNA with a Large-Scale Deletion Causes Two Distinct Mitochondrial Disease Phenotypes in Mice","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"Mitochondrial DNA with a Large-Scale Deletion Causes Two Distinct Mitochondrial Disease Phenotypes in Mice"}]},"item_type_id":"5","owner":"1","path":["849","854","2551"],"pubdate":{"attribute_name":"公開日","attribute_value":"2013-10-21"},"publish_date":"2013-10-21","publish_status":"0","recid":"29416","relation_version_is_last":true,"title":["Mitochondrial DNA with a Large-Scale Deletion Causes Two Distinct Mitochondrial Disease Phenotypes in Mice"],"weko_creator_id":"1","weko_shared_id":5},"updated":"2022-04-27T08:55:58.869696+00:00"}