{"created":"2023-06-19T10:43:16.416882+00:00","id":3479,"links":{},"metadata":{"_buckets":{"deposit":"9bc6c8e3-a474-4ee6-bc59-43041f892c17"},"_deposit":{"created_by":11,"id":"3479","owners":[11],"pid":{"revision_id":0,"type":"depid","value":"3479"},"status":"published"},"_oai":{"id":"oai:shiga-med.repo.nii.ac.jp:00003479","sets":["19:82:83"]},"author_link":["4059","6456","6523","6393","7474","7664","1869","7671","7672","7673","7667","6506","4000","7666","7669","7471","7670","7668","7665","538","6470"],"item_4_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2019-12-11","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"1","bibliographicPageStart":"5517","bibliographicVolumeNumber":"10","bibliographic_titles":[{},{"bibliographic_title":"Nature communications","bibliographic_titleLang":"en"}]}]},"item_4_creator_3":{"attribute_name":"著者別名","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"築山, 智之"}],"nameIdentifiers":[{},{},{}]},{"creatorNames":[{"creatorName":"小林, 憲市"}],"nameIdentifiers":[{},{}]},{"creatorNames":[{"creatorName":"中家, 雅隆"}],"nameIdentifiers":[{},{}]},{"creatorNames":[{"creatorName":"岩谷, 千鶴"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"清田, 弥寿成"}],"nameIdentifiers":[{},{},{}]},{"creatorNames":[{"creatorName":"土屋, 英明"}],"nameIdentifiers":[{},{}]},{"creatorNames":[{"creatorName":"松下, 淳"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"北嶋, 郁"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"河本, 育士"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"中川, 孝博"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"久米, 真司"}],"nameIdentifiers":[{},{},{}]},{"creatorNames":[{"creatorName":"前川, 聡"}],"nameIdentifiers":[{},{},{}]},{"creatorNames":[{"creatorName":"中村, 紳一郎"}],"nameIdentifiers":[{},{}]},{"creatorNames":[{"creatorName":"河内, 明宏"}],"nameIdentifiers":[{},{}]},{"creatorNames":[{"creatorName":"依馬, 正次"}],"nameIdentifiers":[{},{},{}]}]},"item_4_description_4":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"Autosomal dominant polycystic kidney disease (ADPKD) caused by PKD1 mutations is one of the most common hereditary disorders. However, the key pathological processes underlying cyst development and exacerbation in pre-symptomatic stages remain unknown, because rodent models do not recapitulate critical disease phenotypes, including disease onset in heterozygotes. Here, using CRISPR/Cas9, we generate ADPKD models with PKD1 mutations in cynomolgus monkeys. As in humans and mice, near-complete PKD1 depletion induces severe cyst formation mainly in collecting ducts. Importantly, unlike in mice, PKD1 heterozygote monkeys exhibit cyst formation perinatally in distal tubules, possibly reflecting the initial pathology in humans. Many monkeys in these models survive after cyst formation, and cysts progress with age. Furthermore, we succeed in generating selective heterozygous mutations using allele-specific targeting. We propose that our models elucidate the onset and progression of ADPKD, which will serve as a critical basis for establishing new therapeutic strategies, including drug treatments.","subitem_description_type":"Abstract"}]},"item_4_description_42":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"subitem_description":"Journal Article","subitem_description_type":"Other"}]},"item_4_publisher_32":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"Springer Nature"}]},"item_4_relation_10":{"attribute_name":"PubMed番号","attribute_value_mlt":[{"subitem_relation_type":"isIdenticalTo","subitem_relation_type_id":{"subitem_relation_type_id_text":"31822676","subitem_relation_type_select":"PMID"}}]},"item_4_relation_11":{"attribute_name":"DOI","attribute_value_mlt":[{"subitem_relation_name":[{"subitem_relation_name_text":"10.1038/s41467-019-13398-6"}],"subitem_relation_type":"isIdenticalTo","subitem_relation_type_id":{"subitem_relation_type_id_text":"https://doi.org/10.1038/s41467-019-13398-6","subitem_relation_type_select":"DOI"}}]},"item_4_rights_12":{"attribute_name":"権利","attribute_value_mlt":[{"subitem_rights":"© The Author(s) 2019"}]},"item_4_source_id_7":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"2041-1723","subitem_source_identifier_type":"ISSN"}]},"item_4_version_type_15":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_version_resource":"http://purl.org/coar/version/c_970fb48d4fbd8a85","subitem_version_type":"VoR"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"TSUKIYAMA, Tomoyuki"}],"nameIdentifiers":[{},{},{}]},{"creatorNames":[{"creatorName":"KOBAYASHI, Kenichi"}],"nameIdentifiers":[{},{}]},{"creatorNames":[{"creatorName":"NAKAYA, Masataka"}],"nameIdentifiers":[{},{}]},{"creatorNames":[{"creatorName":"IWATANI, Chizuru"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"SEITA, Yasunari"}],"nameIdentifiers":[{},{},{}]},{"creatorNames":[{"creatorName":"TSUCHIYA, Hideaki"}],"nameIdentifiers":[{},{}]},{"creatorNames":[{"creatorName":"MATSUSHITA, Jun"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"KITAJIMA, Kahoru"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"KAWAMOTO, Ikuo"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"NAKAGAWA, Takahiro"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"FUKUDA, Koji"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"IWAKIRI, Teppei"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"IZUMI, Hiroyuki"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"ITAGAKI, Iori"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"KUME, Shinji"}],"nameIdentifiers":[{},{},{}]},{"creatorNames":[{"creatorName":"MAEGAWA, Hiroshi"}],"nameIdentifiers":[{},{},{}]},{"creatorNames":[{"creatorName":"NISHINAKAMURA, Ryuichi"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"NISHIO, Saori"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"NAKAMURA, Shinichiro"}],"nameIdentifiers":[{},{}]},{"creatorNames":[{"creatorName":"KAWAUCHI, Akihiro"}],"nameIdentifiers":[{},{}]},{"creatorNames":[{"creatorName":"EMA, Masatsugu"}],"nameIdentifiers":[{},{},{}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2019-12-16"}],"displaytype":"detail","filename":"s41467-019-13398-6.pdf","filesize":[{"value":"9.2 MB"}],"format":"application/pdf","license_note":"© The Author(s) 2019\nThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"s41467-019-13398-6","url":"https://shiga-med.repo.nii.ac.jp/record/3479/files/s41467-019-13398-6.pdf"},"version_id":"27985e61-a2b8-41d1-b47a-6b639c76be4f"}]},"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":"Monkeys mutant for PKD1 recapitulate human autosomal dominant polycystic kidney disease.","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"Monkeys mutant for PKD1 recapitulate human autosomal dominant polycystic kidney disease."}]},"item_type_id":"4","owner":"11","path":["83"],"pubdate":{"attribute_name":"公開日","attribute_value":"2019-12-16"},"publish_date":"2019-12-16","publish_status":"0","recid":"3479","relation_version_is_last":true,"title":["Monkeys mutant for PKD1 recapitulate human autosomal dominant polycystic kidney disease."],"weko_creator_id":"11","weko_shared_id":-1},"updated":"2023-06-19T12:23:34.949750+00:00"}