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dc.contributor.author | Ishida, Yamato | en |
dc.contributor.author | Tasaki, Koshi | en |
dc.contributor.author | Katoh, Yohei | en |
dc.contributor.author | Nakayama, Kazuhisa | en |
dc.contributor.alternative | 石田, 大和 | ja |
dc.contributor.alternative | 田崎, 晃司 | ja |
dc.contributor.alternative | 加藤, 洋平 | ja |
dc.contributor.alternative | 中山, 和久 | ja |
dc.date.accessioned | 2022-10-14T06:54:08Z | - |
dc.date.available | 2022-10-14T06:54:08Z | - |
dc.date.issued | 2022-08-01 | - |
dc.identifier.uri | http://hdl.handle.net/2433/276723 | - |
dc.description.abstract | Bidirectional protein trafficking within cilia is mediated by the intraflagellar transport (IFT) machinery, which contains the IFT-A and IFT-B complexes powered by the kinesin-2 and dynein-2 motors. Mutations in genes encoding subunits of the IFT-A and dynein-2 complexes cause skeletal ciliopathies. Some subunits of the IFT-B complex, including IFT52, IFT80, and IFT172, are also mutated in skeletal ciliopathies. We here show that IFT52 variants found in individuals with short-rib polydactyly syndrome (SRPS) are compromised in terms of formation of the IFT-B holocomplex from two subcomplexes, and its interaction with heterotrimeric kinesin-II. IFT52-knockout (KO) cells expressing IFT52 variants that mimic the cellular conditions of individuals with SRPS demonstrated mild ciliogenesis defects and a decrease in ciliary IFT-B level. Furthermore, in IFT52-KO cells expressing an SRPS variant of IFT52, ciliary tip localization of ICK/CILK1 and KIF17, both of which are likely to be transported to the tip via binding to the IFT-B complex, were significantly impaired. These results altogether indicate that impaired anterograde trafficking caused by a decrease in the ciliary level of IFT-B or in its binding to kinesin-II underlies the ciliary defects found in skeletal ciliopathies caused by IFT52 variations. | en |
dc.language.iso | eng | - |
dc.publisher | American Society for Cell Biology (ASCB) | en |
dc.rights | © 2022 Ishida et al. | en |
dc.rights | This article is distributed by The American Society for Cell Biology under license from the author(s). Two months after publication it is available to the public under an Attribution–Noncommercial-Share Alike 4.0 International Creative Commons License | en |
dc.rights.uri | http://creativecommons.org/licenses/by-nc-sa/4.0 | - |
dc.title | Molecular basis underlying the ciliary defects caused by IFT52 variations found in skeletal ciliopathies | en |
dc.type | journal article | - |
dc.type.niitype | Journal Article | - |
dc.identifier.jtitle | Molecular Biology of the Cell | en |
dc.identifier.volume | 33 | - |
dc.identifier.issue | 9 | - |
dc.relation.doi | 10.1091/mbc.E22-05-0188 | - |
dc.textversion | publisher | - |
dc.identifier.artnum | ar83 | - |
dc.identifier.pmid | 35704471 | - |
dcterms.accessRights | open access | - |
datacite.awardNumber | 19H00980 | - |
datacite.awardNumber | 20H04904 | - |
datacite.awardNumber | 18H02403 | - |
datacite.awardNumber | 21H02427 | - |
datacite.awardNumber | 22J20116 | - |
datacite.awardNumber.uri | https://kaken.nii.ac.jp/ja/grant/KAKENHI-PROJECT-19H00980/ | - |
datacite.awardNumber.uri | https://kaken.nii.ac.jp/ja/grant/KAKENHI-PUBLICLY-20H04904/ | - |
datacite.awardNumber.uri | https://kaken.nii.ac.jp/ja/grant/KAKENHI-PROJECT-18H02403/ | - |
datacite.awardNumber.uri | https://kaken.nii.ac.jp/ja/grant/KAKENHI-PROJECT-21H02427/ | - |
datacite.awardNumber.uri | https://kaken.nii.ac.jp/ja/grant/KAKENHI-PROJECT-22J20116/ | - |
dc.identifier.eissn | 1939-4586 | - |
jpcoar.funderName | 日本学術振興会 | ja |
jpcoar.funderName | 日本学術振興会 | ja |
jpcoar.funderName | 日本学術振興会 | ja |
jpcoar.funderName | 日本学術振興会 | ja |
jpcoar.funderName | 日本学術振興会 | ja |
jpcoar.awardTitle | 繊毛内タンパク質輸送複合体の構築様式と機能の相関:繊毛病の分子基盤 | ja |
jpcoar.awardTitle | 繊毛内と細胞質を隔てるトランジション・ゾーンの構築様式と選択的タンパク質透過機構 | ja |
jpcoar.awardTitle | 多角的アプローチによる繊毛内タンパク質輸送システムの解明 | ja |
jpcoar.awardTitle | 多角的な研究手法の組み合わせによる繊毛内タンパク質輸送システムの統合的理解 | ja |
jpcoar.awardTitle | 間葉系幹細胞の分化における一次繊毛の機能に着目した繊毛病の分子基盤の解明 | ja |
出現コレクション: | 学術雑誌掲載論文等 |
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