{"@context":{"@vocab":"https://cir.nii.ac.jp/schema/1.0/","rdfs":"http://www.w3.org/2000/01/rdf-schema#","dc":"http://purl.org/dc/elements/1.1/","dcterms":"http://purl.org/dc/terms/","foaf":"http://xmlns.com/foaf/0.1/","prism":"http://prismstandard.org/namespaces/basic/2.0/","cinii":"http://ci.nii.ac.jp/ns/1.0/","datacite":"https://schema.datacite.org/meta/kernel-4/","ndl":"http://ndl.go.jp/dcndl/terms/","jpcoar":"https://github.com/JPCOAR/schema/blob/master/2.0/"},"@id":"https://cir.nii.ac.jp/crid/1361418519747799552.json","@type":"Article","productIdentifier":[{"identifier":{"@type":"DOI","@value":"10.1038/ncb1962"}},{"identifier":{"@type":"URI","@value":"http://www.nature.com/articles/ncb1962.pdf"}},{"identifier":{"@type":"URI","@value":"http://www.nature.com/articles/ncb1962"}},{"identifier":{"@type":"PMID","@value":"19767744"}}],"dc:title":[{"@value":"Regulation of endoplasmic reticulum stress response by a BBF2H7-mediated Sec23a pathway is essential for chondrogenesis"}],"description":[{"notation":[{"@value":"Many tissues have a specific signal transduction system for endoplasmic reticulum (ER) dysfunction; however, the mechanisms underlying the ER stress response in cartilage remain unclear. BBF2H7 (BBF2 human homologue on chromosome 7), an ER-resident basic leucine zipper transcription factor, is activated in response to ER stress and is highly expressed in chondrocytes. In this study, we generated Bbf2h7(-/-) mice to assess the in vivo function of BBF2H7. The mice showed severe chondrodysplasia and died by suffocation shortly after birth because of an immature chest cavity. The cartilage showed a lack of typical columnar structure in the proliferating zone and a decrease in the size of the hypertrophic zone, resulting in a significant reduction of extracellular matrix proteins. Interestingly, proliferating chondrocytes showed abnormally expanded ER, containing aggregated type II collagen (Col2) and cartilage oligomeric matrix protein (COMP). We identified Sec23a, which encodes a coat protein complex II component responsible for protein transport from the ER to the Golgi, as a target of BBF2H7, which directly bound to a CRE-like sequence in the promoter region of Sec23a to activate its transcription. When Sec23a was introduced to Bbf2h7(-/-) chondrocytes, the impaired transport and secretion of cartilage matrix proteins was totally restored, indicating that by activating protein secretion the BBF2H7-Sec23a pathway has a crucial role in chondrogenesis. Our findings provide a new link by which ER stress is converted to signalling for the activation of ER-to-Golgi trafficking."}]}],"creator":[{"@id":"https://cir.nii.ac.jp/crid/1381418519747799681","@type":"Researcher","foaf:name":[{"@value":"Atsushi Saito"}]},{"@id":"https://cir.nii.ac.jp/crid/1381418519747799552","@type":"Researcher","foaf:name":[{"@value":"Shin-ichiro Hino"}]},{"@id":"https://cir.nii.ac.jp/crid/1381418519747799680","@type":"Researcher","foaf:name":[{"@value":"Tomohiko Murakami"}]},{"@id":"https://cir.nii.ac.jp/crid/1381418519747799686","@type":"Researcher","foaf:name":[{"@value":"Soshi Kanemoto"}]},{"@id":"https://cir.nii.ac.jp/crid/1381418519747799682","@type":"Researcher","foaf:name":[{"@value":"Shinichi Kondo"}]},{"@id":"https://cir.nii.ac.jp/crid/1381418519747799683","@type":"Researcher","foaf:name":[{"@value":"Masahiro Saitoh"}]},{"@id":"https://cir.nii.ac.jp/crid/1381418519747799684","@type":"Researcher","foaf:name":[{"@value":"Riko Nishimura"}]},{"@id":"https://cir.nii.ac.jp/crid/1381418519747799554","@type":"Researcher","foaf:name":[{"@value":"Toshiyuki Yoneda"}]},{"@id":"https://cir.nii.ac.jp/crid/1381418519747799553","@type":"Researcher","foaf:name":[{"@value":"Tatsuya Furuichi"}]},{"@id":"https://cir.nii.ac.jp/crid/1381418519747799555","@type":"Researcher","foaf:name":[{"@value":"Shiro Ikegawa"}]},{"@id":"https://cir.nii.ac.jp/crid/1381418519747799556","@type":"Researcher","foaf:name":[{"@value":"Masahito Ikawa"}]},{"@id":"https://cir.nii.ac.jp/crid/1381418519747799685","@type":"Researcher","foaf:name":[{"@value":"Masaru Okabe"}]},{"@id":"https://cir.nii.ac.jp/crid/1381418519747799557","@type":"Researcher","foaf:name":[{"@value":"Kazunori Imaizumi"}]}],"publication":{"publicationIdentifier":[{"@type":"PISSN","@value":"14657392"},{"@type":"EISSN","@value":"14764679"},{"@type":"PISSN","@value":"https://id.crossref.org/issn/14764679"}],"prism:publicationName":[{"@value":"Nature Cell Biology"}],"dc:publisher":[{"@value":"Springer Science and Business Media LLC"}],"prism:publicationDate":"2009-09-20","prism:volume":"11","prism:number":"10","prism:startingPage":"1197","prism:endingPage":"1204"},"reviewed":"false","dc:rights":["http://www.springer.com/tdm"],"url":[{"@id":"http://www.nature.com/articles/ncb1962.pdf"},{"@id":"http://www.nature.com/articles/ncb1962"}],"createdAt":"2009-09-20","modifiedAt":"2023-05-18","foaf:topic":[{"@id":"https://cir.nii.ac.jp/all?q=Cartilage,%20Articular","dc:title":"Cartilage, Articular"},{"@id":"https://cir.nii.ac.jp/all?q=Vesicular%20Transport%20Proteins","dc:title":"Vesicular Transport Proteins"},{"@id":"https://cir.nii.ac.jp/all?q=Golgi%20Apparatus","dc:title":"Golgi Apparatus"},{"@id":"https://cir.nii.ac.jp/all?q=Ribs","dc:title":"Ribs"},{"@id":"https://cir.nii.ac.jp/all?q=Cartilage%20Oligomeric%20Matrix%20Protein","dc:title":"Cartilage Oligomeric Matrix Protein"},{"@id":"https://cir.nii.ac.jp/all?q=Endoplasmic%20Reticulum","dc:title":"Endoplasmic Reticulum"},{"@id":"https://cir.nii.ac.jp/all?q=Mice","dc:title":"Mice"},{"@id":"https://cir.nii.ac.jp/all?q=Chondrocytes","dc:title":"Chondrocytes"},{"@id":"https://cir.nii.ac.jp/all?q=Animals","dc:title":"Animals"},{"@id":"https://cir.nii.ac.jp/all?q=Matrilin%20Proteins","dc:title":"Matrilin Proteins"},{"@id":"https://cir.nii.ac.jp/all?q=Collagen%20Type%20II","dc:title":"Collagen Type II"},{"@id":"https://cir.nii.ac.jp/all?q=Cells,%20Cultured","dc:title":"Cells, Cultured"},{"@id":"https://cir.nii.ac.jp/all?q=In%20Situ%20Hybridization","dc:title":"In Situ Hybridization"},{"@id":"https://cir.nii.ac.jp/all?q=Glycoproteins","dc:title":"Glycoproteins"},{"@id":"https://cir.nii.ac.jp/all?q=Mice,%20Knockout","dc:title":"Mice, Knockout"},{"@id":"https://cir.nii.ac.jp/all?q=Extracellular%20Matrix%20Proteins","dc:title":"Extracellular Matrix Proteins"},{"@id":"https://cir.nii.ac.jp/all?q=Gene%20Expression%20Regulation,%20Developmental","dc:title":"Gene Expression Regulation, Developmental"},{"@id":"https://cir.nii.ac.jp/all?q=Embryo,%20Mammalian","dc:title":"Embryo, Mammalian"},{"@id":"https://cir.nii.ac.jp/all?q=Immunohistochemistry","dc:title":"Immunohistochemistry"},{"@id":"https://cir.nii.ac.jp/all?q=Protein%20Transport","dc:title":"Protein Transport"},{"@id":"https://cir.nii.ac.jp/all?q=Basic-Leucine%20Zipper%20Transcription%20Factors","dc:title":"Basic-Leucine Zipper Transcription Factors"},{"@id":"https://cir.nii.ac.jp/all?q=Chondrogenesis","dc:title":"Chondrogenesis"}],"relatedProduct":[{"@id":"https://cir.nii.ac.jp/crid/1050026026868599936","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@language":"en","@value":"Activation of OASIS family, ER stress transducers, is dependent on its 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