{"_buckets": {"deposit": "486e6050-cf0d-484d-90e9-ac643533bbb6"}, "_deposit": {"id": "25570", "owners": [], "pid": {"revision_id": 0, "type": "depid", "value": "25570"}, "status": "published"}, "_oai": {"id": "oai:niigata-u.repo.nii.ac.jp:00025570", "sets": ["456", "1306"]}, "item_7_alternative_title_1": {"attribute_name": "その他のタイトル", "attribute_value_mlt": [{"subitem_alternative_title": "Molecular mechanisms of abnormal chondrocyte differentiation in the fetal cranial base caused by the effect of activated FGFR2 signaling"}]}, "item_7_biblio_info_6": {"attribute_name": "書誌情報", "attribute_value_mlt": [{"bibliographicIssueDates": {"bibliographicIssueDate": "2006-06", "bibliographicIssueDateType": "Issued"}, "bibliographicIssueNumber": "1", "bibliographicPageEnd": "48", "bibliographicPageStart": "39", "bibliographicVolumeNumber": "36", "bibliographic_titles": [{"bibliographic_title": "新潟歯学会雑誌"}, {"bibliographic_title": "新潟歯学会雑誌", "bibliographic_titleLang": "en"}]}]}, "item_7_description_4": {"attribute_name": "抄録", "attribute_value_mlt": [{"subitem_description": "Apert syndrome exhibits the specific clinical features including abnormal premature fusion of calvarial sutures and syndactyly, which are known to be caused by miss sense mutations of Ser252Trp or Pro253Arg on Fibroblast growth factor receptor 2(FGFR2) gene responsible for abnormal activation of the tyrosin kinase and osteoblast differentiation. On the other hand, premature bony fusion of cranial base synchondroses are another major skeletal abnormalities, however the mutant FGFR2 signaling in the cranial cartilage is still remain to be elucidated. Purpose of this study is to clarify molecular mechanism of the craniofacial endochondral growth affected by abnormal FGFR2 signaling. Transgenic mice bearing the Apert type mutant Fgfr2 gene (Fgfr2ⅢCP253R) driven by promoter/enhancer of Col2a1 gene were used to analyze the effect of cartilage specific expression. Elevated expression level of Runx2/Cbfa1, Indian hedgehog (Ihh) and Matrix metalloproteinase13 (Mmp-13) suggested accelerated chondrocytes differentiation with the exogenous Fgfr2ⅢCP253R gene expression in cranial base cartilage. Taken together with histological findings showing reduced size of cranial base cartilage and accelerated bone formations, activated FGFR2 signaling negatively regulate the growth of cranial base through modulating the endochondral growth process.", "subitem_description_type": "Abstract"}, {"subitem_description": "Apert症候群は頭蓋冠縫合早期異常癒合や合指症など特徴的な病態を呈し、Fibroblast growth factor receptor 2(FGFR2)遺伝子のセリン252トリプトファンあるいはプロリン253アルギニンなどのレセプター活性化型ミスセンスが頭蓋冠縫合部の骨芽細胞の分化を亢進することが原因とされている。一方でApert症候群において軟骨縫合の早期異常閉鎖がもう一つの主要な骨格異常であるにも関わらず、軟骨内における変異型FGFR2シグナリングの役割は定義されていない。この研究では、FGFR2の活性化が頭蓋顔面の軟骨性成長におよぼす影響について分子機構の一端を解明することを目的とした。実験動物にはApert症候群型変異Fgfr2遺伝子(Fgfr2ⅢcP253R)を2型コラゲンのプロモーターによって軟骨組織特異的に発現するトランスジェニックマウス(Tgマウス)を用いた。変異Fgfr2ⅢCP253R遺伝子導入に伴いRunx2/Cbfa1,Indian hedgehog(Ihh),Matrix metalloproteinase 13(Mpm-13)遺伝子の検出レベル上昇が軟骨組織に観察され、FGFR2シグナリング活性化が軟骨分化に変化をもたらしたことが示唆された。組織所見における頭蓋底軟骨縫合の縮小と骨化亢進を考え合わせると、FGFR2シグナリングが軟骨性成長の抑制を通じて頭蓋底の成長を抑制的に調節すると考えられた。総じて、FGFR2シグナリングは骨芽細胞系と軟骨細胞系の細胞分化に対する統合的な調節作用を通じて頭蓋顔面の形態形成と発育に重要な役割を果たすことが示唆された。", "subitem_description_type": "Abstract"}]}, "item_7_full_name_3": {"attribute_name": "著者別名", "attribute_value_mlt": [{"nameIdentifiers": [{"nameIdentifier": "150703", "nameIdentifierScheme": "WEKO"}], "names": [{"name": "Seki, Yukie"}]}, {"nameIdentifiers": [{"nameIdentifier": "150704", "nameIdentifierScheme": "WEKO"}], "names": [{"name": "Nagata, Masaki"}]}, {"nameIdentifiers": [{"nameIdentifier": "150705", "nameIdentifierScheme": "WEKO"}], "names": [{"name": "Kodama, Naoki"}]}, {"nameIdentifiers": [{"nameIdentifier": "150706", "nameIdentifierScheme": "WEKO"}], "names": [{"name": "Takagi, Ritsuo"}]}]}, "item_7_publisher_7": {"attribute_name": "出版者", "attribute_value_mlt": [{"subitem_publisher": "新潟歯学会"}]}, "item_7_select_19": {"attribute_name": "著者版フラグ", "attribute_value_mlt": [{"subitem_select_item": "publisher"}]}, "item_7_source_id_11": {"attribute_name": "書誌レコードID", "attribute_value_mlt": [{"subitem_source_identifier": "AN0018300X", "subitem_source_identifier_type": "NCID"}]}, "item_7_source_id_9": {"attribute_name": "ISSN", "attribute_value_mlt": [{"subitem_source_identifier": "03850153", "subitem_source_identifier_type": "ISSN"}]}, "item_creator": {"attribute_name": "著者", "attribute_type": "creator", "attribute_value_mlt": [{"creatorNames": [{"creatorName": "関, 雪絵"}], "nameIdentifiers": [{"nameIdentifier": "150699", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "永田, 昌毅"}], "nameIdentifiers": [{"nameIdentifier": "150700", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "小玉, 直樹"}], "nameIdentifiers": [{"nameIdentifier": "150701", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "高木, 律男"}], "nameIdentifiers": [{"nameIdentifier": "150702", "nameIdentifierScheme": "WEKO"}]}]}, "item_files": {"attribute_name": "ファイル情報", "attribute_type": "file", "attribute_value_mlt": [{"accessrole": "open_date", "date": [{"dateType": "Available", "dateValue": "2019-08-19"}], "displaytype": "detail", "download_preview_message": "", "file_order": 0, "filename": "NS_36(1)_39-48.pdf", "filesize": [{"value": "5.1 MB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensetype": "license_free", "mimetype": "application/pdf", "size": 5100000.0, "url": {"label": "NS_36(1)_39-48.pdf", "url": "https://niigata-u.repo.nii.ac.jp/record/25570/files/NS_36(1)_39-48.pdf"}, "version_id": "b20dc5d7-0eef-48fc-a4f7-eb1984a41c67"}]}, "item_keyword": {"attribute_name": "キーワード", "attribute_value_mlt": [{"subitem_subject": "fibroblast growth factor receptor type 2", "subitem_subject_scheme": "Other"}, {"subitem_subject": "FGFR2", "subitem_subject_scheme": "Other"}, {"subitem_subject": "Apert syndrome", "subitem_subject_scheme": "Other"}, {"subitem_subject": "頭蓋底", "subitem_subject_scheme": "Other"}, {"subitem_subject": "軟骨分化", "subitem_subject_scheme": "Other"}, {"subitem_subject": "軟骨縫合", "subitem_subject_scheme": "Other"}]}, "item_language": {"attribute_name": "言語", "attribute_value_mlt": [{"subitem_language": "jpn"}]}, "item_resource_type": {"attribute_name": "資源タイプ", "attribute_value_mlt": [{"resourcetype": "departmental bulletin paper", "resourceuri": "http://purl.org/coar/resource_type/c_6501"}]}, "item_title": "FGFR2シグナリング活性化が胎仔頭蓋底の軟骨分化におよぼす影響と分子機構の検討", "item_titles": {"attribute_name": "タイトル", "attribute_value_mlt": [{"subitem_title": "FGFR2シグナリング活性化が胎仔頭蓋底の軟骨分化におよぼす影響と分子機構の検討"}, {"subitem_title": "FGFR2シグナリング活性化が胎仔頭蓋底の軟骨分化におよぼす影響と分子機構の検討", "subitem_title_language": "en"}]}, "item_type_id": "7", "owner": "1", "path": ["456", "1306"], "permalink_uri": "http://hdl.handle.net/10191/23088", "pubdate": {"attribute_name": "公開日", "attribute_value": "2013-08-23"}, "publish_date": "2013-08-23", "publish_status": "0", "recid": "25570", "relation": {}, "relation_version_is_last": true, "title": ["FGFR2シグナリング活性化が胎仔頭蓋底の軟骨分化におよぼす影響と分子機構の検討"], "weko_shared_id": null}