{"created":"2021-03-01T06:12:10.803418+00:00","id":8176,"links":{},"metadata":{"_buckets":{"deposit":"3f7aedf2-6f59-466b-b169-e6b377ea343e"},"_deposit":{"id":"8176","owners":[],"pid":{"revision_id":0,"type":"depid","value":"8176"},"status":"published"},"_oai":{"id":"oai:niigata-u.repo.nii.ac.jp:00008176","sets":["453:456","471:537:538:881"]},"item_7_alternative_title_1":{"attribute_name":"その他のタイトル","attribute_value_mlt":[{"subitem_alternative_title":"Analyses of a Novel Naturally Occurring Dystonin/Bpag1 Mutation as a Mouse Model of Epidermolysis Bullosa Simplex"}]},"item_7_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2017-11","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"11","bibliographicPageEnd":"663","bibliographicPageStart":"655","bibliographicVolumeNumber":"131","bibliographic_titles":[{"bibliographic_title":"新潟医学会雑誌"},{"bibliographic_title":"新潟医学会雑誌","bibliographic_titleLang":"en"}]}]},"item_7_description_4":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"Dystonia musculorum (dt)マウスはDystonin遺伝子(Dst，別名Bpag1)の変異によってジストニア様の運動障害を示す自然発生突然変異マウスである．Dstは組織ごとに異なるアイソフォームを発現し，皮層型アイソフォームは表皮と真皮の間の接着装置であるヘミデスモソームの構成タンパク質であるDst-e (Bpagle，BP230)をコードする．ヒトDst-eのホモ接合体変異は，表皮下に緊満性水庖が生じる単純性表皮水庖症[epidermolysis bullosa simplex(EBS)]の原因となる．本研究では最近新しく遺伝子変異が同定されたDst^<dt-23Rbrc>マウスの皮膚を組織学的に解析した．肉眼所見では，Dst^<dt-23Rbrc>ホモマウスの皮膚に明らかな異常は認められなかったが，尾の皮膚において表皮と真皮の接着強度の低下が確認された．In situ hybridization法により皮膚におけるDst-eの遺伝子発現を検討したところ，Dst^<dt-23Rbrc>ホモマウスの皮膚においてDst-eの発現は検出されなかった．次に，ヘマトキシリン・エオジン(HE)染色と抗pan-Keratin抗体(KL-1)の免疫染色を行い，光学顕微鏡観察を行ったところ，表皮内と真皮内の構造に異常は認められなかったが，表皮真皮接合部に一部裂隙がみられ， そこに水庖が観察された．また抗Uchl1 (PGP9.5)抗体の免疫染色により，Dst^<dt-23Rbrc>ホモマウスの表皮と真皮において感覚神経終末の減少が認められた．さらに電子顕微鏡観察により，上皮細胞一基底膜間の接着装置であるヘミデスモソームにおいてDst-eにより構成される細胞膜裏打ち構造の消失が観察された．以上の結果から，Dst^<dt-23Rbrc>ホモマウスではへミデスモソームの構造異常によって表皮と真皮の接着強度が低下して,これにより表皮下に水庖が生じると考えられた．従ってDSt^<dt-23Rbrc>マウスはEBSの新規モデル動物として有用である．","subitem_description_type":"Abstract"}]},"item_7_full_name_3":{"attribute_name":"著者別名","attribute_value_mlt":[{"nameIdentifiers":[{"nameIdentifier":"57512","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Kuriyama, Momona"}]},{"nameIdentifiers":[{"nameIdentifier":"57513","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Yoshioka, Nozomu"}]},{"nameIdentifiers":[{"nameIdentifier":"57514","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Kabata, Yudai"}]},{"nameIdentifiers":[{"nameIdentifier":"57515","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Ushiki, Tatsuo"}]},{"nameIdentifiers":[{"nameIdentifier":"57516","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Yoshiki, Atsushi"}]},{"nameIdentifiers":[{"nameIdentifier":"57517","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Abe, Riichiro"}]},{"nameIdentifiers":[{"nameIdentifier":"57518","nameIdentifierScheme":"WEKO"}],"names":[{"name":"Takebayashi, Hirohide"}]}]},"item_7_publisher_7":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"新潟医学会"}]},"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":"AN00182415","subitem_source_identifier_type":"NCID"}]},"item_7_source_id_9":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"00290440","subitem_source_identifier_type":"ISSN"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"栗山, 桃奈"}],"nameIdentifiers":[{"nameIdentifier":"57504","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"吉岡, 望"}],"nameIdentifiers":[{"nameIdentifier":"57505","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"加畑, 雄大"}],"nameIdentifiers":[{"nameIdentifier":"57506","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"牛木, 辰男"}],"nameIdentifiers":[{"nameIdentifier":"57507","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"吉木, 淳"}],"nameIdentifiers":[{"nameIdentifier":"57508","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Thomas, J Sproule"}],"nameIdentifiers":[{"nameIdentifier":"57509","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"阿部, 理一郎"}],"nameIdentifiers":[{"nameIdentifier":"57510","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"竹林, 浩秀"}],"nameIdentifiers":[{"nameIdentifier":"57511","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":"2019-08-06"}],"displaytype":"detail","filename":"131(11)_655-663.pdf","filesize":[{"value":"7.3 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"131(11)_655-663.pdf","url":"https://niigata-u.repo.nii.ac.jp/record/8176/files/131(11)_655-663.pdf"},"version_id":"1072b467-b209-47cc-999e-0b541ad80654"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"Dystonia musculorum(dt)マウス","subitem_subject_scheme":"Other"},{"subitem_subject":"Dst ^<dt-23Rbrc>アリール","subitem_subject_scheme":"Other"},{"subitem_subject":"Dst-e(Bpagle,BP230)","subitem_subject_scheme":"Other"},{"subitem_subject":"単純性表皮水庖症","subitem_subject_scheme":"Other"},{"subitem_subject":"epidermolysis bullosa simplex(EBS)","subitem_subject_scheme":"Other"},{"subitem_subject":"水庖性類天庖瘡","subitem_subject_scheme":"Other"},{"subitem_subject":"Bullous pemphigoid(BP)","subitem_subject_scheme":"Other"},{"subitem_subject":"遺伝性感覚性自律神経性ニューロパチーVI型","subitem_subject_scheme":"Other"},{"subitem_subject":"Hereditary sensory and autonomic neuropathy type VI(HSAN6)","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":"単純性表皮水庖症モデルとしての新規Dystonin/Bpag1遺伝子変異マウスの解析","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"単純性表皮水庖症モデルとしての新規Dystonin/Bpag1遺伝子変異マウスの解析"},{"subitem_title":"単純性表皮水庖症モデルとしての新規Dystonin/Bpag1遺伝子変異マウスの解析","subitem_title_language":"en"}]},"item_type_id":"7","owner":"1","path":["456","881"],"pubdate":{"attribute_name":"公開日","attribute_value":"2018-05-07"},"publish_date":"2018-05-07","publish_status":"0","recid":"8176","relation_version_is_last":true,"title":["単純性表皮水庖症モデルとしての新規Dystonin/Bpag1遺伝子変異マウスの解析"],"weko_creator_id":"1","weko_shared_id":null},"updated":"2022-12-15T03:42:34.392102+00:00"}