{"_buckets": {"deposit": "ee23d753-f1e6-4478-8ce9-9c3bfca15248"}, "_deposit": {"id": "16443", "owners": [], "pid": {"revision_id": 0, "type": "depid", "value": "16443"}, "status": "published"}, "_oai": {"id": "oai:niigata-u.repo.nii.ac.jp:00016443", "sets": ["456", "1104"]}, "item_7_alternative_title_1": {"attribute_name": "その他のタイトル", "attribute_value_mlt": [{"subitem_alternative_title": "Adenovirus-mediated Expression of Truncated DRPLA Protein Containing an Expanded Polyglutamine Stretch Results in Intranuclear Inclusion in Neuronal PC12"}]}, "item_7_biblio_info_6": {"attribute_name": "書誌情報", "attribute_value_mlt": [{"bibliographicIssueDates": {"bibliographicIssueDate": "1998-09", "bibliographicIssueDateType": "Issued"}, "bibliographicIssueNumber": "9", "bibliographicPageEnd": "572", "bibliographicPageStart": "563", "bibliographicVolumeNumber": "112", "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": "Dentatorubtal-pallidoluysian atrophy (DRPLA) is an autosomal dominant neurodegenerative disorder caused by unstable expansion of a CAG trinucleotide repeat of DRPLA, gene on chromospme 12p13.31. Although recent investigations suggest that mutant proteins with expanded polyglutamine stretches exert gain of toxic functions to neuronal cells, molecular mechanisms of the toxicity to neuronal cells remain unclear. To elucidate molecular mechanisms of neurodegeneration caused by expanded CAG repeats in DRPLA, we established an efficient expression system of truncated DRPLA proteins with normal or expanded polyglutamine stretches, Using COS/TPC method, we constructed adenovirus vectors containing truncated DRPLA cDNA coding for normal or expaneded polyglutamine stretches (19 or 82 glutamines) and the flanking regions. Using these adenovirus vectors, truncated DRPLA proteins with normal or expanded polyglutatnine stretchs were expressed in neuronal PC12 cells and human cultured skin fibroblasts. Formation of aggregate bodeis were observed in cells expressing the truncated DRPLA protein with the expanded polyglutamine stretch, but not in cells expressing that with the normal stretch. In neuronal PC12 cells, more than 97 %, of cells showed intranuclear inclusions at 3 days after infection, while only 36.0±29.6 % of fibroblasts had intranuclear inclusios at 4 days after infection. Numbers of apoptotic cells were higher in cells expressing the expanded polyglutamine stretch than in those expressing the normal polyglutamine stretch, especially in neuronal PC12. The results showed neuronoal PC12 cells are more vulnerable to mutant DRPLA proteins than fibroblasts. This expression system of truncated wild type and mutant DRPLA proteins is proved to be a useful system to analyze the toxicity of mutant proteins containing expanded polyglutamine stretches in neuronal cells.", "subitem_description_type": "Abstract"}]}, "item_7_full_name_3": {"attribute_name": "著者別名", "attribute_value_mlt": [{"nameIdentifiers": [{"nameIdentifier": "104685", "nameIdentifierScheme": "WEKO"}], "names": [{"name": "Sato, Aki"}]}]}, "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": "佐藤, 晶"}], "nameIdentifiers": [{"nameIdentifier": "104684", "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": "2019-08-07"}], "displaytype": "detail", "download_preview_message": "", "file_order": 0, "filename": "112(9)_563-572.pdf", "filesize": [{"value": "1.6 MB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensetype": "license_free", "mimetype": "application/pdf", "size": 1600000.0, "url": {"label": "112(9)_563-572.pdf", "url": "https://niigata-u.repo.nii.ac.jp/record/16443/files/112(9)_563-572.pdf"}, "version_id": "0d0e7f5c-7fcd-4b53-a6ad-8bde1f89a744"}]}, "item_keyword": {"attribute_name": "キーワード", "attribute_value_mlt": [{"subitem_subject": "DRPLA", "subitem_subject_scheme": "Other"}, {"subitem_subject": "CAG trinucleotid disease", "subitem_subject_scheme": "Other"}, {"subitem_subject": "adenovirus", "subitem_subject_scheme": "Other"}, {"subitem_subject": "COS-TPC method", "subitem_subject_scheme": "Other"}, {"subitem_subject": "neuronal PC12", "subitem_subject_scheme": "Other"}, {"subitem_subject": "歯状核赤核淡蒼球ルイ体萎縮症", "subitem_subject_scheme": "Other"}, {"subitem_subject": "CAGリピート病", "subitem_subject_scheme": "Other"}, {"subitem_subject": "アデノウイルスベクター", "subitem_subject_scheme": "Other"}, {"subitem_subject": "COS-TPC法", "subitem_subject_scheme": "Other"}, {"subitem_subject": "分化型PC12", "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": "歯状核赤核・淡蒼球ルイ体萎縮症蛋白の伸長ポリグルタミン鎖のアデノウイルスベクターを用いた発現による神経細胞障害機構の解析", "item_titles": {"attribute_name": "タイトル", "attribute_value_mlt": [{"subitem_title": "歯状核赤核・淡蒼球ルイ体萎縮症蛋白の伸長ポリグルタミン鎖のアデノウイルスベクターを用いた発現による神経細胞障害機構の解析"}, {"subitem_title": "歯状核赤核・淡蒼球ルイ体萎縮症蛋白の伸長ポリグルタミン鎖のアデノウイルスベクターを用いた発現による神経細胞障害機構の解析", "subitem_title_language": "en"}]}, "item_type_id": "7", "owner": "1", "path": ["456", "1104"], "permalink_uri": "http://hdl.handle.net/10191/46201", "pubdate": {"attribute_name": "公開日", "attribute_value": "2017-02-07"}, "publish_date": "2017-02-07", "publish_status": "0", "recid": "16443", "relation": {}, "relation_version_is_last": true, "title": ["歯状核赤核・淡蒼球ルイ体萎縮症蛋白の伸長ポリグルタミン鎖のアデノウイルスベクターを用いた発現による神経細胞障害機構の解析"], "weko_shared_id": null}