{"_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"}, "item_7_alternative_title_1": {"attribute_name": "\u305d\u306e\u4ed6\u306e\u30bf\u30a4\u30c8\u30eb", "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": "\u66f8\u8a8c\u60c5\u5831", "attribute_value_mlt": [{"bibliographicIssueDates": {"bibliographicIssueDate": "1998-09", "bibliographicIssueDateType": "Issued"}, "bibliographicIssueNumber": "9", "bibliographicPageEnd": "572", "bibliographicPageStart": "563", "bibliographicVolumeNumber": "112", "bibliographic_titles": [{"bibliographic_title": "\u65b0\u6f5f\u533b\u5b66\u4f1a\u96d1\u8a8c"}, {"bibliographic_title": "\u65b0\u6f5f\u533b\u5b66\u4f1a\u96d1\u8a8c", "bibliographic_titleLang": "en"}]}]}, "item_7_description_4": {"attribute_name": "\u6284\u9332", "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\u00b129.6 % 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": "\u8457\u8005\u5225\u540d", "attribute_value_mlt": [{"nameIdentifiers": [{"nameIdentifier": "104685", "nameIdentifierScheme": "WEKO"}], "names": [{"name": "Sato, Aki"}]}]}, "item_7_publisher_7": {"attribute_name": "\u51fa\u7248\u8005", "attribute_value_mlt": [{"subitem_publisher": "\u65b0\u6f5f\u533b\u5b66\u4f1a"}]}, "item_7_select_19": {"attribute_name": "\u8457\u8005\u7248\u30d5\u30e9\u30b0", "attribute_value_mlt": [{"subitem_select_item": "publisher"}]}, "item_7_source_id_11": {"attribute_name": "\u66f8\u8a8c\u30ec\u30b3\u30fc\u30c9ID", "attribute_value_mlt": [{"subitem_source_identifier": "AN00182415", "subitem_source_identifier_type": "NCID"}]}, "item_7_source_id_9": {"attribute_name": 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"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": "\u30ad\u30fc\u30ef\u30fc\u30c9", "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": "\u6b6f\u72b6\u6838\u8d64\u6838\u6de1\u84bc\u7403\u30eb\u30a4\u4f53\u840e\u7e2e\u75c7", "subitem_subject_scheme": "Other"}, {"subitem_subject": "CAG\u30ea\u30d4\u30fc\u30c8\u75c5", "subitem_subject_scheme": "Other"}, {"subitem_subject": "\u30a2\u30c7\u30ce\u30a6\u30a4\u30eb\u30b9\u30d9\u30af\u30bf\u30fc", "subitem_subject_scheme": "Other"}, {"subitem_subject": "COS-TPC\u6cd5", 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