{"_buckets": {"deposit": "712c11f1-c6da-4cfb-a32b-b0a32240fef6"}, "_deposit": {"id": "948", "owners": [], "pid": {"revision_id": 0, "type": "depid", "value": "948"}, "status": "published"}, "_oai": {"id": "oai:repository.dl.itc.u-tokyo.ac.jp:00000948"}, "item_2_biblio_info_7": {"attribute_name": "\u66f8\u8a8c\u60c5\u5831", "attribute_value_mlt": [{"bibliographicIssueDates": {"bibliographicIssueDate": "2017-01-03", "bibliographicIssueDateType": "Issued"}, "bibliographicPageStart": "39994", "bibliographicVolumeNumber": "7", "bibliographic_titles": [{"bibliographic_title": "Scientific Reports Online Edition"}]}]}, "item_2_description_5": {"attribute_name": "\u6284\u9332", "attribute_value_mlt": [{"subitem_description": "Nuclear dysfunction in motor neurons has been hypothesized to be a principal cause of amyotrophic lateral sclerosis (ALS) pathogenesis. Here, we investigated the mechanism by which the nuclear pore complex (NPC) is disrupted in dying motor neurons in a mechanistic ALS mouse model (adenosine deaminase acting on RNA 2 (ADAR2) conditional knockout (AR2) mice) and in ALS patients. We showed that nucleoporins (Nups) that constituted the NPC were cleaved by activated calpain via a Ca2+-permeable AMPA receptor-mediated mechanism in dying motor neurons lacking ADAR2 expression in AR2 mice. In these neurons, nucleo-cytoplasmic transport was disrupted, and the level of the transcript elongation enzyme RNA polymerase II phosphorylated at Ser2 was significantly decreased. Analogous changes were observed in motor neurons lacking ADAR2 immunoreactivity in sporadic ALS patients. Therefore, calpain-dependent NPC disruption may participate in ALS pathogenesis, and inhibiting Ca2+-mediated cell death signals may be a therapeutic strategy for ALS.", "subitem_description_type": "Abstract"}]}, "item_2_description_6": {"attribute_name": "\u5185\u5bb9\u8a18\u8ff0", "attribute_value_mlt": [{"subitem_description": "UTokyo Research\u63b2\u8f09\u300c\u6838\u3068\u7d30\u80de\u8cea\u9593\u306e\u5206\u5b50\u8f38\u9001\u7d4c\u8def\u306e\u7834\u7dbb\u304c\u7b4b\u840e\u7e2e\u6027\u5074\u7d22\u786c\u5316\u75c7\u306b\u95a2\u4e0e\u300d URI: http://www.u-tokyo.ac.jp/ja/utokyo-research/research-news/disruption-of-molecule-transport-pathway-between-nucleus-and-cytoplasm-involved-in-als.html", "subitem_description_type": "Other"}, {"subitem_description": "UTokyo Research \"Disruption of molecule transport pathway between nucleus and cytoplasm involved in ALS\" URI: http://www.u-tokyo.ac.jp/en/utokyo-research/research-news/disruption-of-molecule-transport-pathway-between-nucleus-and-cytoplasm-involved-in-als.html", "subitem_description_type": "Other"}]}, "item_2_publisher_20": {"attribute_name": "\u51fa\u7248\u8005", "attribute_value_mlt": [{"subitem_publisher": "Nature Publishing Group"}]}, "item_2_relation_11": {"attribute_name": "DOI", "attribute_value_mlt": [{"subitem_relation_type_id": {"subitem_relation_type_id_text": "info:doi/10.1038/srep39994", "subitem_relation_type_select": "DOI"}}]}, "item_2_relation_25": {"attribute_name": "\u95a2\u4fc2URI", "attribute_value_mlt": [{"subitem_relation_type_id": {"subitem_relation_type_id_text": "http://www.u-tokyo.ac.jp/ja/utokyo-research/research-news/disruption-of-molecule-transport-pathway-between-nucleus-and-cytoplasm-involved-in-als.html", "subitem_relation_type_select": "URI"}}, {"subitem_relation_type_id": {"subitem_relation_type_id_text": "http://www.u-tokyo.ac.jp/en/utokyo-research/research-news/disruption-of-molecule-transport-pathway-between-nucleus-and-cytoplasm-involved-in-als.html", "subitem_relation_type_select": "URI"}}, {"subitem_relation_type_id": {"subitem_relation_type_id_text": "https://dx.doi.org/10.1038/srep39994", "subitem_relation_type_select": "URI"}}]}, "item_2_rights_12": {"attribute_name": "\u6a29\u5229", "attribute_value_mlt": [{"subitem_rights": "Copyright \u00a9 2017, Rights Managed by Nature Publishing Group"}]}, "item_2_select_14": {"attribute_name": "\u8457\u8005\u7248\u30d5\u30e9\u30b0", "attribute_value_mlt": [{"subitem_select_item": "publisher"}]}, "item_2_source_id_8": {"attribute_name": "ISSN", "attribute_value_mlt": [{"subitem_source_identifier": "2045-2322", "subitem_source_identifier_type": "ISSN"}]}, "item_2_text_34": {"attribute_name": "\u8cc7\u6e90\u30bf\u30a4\u30d7", "attribute_value_mlt": [{"subitem_text_value": "Jounal Article"}]}, "item_2_text_4": {"attribute_name": "\u8457\u8005\u6240\u5c5e", "attribute_value_mlt": [{"subitem_text_value": "Graduate School of Medicine, The University of Tokyo"}, {"subitem_text_value": "Department of Neurology, Tokyo Medical University"}, {"subitem_text_value": "Clinical Research Center for Medicine, International University of Health and Welfare"}]}, "item_creator": {"attribute_name": "\u8457\u8005", "attribute_type": "creator", "attribute_value_mlt": [{"creatorNames": [{"creatorName": "Yamashita, Takenari"}], "nameIdentifiers": [{"nameIdentifier": "3847", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Aizawa, Hitoshi"}], "nameIdentifiers": [{"nameIdentifier": "3848", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Teramoto, Sayaka"}], "nameIdentifiers": [{"nameIdentifier": "3849", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Akamatsu, Megumi"}], "nameIdentifiers": [{"nameIdentifier": "3850", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Kwak, Shin"}], "nameIdentifiers": [{"nameIdentifier": "3851", "nameIdentifierScheme": "WEKO"}]}]}, "item_files": {"attribute_name": "\u30d5\u30a1\u30a4\u30eb\u60c5\u5831", "attribute_type": "file", "attribute_value_mlt": [{"accessrole": "open_date", "date": [{"dateType": "Available", "dateValue": "2017-05-30"}], "displaytype": "detail", "download_preview_message": "", "file_order": 0, "filename": "srep39994.pdf", "filesize": [{"value": "2.0 MB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensetype": "license_free", "mimetype": "application/pdf", "size": 2000000.0, "url": {"label": "srep39994.pdf", "url": "https://repository.dl.itc.u-tokyo.ac.jp/record/948/files/srep39994.pdf"}, "version_id": "724bbb05-9cc5-417a-94c9-9f59c01eaa5b"}]}, "item_keyword": {"attribute_name": "\u30ad\u30fc\u30ef\u30fc\u30c9", "attribute_value_mlt": [{"subitem_subject": "Amyotrophic lateral sclerosis", "subitem_subject_scheme": "Other"}, {"subitem_subject": "Molecular neuroscience", "subitem_subject_scheme": "Other"}]}, "item_language": {"attribute_name": "\u8a00\u8a9e", "attribute_value_mlt": [{"subitem_language": "eng"}]}, "item_resource_type": {"attribute_name": "\u8cc7\u6e90\u30bf\u30a4\u30d7", "attribute_value_mlt": [{"resourcetype": "journal article", "resourceuri": "http://purl.org/coar/resource_type/c_6501"}]}, "item_title": "Calpain-dependent disruption of nucleo-cytoplasmic transport in ALS motor neurons", "item_titles": {"attribute_name": "\u30bf\u30a4\u30c8\u30eb", "attribute_value_mlt": [{"subitem_title": "Calpain-dependent disruption of nucleo-cytoplasmic transport in ALS motor neurons"}]}, "item_type_id": "2", "owner": "1", "path": ["125/211", "9/10/11"], "permalink_uri": "http://hdl.handle.net/2261/61310", "pubdate": {"attribute_name": "\u516c\u958b\u65e5", "attribute_name_i18n": "\u516c\u958b\u65e5", "attribute_value": "2017-01-19"}, "publish_date": "2017-01-19", "publish_status": "0", "recid": "948", "relation": {}, "relation_version_is_last": true, "title": ["Calpain-dependent disruption of nucleo-cytoplasmic transport in ALS motor neurons"], "weko_shared_id": null}