{"_buckets": {"deposit": "7e594caa-0f2e-44ad-9c74-b7e708fa1567"}, "_deposit": {"id": "9079", "owners": [], "pid": {"revision_id": 0, "type": "depid", "value": "9079"}, "status": "published"}, "_oai": {"id": "oai:niigata-u.repo.nii.ac.jp:00009079", "sets": ["456", "542"]}, "item_7_alternative_title_1": {"attribute_name": "その他のタイトル", "attribute_value_mlt": [{"subitem_alternative_title": "Analysis for Clinical Features and Molecular Mechanism of Early onset Ataxia with Ocular Motor Apraxia and Hypoalbuminemia"}]}, "item_7_biblio_info_6": {"attribute_name": "書誌情報", "attribute_value_mlt": [{"bibliographicIssueDates": {"bibliographicIssueDate": "2014-01", "bibliographicIssueDateType": "Issued"}, "bibliographicIssueNumber": "1", "bibliographicPageEnd": "6", "bibliographicPageStart": "1", "bibliographicVolumeNumber": "128", "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": "脊髄小脳変性症は, 小脳の神経細胞の変性, 脱落による運動失調を呈する神経変性疾患の一群であり, 弧発性, 遺伝性のものに分類できる. 劣性遺伝性の脊髄小脳変性症では, 欧米ではフリードライヒ失調症が最も頻度が高いが, 本邦ではフリードライヒ失調症は存在せず, フリードライヒ失調症と類似の運動症状を示し, 眼球運動失行と低アルブミン血症を伴う早発型失調症(EAOH)が最も頻度が高い. EAOHは, 患者数は少なく稀な疾患であるが, 臨床概念の確立, 原因遺伝子aprataxinの発見, アプラタキシン蛋白の機能解析と基礎研究から臨床まで多くの点で新潟大学の研究者が関わってきた疾患であり, 本学の研究者の研究結果を中心に, 本疾患を紹介させていただいた.", "subitem_description_type": "Abstract"}]}, "item_7_full_name_3": {"attribute_name": "著者別名", "attribute_value_mlt": [{"nameIdentifiers": [{"nameIdentifier": "63242", "nameIdentifierScheme": "WEKO"}], "names": [{"name": "Yokoseki, Akio"}]}]}, "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": "横関, 明男"}], "nameIdentifiers": [{"nameIdentifier": "63241", "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", "download_preview_message": "", "file_order": 0, "filename": "128(1)_1-6.pdf", "filesize": [{"value": "2.3 MB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensetype": "license_free", "mimetype": "application/pdf", "size": 2300000.0, "url": {"label": "128(1)_1-6.pdf", "url": "https://niigata-u.repo.nii.ac.jp/record/9079/files/128(1)_1-6.pdf"}, "version_id": "101a569f-9aed-4533-9b4b-3b7701910de8"}]}, "item_keyword": {"attribute_name": "キーワード", "attribute_value_mlt": [{"subitem_subject": "EAOH", "subitem_subject_scheme": "Other"}, {"subitem_subject": "AOA1", "subitem_subject_scheme": "Other"}, {"subitem_subject": "aprataxin", "subitem_subject_scheme": "Other"}, {"subitem_subject": "genotype-phenotype correlation", "subitem_subject_scheme": "Other"}, {"subitem_subject": "single strand break repair", "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": "眼球運動失行と低アルブミン血症を伴う早発型失調症(EAOH)の臨床病型と分子病態メカニズムの解明", "item_titles": {"attribute_name": "タイトル", "attribute_value_mlt": [{"subitem_title": "眼球運動失行と低アルブミン血症を伴う早発型失調症(EAOH)の臨床病型と分子病態メカニズムの解明"}, {"subitem_title": "眼球運動失行と低アルブミン血症を伴う早発型失調症(EAOH)の臨床病型と分子病態メカニズムの解明", "subitem_title_language": "en"}]}, "item_type_id": "7", "owner": "1", "path": ["456", "542"], "permalink_uri": "http://hdl.handle.net/10191/43591", "pubdate": {"attribute_name": "公開日", "attribute_value": "2016-09-02"}, "publish_date": "2016-09-02", "publish_status": "0", "recid": "9079", "relation": {}, "relation_version_is_last": true, "title": ["眼球運動失行と低アルブミン血症を伴う早発型失調症(EAOH)の臨床病型と分子病態メカニズムの解明"], "weko_shared_id": null}