{"created":"2023-05-15T13:45:08.845798+00:00","id":34,"links":{},"metadata":{"_buckets":{"deposit":"17dba0f4-f26a-4b9b-ba27-50fdf6c247b7"},"_deposit":{"created_by":4,"id":"34","owners":[4],"pid":{"revision_id":0,"type":"depid","value":"34"},"status":"published"},"_oai":{"id":"oai:hirosakiuhw.repo.nii.ac.jp:00000034","sets":["5:6:9"]},"author_link":["185","186","184"],"item_3_alternative_title_21":{"attribute_name":"その他のタイトル","attribute_value_mlt":[{"subitem_alternative_title":"ラフォラ病の脳病理：形成途上期ラフォラ小体の微細構造変化"}]},"item_3_alternative_title_22":{"attribute_name":"タイトル（ヨミ）","attribute_value_mlt":[{"subitem_alternative_title":"ラフォラ ビョウ ノ ノウ ビョウリ ケイセイ トジョウキ ラフォラ ショウタイ ノ ビサイ コウゾウ ヘンカ"}]},"item_3_biblio_info_8":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2012-03-30","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"1","bibliographicPageEnd":"32","bibliographicPageStart":"19","bibliographicVolumeNumber":"3","bibliographic_titles":[{"bibliographic_title":"弘前医療福祉大学紀要"}]}]},"item_3_description_42":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"subitem_description":"Article"}]},"item_3_description_5":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"Lafora disease（LD）is an autosomal recessively inheritable neurodegeneration due to glycogen metabolic disorder, characterized pathologically by the presence of Lafora bodies（LB）（called polyglucosan bodies; malformed insoluble glucose polymers）in CNS neurons and cells in many other organs. Recently, it has become evident that about 90% of cases of LD are caused by mutations in\neither the EPM2A（laforin）or the EPM2B（malin）gene. Few previous EM studies have observed LB in the developing phase in detail. This study aimed at obtaining key findings that may reveal molecular events involved in the disease mechanisms underlying the morphology, by EM observations on LB in\nbrain tissue from a previously reported case of LD. The observations revealed that the two main components of LB, i.e. poorly branched, irregular fine filaments and amorphous small dense granules, were distributed sparsely in the LB in the early phase, whereas in the developing phase the fine filaments were innumerable together with a substantial number of dense granules and polysomes along with some lysosomes and no apparent autophagosomes. They intermingled and formed LB. In the developed phase, cored LB consisted of aggregates of dense granules forming the core and radially arranged filaments forming the outer rim. Recently, it has been reported that the lack of laforin-malin complexes causes the dysfunction of autophagy, which plays a primary role in the LB formation. The\nlack of Laforin-malin complexes and impaired autophagy for aggresome clearance were discussed. In conclusion, the fine structural changes of LB in the developing phase could be the key findings that link to the molecular mechanisms of not only LB but also LD.","subitem_description_type":"Abstract"}]},"item_3_description_7":{"attribute_name":"引用","attribute_value_mlt":[{"subitem_description":"弘前医療福祉大学紀要, 3(1), 2012, p.19-32","subitem_description_type":"Other"}]},"item_3_full_name_2":{"attribute_name":"著者(ヨミ)","attribute_value_mlt":[{"nameIdentifiers":[{}],"names":[{"name":"ヨシムラ, ノリアキ"}]}]},"item_3_full_name_3":{"attribute_name":"著者別名","attribute_value_mlt":[{"nameIdentifiers":[{}],"names":[{"name":"吉村, 教暤"}]}]},"item_3_publisher_35":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"弘前医療福祉大学紀要編集委員会"}]},"item_3_source_id_11":{"attribute_name":"書誌レコードID","attribute_value_mlt":[{"subitem_source_identifier":"AA12475995","subitem_source_identifier_type":"NCID"}]},"item_3_source_id_9":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"2185-0550","subitem_source_identifier_type":"ISSN"}]},"item_3_subject_19":{"attribute_name":"日本十進分類法","attribute_value_mlt":[{"subitem_subject":"491.6","subitem_subject_scheme":"NDC"}]},"item_3_subject_23":{"attribute_name":"NIIサブジェクト","attribute_value_mlt":[{"subitem_subject":"基礎医学","subitem_subject_scheme":"Other"}]},"item_3_text_4":{"attribute_name":"著者所属","attribute_value_mlt":[{"subitem_text_value":"Brain Pathology and Medical Neuroscience, Hirosaki University of Health and Welfare"},{"subitem_text_value":"弘前医療福祉大学 脳病理学・病態神経学"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"Yoshimura, Noriaki"}],"nameIdentifiers":[{}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2018-03-15"}],"displaytype":"detail","filename":"HirosakiIryoFukushi_3(1)_19.pdf","filesize":[{"value":"2.9 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"HirosakiIryoFukushi_3(1)_19.pdf","url":"https://hirosakiuhw.repo.nii.ac.jp/record/34/files/HirosakiIryoFukushi_3(1)_19.pdf"},"version_id":"1d7313c6-019f-4b79-bef0-aa7d2df0452b"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"Lafora bodies in the developing phase","subitem_subject_scheme":"Other"},{"subitem_subject":"fine structural changes","subitem_subject_scheme":"Other"},{"subitem_subject":"polyglucosan","subitem_subject_scheme":"Other"},{"subitem_subject":"aggresome and autophagy","subitem_subject_scheme":"Other"},{"subitem_subject":"laforin and malin","subitem_subject_scheme":"Other"},{"subitem_subject":"発達途上期LB","subitem_subject_scheme":"Other"},{"subitem_subject":"微細構造変化","subitem_subject_scheme":"Other"},{"subitem_subject":"ポリグルコサン","subitem_subject_scheme":"Other"},{"subitem_subject":"ラフォリンとマリン","subitem_subject_scheme":"Other"},{"subitem_subject":"アグリソームとオートファジー","subitem_subject_scheme":"Other"}]},"item_language":{"attribute_name":"言語","attribute_value_mlt":[{"subitem_language":"eng"}]},"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":"Brain pathology of Lafora disease : fine structural changes of Lafora (polyglucosan) bodies in the developing phase of the formation","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"Brain pathology of Lafora disease : fine structural changes of Lafora (polyglucosan) bodies in the developing phase of the formation"}]},"item_type_id":"3","owner":"4","path":["9"],"pubdate":{"attribute_name":"公開日","attribute_value":"2012-10-02"},"publish_date":"2012-10-02","publish_status":"0","recid":"34","relation_version_is_last":true,"title":["Brain pathology of Lafora disease : fine structural changes of Lafora (polyglucosan) bodies in the developing phase of the formation"],"weko_creator_id":"4","weko_shared_id":-1},"updated":"2023-05-15T17:03:59.247745+00:00"}