{"created":"2023-05-15T13:45:09.485581+00:00","id":44,"links":{},"metadata":{"_buckets":{"deposit":"4d11965a-9242-465e-93f8-93a68c5d35d7"},"_deposit":{"created_by":4,"id":"44","owners":[4],"pid":{"revision_id":0,"type":"depid","value":"44"},"status":"published"},"_oai":{"id":"oai:hirosakiuhw.repo.nii.ac.jp:00000044","sets":["5:6:10"]},"author_link":["276","274","275"],"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 ： 大脳皮質神経回路網内におけるポリグルコサン凝集体（Lafora小体）の局在"}]},"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 ダイノウ ヒシツ シンケイ カイロモウ ナイ ニオケル ポリグルコサン ギョウシュウタイ Lafora ショウタイ ノ キョクザイ"}]},"item_3_biblio_info_8":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2013-03-29","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"1","bibliographicPageEnd":"24","bibliographicPageStart":"13","bibliographicVolumeNumber":"4","bibliographic_titles":[{}]}]},"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 metabolic disorder of carbohydrate, characterized clinically by myoclonus, epileptic convulsions and　mental deterioration, and pathologically by widespread presence of polyglucosan bodies (Lafora bodies; LB) in the CNS neurons and the cells of other organs. Although the histochemistry and ultrastructure of LB have been well reported, localization of LB within neuronal networks has not been well clarified yet. The purpose of the present study is to elucidate this issue. A male patient with LD who died at 30 years of age was autopsied. The autopsy case was reported elsewhere. This time, specimens taken from the frontal and temporal cortices were observed by electron microscopy. There were a number of synapses on the cell membrane which covered large LB within the perikaryon and dendritic shaft. LB were encountered at times in myelinated axons. Very small LB (ø <2 μm) were occasionally found in the presynaptic terminals of asymmetric synapses, but hardly found in the postsynaptic endings. It is known that disrupting the delicate balance of inhibitory and excitatory synaptic transmission can trigger the disordered, synchronous firing of neurons that underlies a seizure. The results of the present study strongly suggest that disruption of the delicate balance of such synaptic transmissions, which can trigger the disordered synchronous discharges of neurons, may well take place in the cerebral cortices in 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, 4(1), 2013, p.13-24","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":"493.7","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":"弘前医療福祉大学 脳病理学・病態神経学"},{"subitem_text_value":"Brain Pathology and Medical Neuroscience, Hirosaki University of Health and Welfare"}]},"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_4(1)_13.pdf","filesize":[{"value":"2.1 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"HirosakiIryoFukushi_4(1)_13.pdf","url":"https://hirosakiuhw.repo.nii.ac.jp/record/44/files/HirosakiIryoFukushi_4(1)_13.pdf"},"version_id":"67b5a827-f5b0-4a0d-8d41-7abc3e9a170c"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"Lafora bodies","subitem_subject_scheme":"Other"},{"subitem_subject":"localization within neuronal networks","subitem_subject_scheme":"Other"},{"subitem_subject":"synapses","subitem_subject_scheme":"Other"},{"subitem_subject":"myoclonus epilepsy","subitem_subject_scheme":"Other"},{"subitem_subject":"pathogenesis","subitem_subject_scheme":"Other"},{"subitem_subject":"ラフォラ小体（LB / PG B）","subitem_subject_scheme":"Other"},{"subitem_subject":"神経回路網","subitem_subject_scheme":"Other"},{"subitem_subject":"シナプス","subitem_subject_scheme":"Other"},{"subitem_subject":"ミオクローヌスてんかん","subitem_subject_scheme":"Other"},{"subitem_subject":"発症機序","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 : localization of polyglucosan aggregations (Lafora bodies) within neuronal networks of the cerebral cortex","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"Brain pathology of Lafora disease : localization of polyglucosan aggregations (Lafora bodies) within neuronal networks of the cerebral cortex"}]},"item_type_id":"3","owner":"4","path":["10"],"pubdate":{"attribute_name":"公開日","attribute_value":"2013-06-14"},"publish_date":"2013-06-14","publish_status":"0","recid":"44","relation_version_is_last":true,"title":["Brain pathology of Lafora disease : localization of polyglucosan aggregations (Lafora bodies) within neuronal networks of the cerebral cortex"],"weko_creator_id":"4","weko_shared_id":-1},"updated":"2023-05-15T14:25:20.912057+00:00"}