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Brain pathology of Lafora disease : localization of polyglucosan aggregations (Lafora bodies) within neuronal networks of the cerebral cortex
https://hirosakiuhw.repo.nii.ac.jp/records/44
https://hirosakiuhw.repo.nii.ac.jp/records/442cb043d8-f78c-424a-bda1-3308a7ad1ab1
| 名前 / ファイル | ライセンス | アクション |
|---|---|---|
HirosakiIryoFukushi_4(1)_13.pdf (2.1 MB)
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| Item type | 紀要論文 / Departmental Bulletin Paper(1) | |||||||
|---|---|---|---|---|---|---|---|---|
| 公開日 | 2013-06-14 | |||||||
| タイトル | ||||||||
| タイトル | Brain pathology of Lafora disease : localization of polyglucosan aggregations (Lafora bodies) within neuronal networks of the cerebral cortex | |||||||
| 言語 | ||||||||
| 言語 | eng | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | Lafora bodies | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | localization within neuronal networks | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | synapses | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | myoclonus epilepsy | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | pathogenesis | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | ラフォラ小体（LB / PG B） | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | 神経回路網 | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | シナプス | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | ミオクローヌスてんかん | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | 発症機序 | |||||||
| 資源タイプ | ||||||||
| 資源タイプ識別子 | http://purl.org/coar/resource_type/c_6501 | |||||||
| 資源タイプ | departmental bulletin paper | |||||||
| 著者 |
Yoshimura, Noriaki
× Yoshimura, Noriaki
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| 著者(ヨミ) | ||||||||
| 姓名 | ヨシムラ, ノリアキ | |||||||
| 著者別名 | ||||||||
| 姓名 | 吉村, 教暤 | |||||||
| 著者所属 | ||||||||
| 弘前医療福祉大学 脳病理学・病態神経学 | ||||||||
| 著者所属 | ||||||||
| Brain Pathology and Medical Neuroscience, Hirosaki University of Health and Welfare | ||||||||
| 抄録 | ||||||||
| 内容記述タイプ | Abstract | |||||||
| 内容記述 | 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. | |||||||
| 引用 | ||||||||
| 内容記述タイプ | Other | |||||||
| 内容記述 | 弘前医療福祉大学紀要, 4(1), 2013, p.13-24 | |||||||
| 書誌情報 | 巻 4, 号 1, p. 13-24, 発行日 2013-03-29 | |||||||
| ISSN | ||||||||
| 収録物識別子タイプ | ISSN | |||||||
| 収録物識別子 | 2185-0550 | |||||||
| 書誌レコードID | ||||||||
| 収録物識別子タイプ | NCID | |||||||
| 収録物識別子 | AA12475995 | |||||||
| 日本十進分類法 | ||||||||
| 主題Scheme | NDC | |||||||
| 主題 | 493.7 | |||||||
| その他のタイトル | ||||||||
| その他のタイトル | 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 | |||||||
| タイトル（ヨミ） | ||||||||
| その他のタイトル | 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 | |||||||
| NIIサブジェクト | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | 基礎医学 | |||||||
| 出版者 | ||||||||
| 出版者 | 弘前医療福祉大学紀要編集委員会 | |||||||
| 資源タイプ | ||||||||
| 内容記述 | Article | |||||||
