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Brain pathology of Lafora disease : fine structural changes of Lafora (polyglucosan) bodies in the developing phase of the formation
https://hirosakiuhw.repo.nii.ac.jp/records/34
https://hirosakiuhw.repo.nii.ac.jp/records/3480621751-3b1a-423a-bec4-dae7031b1644
| 名前 / ファイル | ライセンス | アクション |
|---|---|---|
HirosakiIryoFukushi_3(1)_19.pdf (2.9 MB)
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| Item type | 紀要論文 / Departmental Bulletin Paper(1) | |||||||
|---|---|---|---|---|---|---|---|---|
| 公開日 | 2012-10-02 | |||||||
| タイトル | ||||||||
| タイトル | Brain pathology of Lafora disease : fine structural changes of Lafora (polyglucosan) bodies in the developing phase of the formation | |||||||
| 言語 | ||||||||
| 言語 | eng | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | Lafora bodies in the developing phase | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | fine structural changes | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | polyglucosan | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | aggresome and autophagy | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | laforin and malin | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | 発達途上期LB | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | 微細構造変化 | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | ポリグルコサン | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | ラフォリンとマリン | |||||||
| キーワード | ||||||||
| 主題Scheme | Other | |||||||
| 主題 | アグリソームとオートファジー | |||||||
| 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 | ||||||||
| Brain Pathology and Medical Neuroscience, Hirosaki University of Health and Welfare | ||||||||
| 著者所属 | ||||||||
| 弘前医療福祉大学 脳病理学・病態神経学 | ||||||||
| 抄録 | ||||||||
| 内容記述タイプ | Abstract | |||||||
| 内容記述 | 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 either 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 brain 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 lack 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. |
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| 引用 | ||||||||
| 内容記述タイプ | Other | |||||||
| 内容記述 | 弘前医療福祉大学紀要, 3(1), 2012, p.19-32 | |||||||
| 書誌情報 |
弘前医療福祉大学紀要 巻 3, 号 1, p. 19-32, 発行日 2012-03-30 |
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| 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 | 491.6 | |||||||
| 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：形成途上期ラフォラ小体の微細構造変化 | |||||||
| 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 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 | |||||||
| 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 | |||||||
