Motoneuron degeneration in the trigeminal motor nucleus innervating the masseter muscle in dystonia musculorum mice

• 資料種別: 博士論文
• タイトル: Motoneuron degeneration in the trigeminal motor nucleus innervating the masseter muscle in dystonia musculorum mice
• 著者・編者: Hossain, Md Ibrahim
• 出版事項: 新潟大学
• 出版年月日等: 2018-03-23
• 出版年（W3CDTF）: 2018-03-23
• 並列タイトル等: Motoneuron degeneration in the trigeminal motor nucleus innervating the masseter muscle in dystonia musculorum mice; ジストニアマスキュローラムマウスの咀嚼筋を支配する三叉神経運動核における運動ニューロン変性
• 授与機関名: 新潟大学
• 授与年月日: 2018-03-23
• 授与年月日（W3CDTF）: 2018-03-23
• 報告番号: 甲第4422号
• 学位: 博士（医学）
• 博論授与番号: 甲第4422号
• 本文の言語コード: eng
• 件名標目: dystonia musculorum; trigeminal motor nucleus (Mo5); motor neuron; neurodegeneration; mandibular nerve; masseter muscle
• 一般注記: Dystonia musculorum (dt) mice, which have a mutation in the Dystonin (Dst) gene, are used as animal models to investigate the human disease known as hereditary sensory and autonomic neuropathy type VI. Massive neuronal cell death is observed, mainly in the peripheral nervous system (PNS) of dt mice. We and others have recently reported a histopathological feature of these mice that neurofilament (NF) accumulates in various areas of the central nervous system (CNS), including motor pathways. Although dt mice show motor disorder and growth retardation, the causes for these are still unknown. Here we performed histopathological analyses on motor units of the trigeminal motor nucleus (Mo5 nucleus), because they are a good system to understand neuronal responses in the mutant CNS, and abnormalities in this system may lead to problems in mastication, with subsequent growth retardation. We report that motoneurons with NF accumulation in the Mo5 nuclei of Dst^(Gt) homozygous mice express the stress-induced genes CHOP, ATF3, and lipocalin 2 (Lcn2). We also show a reduced number of Mo5 motoneurons and a reduced size of Mo5 nuclei in Dst^(Gt) homozygous mice, possibly due to apoptosis, given the presence of cleaved caspase 3-positive Mo5 motoneurons. In the mandibular (V3) branches of the trigeminal nerve, which contains axons of Mo5 motoneurons and trigeminal sensory neurons, there was infiltration of Iba1-positive macrophages. Finally, we report atrophy of the masseter muscles in Dst^(Gt) homozygous mice, which showed abnormal nuclear localization of myofibrils and increased expression of atrogin-1 mRNA, a muscle atrophy-related gene and weaker masseter muscle strength with uncontrolled muscle activity by electromyography (EMG). Taken together, our findings strongly suggest that mastication in dt mice is affected due to abnormalities of Mo5 motoneurons and masseter muscles, leading to growth retardation at the post-weaning stages.; 学位の種類: 博士（医学）. 報告番号: 甲第4422号. 学位記番号: 新大院博（医）甲第821号. 学位授与年月日: 平成30年3月23日; Neurochemistry International. Article first published online: 21 October 2017.; 新大院博（医）甲第821号; 開始ページ : 1; 終了ページ : 26
• DOI: info:doi/10.1016/j.neuint.2017.10.009; https://doi.org/info:doi/10.1016/j.neuint.2017.10.009
• 国立国会図書館永続的識別子: info:ndljp/pid/11216986; https://dl.ndl.go.jp/pid/11216986
• コレクション（共通）: 障害者向け資料
• コレクション（障害者向け資料：レベル1）: テキストデータ
• コレクション（個別）: 国立国会図書館デジタルコレクション > デジタル化資料 > 博士論文; https://dl.ndl.go.jp/collections/A00014
• 収集根拠: 博士論文（自動収集）
• 受理日（W3CDTF）: 2019-01-04T08:16:43+09:00
• 作成日（W3CDTF）: 2019-08-05
• 記録形式（IMT）: application/pdf
• オンライン閲覧公開範囲: 国立国会図書館内限定公開
• デジタル化資料送信: 図書館・個人送信対象外
• 遠隔複写可否（NDL）: 可
• 掲載誌（URI）: http://hdl.handle.net/10191/50460
• 連携機関・データベース: 国立国会図書館 : 国立国会図書館デジタルコレクション; https://dl.ndl.go.jp