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The morphology of amyloid fibrils and their impact on tissue damage in hereditary transthyretin amyloidosis: An ultrastructural study
http://hdl.handle.net/2237/00029275
http://hdl.handle.net/2237/0002927577ee7731-3e14-402a-aa5a-4dab0205edd2
名前 / ファイル | ライセンス | アクション |
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amyloid_fibrils (4.6 MB)
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Item type | 学術雑誌論文 / Journal Article(1) | |||||
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公開日 | 2019-02-12 | |||||
タイトル | ||||||
タイトル | The morphology of amyloid fibrils and their impact on tissue damage in hereditary transthyretin amyloidosis: An ultrastructural study | |||||
言語 | en | |||||
著者 |
Koike, Haruki
× Koike, Haruki× Nishi, Ryoji× Ikeda, Shohei× Kawagashira, Yuichi× Iijima, Masahiro× Sakurai, Takeo× Shimohata, Takayoshi× Katsuno, Masahisa× Sobue, Gen |
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アクセス権 | ||||||
アクセス権 | open access | |||||
アクセス権URI | http://purl.org/coar/access_right/c_abf2 | |||||
権利 | ||||||
言語 | en | |||||
権利情報 | © 2018. This manuscript version is made available under the CC-BY-NC-ND 4.0 license http://creativecommons.org/licenses/by-nc-nd/4.0/ | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | CIDP | |||||
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主題Scheme | Other | |||||
主題 | Electron microscopy | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | Familial amyloid polyneuropathy | |||||
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主題Scheme | Other | |||||
主題 | Pathogenesis | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | Pathology | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | Schwann cell | |||||
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主題Scheme | Other | |||||
主題 | Toxicity | |||||
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主題Scheme | Other | |||||
主題 | Ultrastructure | |||||
抄録 | ||||||
内容記述 | Introduction: We evaluated the morphology of amyloid fibrils in the peripheral nervous system using biopsy or autopsy specimens from hereditary transthyretin amyloidosis patients. The impact of amyloid fibril formation on neighboring tissues was also investigated. Methods: Sural nerve biopsy specimens from 34 patients were examined using electron microscopy. Twenty-eight patients had Val30Met mutations, and the remaining 6 patients had non-Val30Met mutations (i.e., Glu54Lys, Pro24Ser, Thr49Ala, Val71Ala, Val94Gly, and Ala97Gly). The patients with the Val30Met mutation included a case from Brazil (supposedly of Portuguese origin), 6 early-onset cases from endemic foci in Japan, and 21 late-onset cases from non-endemic areas in Japan. Results: Long amyloid fibers were abundant in the early-onset Val30Met cases from the Japanese endemic foci and Brazil, whereas the amyloid fibrils were generally short in the late-onset Val30Met and non-Val30Met cases. The amyloid fibrils seemed to mature from dotty structures among amorphous electron-dense extracellular materials and pull surrounding tissues during the maturation process. The distortion of Schwann cells close to amyloid fibril masses was conspicuous, particularly in cases with long amyloid fibrils. Atrophy was conspicuous in non-myelinating Schwann cells and bands of Büngner (i.e., Schwann cell subunits that previously contained myelinated axons), particularly those completely surrounded by amyloid fibrils. In contrast, the myelinated fibers tended to be only partially surrounded by amyloid fibrils and morphologically preserved due to their large size. Only a few demyelinated axons were found. Conclusion: Pre-fibrillar amyloid precursors appear to play a pivotal role during the initial phase of amyloid fibril formation. The mechanical distortion and subsequent atrophy of Schwann cells resulting from the elongation of amyloid fibrils may be related to small-fiber predominant loss, which is a classical characteristic of amyloid neuropathy. Although rather rare, the destruction of myelin (i.e., demyelination) resulting from amyloid deposition may relate to nerve conduction abnormalities mimicking chronic inflammatory demyelinating polyneuropathy. | |||||
言語 | en | |||||
内容記述タイプ | Abstract | |||||
内容記述 | ||||||
内容記述 | ファイル公開:2019-11-15 | |||||
言語 | ja | |||||
内容記述タイプ | Other | |||||
出版者 | ||||||
言語 | en | |||||
出版者 | Elsevier | |||||
言語 | ||||||
言語 | eng | |||||
資源タイプ | ||||||
資源タイプresource | http://purl.org/coar/resource_type/c_6501 | |||||
タイプ | journal article | |||||
出版タイプ | ||||||
出版タイプ | AM | |||||
出版タイプResource | http://purl.org/coar/version/c_ab4af688f83e57aa | |||||
DOI | ||||||
関連タイプ | isVersionOf | |||||
識別子タイプ | DOI | |||||
関連識別子 | https://doi.org/10.1016/j.jns.2018.09.011 | |||||
ISSN(print) | ||||||
収録物識別子タイプ | PISSN | |||||
収録物識別子 | 0022-510X | |||||
書誌情報 |
en : Journal of the Neurological Sciences 巻 394, p. 99-106, 発行日 2018-11-15 |
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著者版フラグ | ||||||
値 | author |