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  1. C100 医学部/医学系研究科
  2. C100a 雑誌掲載論文
  3. 学術雑誌

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/2237/00029275
77ee7731-3e14-402a-aa5a-4dab0205edd2
名前 / ファイル ライセンス アクション
amyloid_fibrils.pdf amyloid_fibrils (4.6 MB)
Item type 学術雑誌論文 / Journal Article(1)
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

WEKO 88772

en Koike, Haruki

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Nishi, Ryoji

× Nishi, Ryoji

WEKO 88773

en Nishi, Ryoji

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Ikeda, Shohei

× Ikeda, Shohei

WEKO 88774

en Ikeda, Shohei

Search repository
Kawagashira, Yuichi

× Kawagashira, Yuichi

WEKO 88775

en Kawagashira, Yuichi

Search repository
Iijima, Masahiro

× Iijima, Masahiro

WEKO 88776

en Iijima, Masahiro

Search repository
Sakurai, Takeo

× Sakurai, Takeo

WEKO 88777

en Sakurai, Takeo

Search repository
Shimohata, Takayoshi

× Shimohata, Takayoshi

WEKO 88778

en Shimohata, Takayoshi

Search repository
Katsuno, Masahisa

× Katsuno, Masahisa

WEKO 88779

en Katsuno, Masahisa

Search repository
Sobue, Gen

× Sobue, Gen

WEKO 88780

en Sobue, Gen

Search repository
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
キーワード
主題Scheme Other
主題 Electron microscopy
キーワード
主題Scheme Other
主題 Familial amyloid polyneuropathy
キーワード
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
キーワード
主題Scheme Other
主題 Toxicity
キーワード
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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