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  1. 050 医学部, 大学院医学系研究科
  2. 0501 学術論文

The pathological and biochemical identification of possible seed-lesions of transmitted transthyretin amyloidosis after domino liver transplantation.

http://hdl.handle.net/10091/00020851
http://hdl.handle.net/10091/00020851
fd517769-2735-4d31-881b-ae7b43450ca9
名前 / ファイル ライセンス アクション
25461275_02.pdf 25461275_02.pdf (484.4 kB)
Item type 学術雑誌論文 / Journal Article(1)
公開日 2018-09-12
タイトル
タイトル The pathological and biochemical identification of possible seed-lesions of transmitted transthyretin amyloidosis after domino liver transplantation.
言語
言語 eng
DOI
関連識別子 https://doi.org/10.1002/cjp2.36
関連名称 10.1002/cjp2.36
キーワード
主題 domino liver transplantation, familial amyloid polyneuropathy, transmitted amyloidosis, transthyretin, seed-lesions, laser microdissection system
資源タイプ
資源 http://purl.org/coar/resource_type/c_6501
タイプ journal article
著者 Yoshinaga, Tsuneaki

× Yoshinaga, Tsuneaki

en Yoshinaga, Tsuneaki

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Yazaki, Masahide

× Yazaki, Masahide

en Yazaki, Masahide

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Sekijima, Yoshiki

× Sekijima, Yoshiki

en Sekijima, Yoshiki

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Kametani, Fuyuki

× Kametani, Fuyuki

en Kametani, Fuyuki

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Miyashita, Kana

× Miyashita, Kana

en Miyashita, Kana

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Hachiya, Naomi

× Hachiya, Naomi

en Hachiya, Naomi

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Tanaka, Tomohiro

× Tanaka, Tomohiro

en Tanaka, Tomohiro

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Kokudo, Norihiro

× Kokudo, Norihiro

en Kokudo, Norihiro

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Higuchi, Keiichi

× Higuchi, Keiichi

en Higuchi, Keiichi

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Ikeda, Shu‐ichi

× Ikeda, Shu‐ichi

en Ikeda, Shu‐ichi

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信州大学研究者総覧へのリンク
氏名 Yazaki, Masahide
URL http://soar-rd.shinshu-u.ac.jp/profile/ja.gpTauakh.html
出版者
出版者 Wiley Open Access
引用
内容記述 The Journal of Pathology.2(2):72-79(2015)
書誌情報 The Journal of Pathology

巻 2, 号 2, p. 72-79, 発行日 2015-12-28
抄録
内容記述 The most serious issue in domino liver transplantation (DLT) using liver grafts from patients with transthyretin (TTR)‐related familial amyloid polyneuropathy (FAP) is the development of iatrogenic transmitted amyloidosis (de novo amyloidosis) in DLT‐recipients. However, little is known regarding the mechanisms of the initial stage of amyloid formation in these recipients. We detected initial lesions (possible seed‐lesions) of this iatrogenic amyloidosis in two recipients following liver grafting from FAP patients. Patient 1 underwent DLT at age 65 from an FAP patient with a Val30Met TTR variant and patient 2 received DLT from an FAP patient with a Val30Leu TTR variant at age 32. Patient 2 was started on diflunisal administration from 4 years after DLT. While neither patient had symptoms of FAP, small amyloid deposits were detected on the gastroduodenal mucosae 14 months and 12 years after DLT in patient 1 and patient 2, respectively. The amyloid was analyzed using a laser microdissection system and tandem mass spectrometry. Biochemical analysis indicated that the amyloid was composed mostly of variant TTR produced from the transplanted liver in both patients. In patient 1, wild‐type TTR amyloid was detectable in the duodenal mucosa obtained 2 years after DLT. This is the first study to successfully capture the pathological and biochemical features of initial‐stage amyloid lesions in DLT recipients. The findings clearly indicate that amyloid deposition can start by deposition of variant TTR followed by deposition of wild‐type TTR, and blocking of amyloid seed formation from variant TTR may be a key to prevent or delay the development of DLT‐associated amyloidosis.
資源タイプ(コンテンツの種類)
ISSN
収録物識別子タイプ PISSN
収録物識別子 2056-4538
PubMed
識別子タイプ PMID
関連識別子 https://www.ncbi.nlm.nih.gov/pubmed/27499917
関連名称 27499917
権利
権利情報 © 2015 The Authors The Journal of Pathology: Clinical Research published by The Pathological Society of Great Britain and Ireland and John Wiley & Sons Ltd
出版タイプ
出版タイプ VoR
出版タイプResource http://purl.org/coar/version/c_970fb48d4fbd8a85
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