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N-Terminal Regions of Prion Protein : Functions and Roles in Prion Diseases
https://tokushima-u.repo.nii.ac.jp/records/2008558
https://tokushima-u.repo.nii.ac.jp/records/2008558cea2a84d-b4bd-4e06-8c0e-29e4f4ef3dd7
名前 / ファイル | ライセンス | アクション |
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Item type | 文献 / Documents(1) | |||||||||||||||||
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公開日 | 2021-02-16 | |||||||||||||||||
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アクセス権 | open access | |||||||||||||||||
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資源タイプ識別子 | http://purl.org/coar/resource_type/c_6501 | |||||||||||||||||
資源タイプ | journal article | |||||||||||||||||
出版社版DOI | ||||||||||||||||||
関連識別子 | https://doi.org/10.3390/ijms21176233 | |||||||||||||||||
関連名称 | 10.3390/ijms21176233 | |||||||||||||||||
出版タイプ | ||||||||||||||||||
出版タイプ | VoR | |||||||||||||||||
出版タイプResource | http://purl.org/coar/version/c_970fb48d4fbd8a85 | |||||||||||||||||
タイトル | ||||||||||||||||||
タイトル | N-Terminal Regions of Prion Protein : Functions and Roles in Prion Diseases | |||||||||||||||||
著者 |
原, 英之
× 原, 英之
WEKO
557
× 坂口, 末廣
WEKO
220
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内容記述 | The normal cellular isoform of prion protein, designated PrPC, is constitutively converted to the abnormally folded, amyloidogenic isoform, PrPSc, in prion diseases, which include Creutzfeldt-Jakob disease in humans and scrapie and bovine spongiform encephalopathy in animals. PrPC is a membrane glycoprotein consisting of the non-structural N-terminal domain and the globular C-terminal domain. During conversion of PrPC to PrPSc, its 2/3 C-terminal region undergoes marked structural changes, forming a protease-resistant structure. In contrast, the N-terminal region remains protease-sensitive in PrPSc. Reverse genetic studies using reconstituted PrPC-knockout mice with various mutant PrP molecules have revealed that the N-terminal domain has an important role in the normal function of PrPC and the conversion of PrPC to PrPSc. The N-terminal domain includes various characteristic regions, such as the positively charged residue-rich polybasic region, the octapeptide repeat (OR) region consisting of five repeats of an octapeptide sequence, and the post-OR region with another positively charged residue-rich polybasic region followed by a stretch of hydrophobic residues. We discuss the normal functions of PrPC, the conversion of PrPC to PrPSc, and the neurotoxicity of PrPSc by focusing on the roles of the N-terminal regions in these topics. | |||||||||||||||||
キーワード | ||||||||||||||||||
主題 | prion protein | |||||||||||||||||
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主題 | prion | |||||||||||||||||
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主題 | prion disease | |||||||||||||||||
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主題 | neurodegeneration | |||||||||||||||||
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主題 | protein conformation | |||||||||||||||||
書誌情報 |
en : International Journal of Molecular Sciences 巻 21, 号 17, p. 6233, 発行日 2020-08-28 |
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収録物識別子タイプ | ISSN | |||||||||||||||||
収録物識別子 | 14220067 | |||||||||||||||||
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収録物識別子タイプ | ISSN | |||||||||||||||||
収録物識別子 | 16616596 | |||||||||||||||||
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収録物識別子タイプ | NCID | |||||||||||||||||
収録物識別子 | AA12038549 | |||||||||||||||||
出版者 | ||||||||||||||||||
出版者 | MDPI | |||||||||||||||||
権利情報 | ||||||||||||||||||
権利情報 | © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). | |||||||||||||||||
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識別子 | 372282 | |||||||||||||||||
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言語 | eng |