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Molecular biology of prion protein and its first homologous protein
https://tokushima-u.repo.nii.ac.jp/records/2004695
https://tokushima-u.repo.nii.ac.jp/records/2004695d432e701-09a1-431c-b1bd-e6112498e1f1
| 名前 / ファイル | ライセンス | アクション |
|---|---|---|
jmi_54_3-4_211.pdf (712 KB)
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| Item type | 文献 / Documents(1) | |||||||||||
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| 公開日 | 2018-06-06 | |||||||||||
| アクセス権 | ||||||||||||
| アクセス権 | open access | |||||||||||
| 資源タイプ | ||||||||||||
| 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/10.2152/jmi.54.211 | |||||||||||
| 関連名称 | 10.2152/jmi.54.211 | |||||||||||
| 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 | Molecular biology of prion protein and its first homologous protein | |||||||||||
| 著者 |
坂口, 末廣
× 坂口, 末廣
WEKO
220
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| 内容記述 | Conformational conversion of the normal cellular isoform of prion protein, PrPC, a glycoprotein anchored to the cell membrane by a glycosylphosphatidylinositol moiety, into the abnormally folded, amyloidogenic prion protein, PrPSc, plays a pivotal role in the pathogenesis of prion diseases. It has been suggested that PrPC might be functionally disturbed by constitutive conversion to PrPSc due to either the resulting depletion of PrPC or the dominant negative effects of PrPSc on PrPC or both. Consistent with this, we and others showed that mice devoid of PrPC (PrP-/-) spontaneously developed abnormal phenotypes very similar to the neurological abnormalities of prion diseases, supporting the concept that functional loss of PrPC might at least be partly involved in the pathogenesis of the diseases. However, no neuronal cell death could be detected in PrP-/- mice, indicating that the functional loss of PrPC alone might not be enough to induce neuronal cell death, one of major pathological hallmarks of prion diseases. Interestingly, it was recently shown that the first identified PrP-like protein, termed PrPLP/Doppel (Dpl), is neurotoxic in the absence of PrPC, causing Purkinje cell degeneration in the cerebellum of mice. Although it is not understood if PrPSc could have a neurotoxic potential similar to PrPLP/ Dpl, it is very interesting to speculate that accumulation of PrPSc and the functional disturbance of PrPC, both of which are caused by constitutive conversion, might be required for the neurodegeneration in prion diseases. | |||||||||||
| キーワード | ||||||||||||
| 主題 | prion | |||||||||||
| キーワード | ||||||||||||
| 主題 | prion protein | |||||||||||
| キーワード | ||||||||||||
| 主題 | prion protein-like protein | |||||||||||
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| 主題 | knockout mice | |||||||||||
| キーワード | ||||||||||||
| 主題 | neurodegeneration | |||||||||||
| 書誌情報 |
en : The Journal of Medical Investigation 巻 54, 号 3-4, p. 211-223, 発行日 2007-08 |
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| 収録物ID | ||||||||||||
| 収録物識別子タイプ | ISSN | |||||||||||
| 収録物識別子 | 13496867 | |||||||||||
| 収録物ID | ||||||||||||
| 収録物識別子タイプ | ISSN | |||||||||||
| 収録物識別子 | 13431420 | |||||||||||
| 収録物ID | ||||||||||||
| 収録物識別子タイプ | NCID | |||||||||||
| 収録物識別子 | AA11166929 | |||||||||||
| 収録物ID | ||||||||||||
| 収録物識別子タイプ | NCID | |||||||||||
| 収録物識別子 | AA12022913 | |||||||||||
| 出版者 | ||||||||||||
| 出版者 | Faculty of Medicine Tokushima University | |||||||||||
| EID | ||||||||||||
| 識別子 | 171919 | |||||||||||
| 言語 | ||||||||||||
| 言語 | eng | |||||||||||
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Cite as
坂口, 末廣, 2007, Molecular biology of prion protein and its first homologous protein: Faculty of Medicine Tokushima University, 211–223 p.
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