Prion protein misfolding.
Prion protein misfolding.
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DOI：
10.2174/156652409789105543
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发表时间：
2009-09
影响因子：
2.5
通讯作者：
Groschup MH
中科院分区：
文献类型：
--
作者：
Kupfer L;Hinrichs W;Groschup MH
传染性海绵状脑病 (TSE) 发展的关键事件是宿主编码的膜蛋白（细胞 PrPC）构象变化为与疾病相关的原纤维形成同种型 PrPSc。从富含 α 螺旋的细胞形式到主要含有 β 折叠的细胞形式的构象转变启动了“自催化”反应，导致淀粉样原纤维在中枢神经系统 (CNS) 中积聚并导致神经变性，这是 TSE 的标志。导致构象变化的确切分子机制仍不清楚。多肽链如何采用至少两种稳定构象还有待阐明。本综述重点关注朊病毒蛋白在蛋白质-蛋白质相互作用和朊病毒蛋白错误折叠起始方面的结构方面。因此，它强调了蛋白质中可能在从 PrPC 到 PrPSc 的构象转变中发挥显著作用的部分，从而诱导蛋白质错误折叠的致命连锁反应。此外，将在某些情况下能够采取不溶性纤维状态的不同蛋白质的特征与 PrP 进行比较，试图了解朊病毒疾病的独特特征。
The crucial event in the development of transmissible spongiform encephalopathies (TSEs) is the conformational change of a host-encoded membrane protein - the cellular PrPC - into a disease associated, fibril-forming isoform PrPSc. This conformational transition from the α-helix-rich cellular form into the mainly β-sheet containing counterpart initiates an ‘autocatalytic’ reaction which leads to the accumulation of amyloid fibrils in the central nervous system (CNS) and to neurodegeneration, a hallmark of TSEs. The exact molecular mechanisms which lead to the conformational change are still unknown. It also remains to be brought to light how a polypeptide chain can adopt at least two stable conformations. This review focuses on structural aspects of the prion protein with regard to protein-protein interactions and the initiation of prion protein misfolding. It therefore highlights parts of the protein which might play a notable role in the conformational transition from PrPC to PrPSc and consequently in inducing a fatal chain reaction of protein misfolding. Furthermore, features of different proteins, which are able to adopt insoluble fibrillar states under certain circumstances, are compared to PrP in an attempt to understand the unique characteristics of prion diseases.
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DOI：
10.1073/pnas.94.10.4931
发表时间：
1997-05-13
影响因子：
11.1
作者：
Bossers, A;Belt, PBGM;Smits, MA
通讯作者：
Smits, MA
影响因子：
3.8
作者：
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通讯作者：
Torres, Juan M.
影响因子：
16.2
作者：
DeArmond, SJ;Sánchez, H;Prusiner, SB
通讯作者：
Prusiner, SB
影响因子：
4.8
作者：
Baskakov, IV;Legname, G;Cohen, FE
通讯作者：
Cohen, FE
影响因子：
64.8
作者：
Brown, DR;Schmidt, B;Kretzschmar, HA
通讯作者：
Kretzschmar, HA