The battle of the fold: chaperones take on prions.
The battle of the fold: chaperones take on prions.
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DOI：
10.1016/j.tig.2005.12.004
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发表时间：
2006-02
期刊：
影响因子：
--
通讯作者：
H. True
中科院分区：
文献类型：
--
作者：
H. True
蛋白质构象疾病，如阿尔茨海默氏症、帕金森氏症和亨廷顿氏症，影响着我们大部分的老龄人口。细胞已经进化出拯救和回收错误折叠蛋白质的机制，但这些系统并不完美。分子伴侣可以通过破坏聚集体并协助重折叠过程来拯救错误折叠的蛋白质。不能通过重折叠拯救的蛋白质可以通过分子伴侣递送到蛋白酶体以进行回收。一类“错误折叠”的蛋白质，朊病毒，似乎逃避这种机制的检测，并坚持在错误折叠的状态。事实上，朊病毒似乎篡夺了重折叠机制，实际上利用了伴侣来传播朊病毒状态。最近的数据已经开始揭示这种独特关系背后的机制。
Protein conformational diseases, such as Alzheimer's, Parkinson's and Huntington's, affect a large portion of our aging population. Cells have evolved mechanisms for rescuing and recycling misfolded proteins, but these systems are not perfect. Chaperones can rescue misfolded proteins by breaking up aggregates and assisting in the refolding process. Proteins that cannot be rescued by refolding can be delivered to the proteasome by chaperones to be recycled. One class of ‘misfolded' proteins, prions, appears to evade detection by this machinery and persist in a misfolded state. In fact, it seems that the prions usurp the refolding machinery and actually employ chaperones to propagate the prion state. Recent data has begun to uncover the mechanism behind this unique relationship.