Prion-Like Characteristics of Polyglutamine-Containing Proteins.
Prion-Like Characteristics of Polyglutamine-Containing Proteins.
复制标题
DOI：
10.1101/cshperspect.a024257
复制
发表时间：
2018-02-01
影响因子：
5.4
通讯作者：
Kopito RR
中科院分区：
文献类型：
--
作者：
Pearce MMP;Kopito RR
传染性海绵状脑病是由朊病毒蛋白（PrP）转化为自我复制构象引起的传染性神经退行性疾病，该自我复制构象通过天然折叠的PrP分子在细胞内或细胞间的模板转化而传播。最近的研究提供了令人信服的证据，朊病毒样行为是与神经退行性疾病相关的大多数蛋白质聚集体的一般性质。这些疾病中的许多与自发性蛋白质聚集有关，但基因突变可以增加特定蛋白质的聚集倾向，包括聚谷氨酰胺（polyQ）束的扩张，这是9种遗传性神经退行性疾病的病因。在亨廷顿病中由polyQ扩增的亨廷顿蛋白（Htt）形成的聚集体可以在细胞之间转移，并以朊病毒样方式接种细胞质野生型Htt的聚集。此外，朊病毒样特性的谷氨酰胺丰富的蛋白质的基础非病理过程在酵母和高等真核生物。在这里，我们回顾目前的证据支持朊病毒样特征的polyQ和谷氨酰胺丰富的蛋白质。
Transmissible spongiform encephalopathies are infectious neurodegenerative diseases caused by the conversion of prion protein (PrP) into a self-replicating conformation that spreads via templated conversion of natively folded PrP molecules within or between cells. Recent studies provide compelling evidence that prion-like behavior is a general property of most protein aggregates associated with neurodegenerative diseases. Many of these disorders are associated with spontaneous protein aggregation, but genetic mutations can increase the aggregation propensity of specific proteins, including expansion of polyglutamine (polyQ) tracts, which is causative of nine inherited neurodegenerative diseases. Aggregates formed by polyQ-expanded huntingtin (Htt) in Huntington’s disease can transfer between cells and seed the aggregation of cytoplasmic wild-type Htt in a prion-like manner. Additionally, prion-like properties of glutamine-rich proteins underlie nonpathological processes in yeast and higher eukaryotes. Here, we review current evidence supporting prion-like characteristics of polyQ and glutamine-rich proteins.
登录
查看更多内容
影响因子：
64.8
作者：
ALPER, T;CRAMP, WA;CLARKE, MC
通讯作者：
CLARKE, MC
影响因子：
14.8
作者：
Barmada, Sami J.;Serio, Andrea;Arjun, Arpana;Bilican, Bilada;Daub, Aaron;Ando, D. Michael;Tsvetkov, Andrey;Pleiss, Michael;Li, Xingli;Peisach, Daniel;Shaw, Christopher;Chandran, Siddharthan;Finkbeiner, Steven
通讯作者：
Finkbeiner, Steven
影响因子：
16.2
作者：
de Calignon A;Polydoro M;Suárez-Calvet M;William C;Adamowicz DH;Kopeikina KJ;Pitstick R;Sahara N;Ashe KH;Carlson GA;Spires-Jones TL;Hyman BT
通讯作者：
Hyman BT
影响因子：
64.8
作者：
Bennett, Eric J.;Shaler, Thomas A.;Kopito, Ron R.
通讯作者：
Kopito, Ron R.
影响因子：
2.9
作者：
Chen, SM;Berthelier, V;Wetzel, R
通讯作者：
Wetzel, R