肽基脯氨酰异构酶1缺失在朊病毒疾病动物模型中的作用。
Effects of peptidyl-prolyl isomerase 1 depletion in animal models of prion diseases.
作者信息
Legname Giuseppe, Virgilio Tommaso, Bistaffa Edoardo, De Luca Chiara Maria Giulia, Catania Marcella, Zago Paola, Isopi Elisa, Campagnani Ilaria, Tagliavini Fabrizio, Giaccone Giorgio, Moda Fabio
机构信息
a Laboratory of Prion Biology, Department of Neuroscience , Scuola Internazionale Superiore di Studi Avanzati (SISSA) , Trieste , Italy.
c ELETTRA Laboratory , Sincrotrone Trieste S.C.p.A , Basovizza, Trieste , Italy.
出版信息
Prion. 2018 Mar 4;12(2):127-137. doi: 10.1080/19336896.2018.1464367. Epub 2018 May 18.
Pin1 is a peptidyl-prolyl isomerase that induces the cis-trans conversion of specific Ser/Thr-Pro peptide bonds in phosphorylated proteins, leading to conformational changes through which Pin1 regulates protein stability and activity. Since down-regulation of Pin1 has been described in several neurodegenerative disorders, including Alzheimer's Disease (AD), Parkinson's Disease (PD) and Huntington's Disease (HD), we investigated its potential role in prion diseases. Animals generated on wild-type (Pin1), hemizygous (Pin1) or knock-out (Pin1) background for Pin1 were experimentally infected with RML prions. The study indicates that, neither the total depletion nor reduced levels of Pin1 significantly altered the clinical and neuropathological features of the disease.
Pin1是一种肽基脯氨酰异构酶，可诱导磷酸化蛋白中特定丝氨酸/苏氨酸-脯氨酸肽键的顺反异构化，从而导致构象变化，Pin1通过这种构象变化调节蛋白稳定性和活性。由于在包括阿尔茨海默病（AD）、帕金森病（PD）和亨廷顿病（HD）在内的多种神经退行性疾病中均发现Pin1表达下调，我们研究了其在朊病毒疾病中的潜在作用。分别在野生型（Pin1）、半合子（Pin1）或Pin1基因敲除（Pin1）背景下培育的动物，经实验感染RML朊病毒。研究表明，Pin1的完全缺失或水平降低均未显著改变该疾病的临床和神经病理学特征。
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