中文摘要：

通过考查铜离子在朊病毒疾病发病过程中的作用，将有助于阐明朊病毒形成及发病机制以及重金属铜离子对朊病毒发病过程的影响，为进一步对朊病毒疾病的防治提供重要依据。实验运用采用转PrP基因线虫模型来研究朊蛋白与铜离子的相互作用。结果表明，线虫暴露于10^-3mol·L^-1铜离子浓度条件下，其对食物敏感性会下降，生命周期会减短，产卵数会减少；当线虫暴露于10^-4mol·L-1铜离子浓度条件下，会减少以上三种影响；当线虫暴露于10^-6mol·L-1铜离子浓度条件下，对线虫几乎没有影响。研究数据显示，在有高浓度铜离子影响情况下，转入朊蛋白102位点突变（dat-1-PrP102Mut）（w102）的线虫比转入朊蛋白（dat-1-PrPwt）（w101）的线虫损伤更严重，转入朊蛋白（W101）的线虫比野生型线虫（N2）损伤更严重。

英文摘要：

By giving deep insight into the impact of cupric ion in the proin disease course, and clarifing the nosogenesis of proin disease and more important to proin disease control. In the present study,we investigated the interaction between prion protein and different concentration Cu2＋ in PrP transgenic Caenorhabditis elegans （C. elegans ） model. The data showed that when C. elegans was exposed to 10-3 mol·L-1 Cu2＋, the food sensitivity, life cycle and laying of C. elegans were significantly descended respectively. However, the influences were dramatically attenuated when C. elegans was exposed to 10-4 mol＇L-1 Cu2~ and there was no change while there was the presence of 104 mol·L-1 Cu2＋,which indicated that Cu2＋ influence the prion protein in a concentration-dependent manner in the transgenic Caenorhabditis elegans model. Meanwhile, our research confirmed that when C. elegans was exposed to high concentration of Cu2＋, W101 （dat-l-PrPWt） which expressing PrP was damaged more severely than the wild type C. elegans N2. Similarly, the W102 （dat-l-PrP102Mut）expressing mutations in the 102 site of PrP was damaged more severely than W101. It implied the 102 site of PrP was involved with the interaction between Cu2＋ and prion protein.