Li Sha, Ju Chuanjing, Han Chao, Li Zhongyi, Liu Wensen, Ye Xuemin, Xu Jing, Xulong Liang, Wang Xiong, Chen Zhibao, Meng Keyin, Wan Jiayu
a Institute of Military Veterinary; Academy of Military Medical Science ; Changchun , China.
Prion. 2014;8(5):339-43. doi: 10.4161/19336896.2014.971574.
Previous studies have demonstrated that Shadoo (Sho), a GPI-linked glycoprotein encoded by the Sprn gene with a membrane localization similar to PrP(C), is reduced in the brains of rodents with terminal prion disease. To determine the functional significance of Sho in prion disease pathogenesis, Sho-deficient mice were generated by gene targeting. Sho knockout and control wild-type (WT) mice were infected with themouse-adapted scrapie strains 22L or RML. No significant differences in survival, the incubation period of prion disease or other disease features were observed between Sho mutant and WT mice. In this model of prion disease, Sho removal had no effect on disease pathogenesis.
先前的研究表明,Shadoo(Sho)是一种由Sprn基因编码的糖基磷脂酰肌醇(GPI)连接糖蛋白,其膜定位与朊病毒蛋白(PrP(C))相似,在患有晚期朊病毒病的啮齿动物大脑中含量降低。为了确定Sho在朊病毒病发病机制中的功能意义,通过基因靶向技术构建了Sho基因缺陷小鼠。将Sho基因敲除小鼠和对照野生型(WT)小鼠接种适应小鼠的瘙痒病毒株22L或RML。在Sho基因缺陷小鼠和WT小鼠之间,未观察到生存、朊病毒病潜伏期或其他疾病特征的显著差异。在这个朊病毒病模型中,去除Sho对疾病发病机制没有影响。