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Exploration of the main sites for the transformation of normal prion protein (PrPC) into pathogenic prion protein (PrPsc)

机译:将正常朊病毒蛋白(PRPC)转化为致病朊病毒蛋白(PRPSC)的主要部位探索

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摘要

Introduction: The functions and mechanisms of prion proteins (PrPC) are currently unknown, but most experts believe that deformed or pathogenic prion proteins (PrPSc) originate from PrPC, and that there may be plural main sites for the conversion of normal PrPC into PrPSc. In order to better understand the mechanism of PrPC transformation to PrPSc, the most important step is to determine the replacement or substitution site. Material and Methods: BALB/c mice were challenged with prion RML strain and from 90 days post-challenge (dpc) mice were sacrificed weekly until all of them had been at 160 dpc. The ultra-structure and pathological changes of the brain of experimental mice were observed and recorded by transmission electron microscopy. Results: There were a large number of pathogen-like particles aggregated in the myelin sheath of the brain nerves, followed by delamination, hyperplasia, swelling, disintegration, phagocytic vacuolation, and other pathological lesions in the myelin sheath. The aggregated particles did not overflow from the myelin in unstained samples. The phenomenon of particle aggregation persisted all through the disease course, and was the earliest observed pathological change. Conclusion: It was deduced that the myelin sheath and lipid rafts in brain nerves, including axons and dendrites, were the main sites for the conversion of PrPC to PrPSc, and the PrPSc should be formed directly by the conversion of protein conformation without the involvement of nucleic acids.
机译:简介:朊病毒蛋白(PRPC)的功能和机制目前未知,但大多数专家认为,变形或致病朊病毒蛋白(PRPSC)源自PRPC,并且可能有多个主要位点用于将正常的PRPC转化为PRPSC。为了更好地了解PRPC转换的机制,最重要的步骤是确定替代或替代网站。材料和方法:BALB / C小鼠用朊病毒rML菌株攻击,每周攻击后90天,直到所有人都处于160 DPC之前。通过透射电子显微镜观察和记录实验小鼠的大脑的超结构和病理变化。结果:在脑神经中髓鞘中聚集了大量的病原体颗粒,其次是分层,增生,溶胀,崩解,骨髓鞘中的其他病理病变。聚集的颗粒在未染色的样品中没有从髓鞘溢出。颗粒聚集现象持续存在于疾病过程中,并且是最早观察到的病理变化。结论:推导出肌素护套和脑神经(包括轴突和树突)的脂质筏是PRPC转化为PRPSC的主要部位,PRPSC应直接通过蛋白质构象转化而不是参与的转化形成核酸。

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  • 来源
    《Journal of Veterinary Research》 |2017年第1期|共12页
  • 作者单位

    Jilin Univ Inst Zoonosis Key Lab Zoonosis Res China Japan Union Hosp Minist Educ Changchun 130062 Peoples R China;

    Jilin Univ Inst Zoonosis Key Lab Zoonosis Res China Japan Union Hosp Minist Educ Changchun 130062 Peoples R China;

    Jilin Univ Inst Zoonosis Key Lab Zoonosis Res China Japan Union Hosp Minist Educ Changchun 130062 Peoples R China;

    Jilin Univ Inst Zoonosis Key Lab Zoonosis Res China Japan Union Hosp Minist Educ Changchun 130062 Peoples R China;

    Jilin Univ Inst Zoonosis Key Lab Zoonosis Res China Japan Union Hosp Minist Educ Changchun 130062 Peoples R China;

    Jilin Univ Inst Zoonosis Key Lab Zoonosis Res China Japan Union Hosp Minist Educ Changchun 130062 Peoples R China;

    Jilin Univ Inst Zoonosis Key Lab Zoonosis Res China Japan Union Hosp Minist Educ Changchun 130062 Peoples R China;

    Jilin Univ Inst Zoonosis Key Lab Zoonosis Res China Japan Union Hosp Minist Educ Changchun 130062 Peoples R China;

    Jilin Univ Inst Zoonosis Key Lab Zoonosis Res China Japan Union Hosp Minist Educ Changchun 130062 Peoples R China;

    Jilin Univ Inst Zoonosis Key Lab Zoonosis Res China Japan Union Hosp Minist Educ Changchun 130062 Peoples R China;

  • 收录信息
  • 原文格式 PDF
  • 正文语种 eng
  • 中图分类 动物医学(兽医学);
  • 关键词

    mice; brain; prion proteins; myelin sheath; particle aggregation;

    机译:小鼠;脑;朊病毒蛋白;髓鞘;粒子聚集;

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