中文摘要：

Prion 疾病是一组传染致命的 neurodegenerative 疾病。进反常错误褶层 isoform (PrPSc ) 的细胞的 prion 蛋白质(PrPC ) 的 conformational 变换是在 prion 疾病病理的关键事件。在正常条件下面，高精力的障碍把 PrPC 与 PrPSc isoform 分开。然而，病原的变化，修正以及一些余因子例如 glycosaminoglycans， nucleic 酸，和类脂化合物，能调制 conformational 变换过程。理解 prion 蛋白质的 conformational 变换的机制为生物医学的研究和 prion 疾病的治疗是必要的。特别地，与 prion 蛋白质交往的余因子的描述可能提供新诊断、治疗学的策略。

英文摘要：

Prion diseases are a group of infectious fatal neurodegenerative diseases. The conformational conversion of a cellular prion protein （PrPc） into an abnormal misfolded isoform （PrPso） is the key event in prion diseases pathology. Under normal conditions, the high-energy barrier separates PrPc from PrPsc isoform. However, pathogenic mutations, modifications as well as some cofactors, such as glycosaminoglycans, nucleic acids, and lipids, could modulate the conformational conversion process. Understanding the mechanism of conformational conver- sion of prion protein is essential for the biomedical research and the treatment of prion diseases. Particularly, the characterization of cofactors interacting with prion protein might provide new diagnostic and therapeutic strategies.