Conformational conversion of prion protein in prion diseases  

作　　者：Zheng Zhou Gengfu Xiao 

机构地区：[1]State Key Laboratory of Virology, Wuhan Institute of Virology, Chinese Academy of Sciences, Wuhan 430071, China

出　　处：《Acta Biochimica et Biophysica Sinica》2013年第6期465-476,共12页生物化学与生物物理学报（英文版）

基　　金：Funding This work was supported by the grants from the National Natural Science Foundation of China （No. 30970150 and No. 31170156）.

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.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.

关 键 词：prion disease prion protein conformationalconversion COFACTOR 

分 类 号：S852.659.7[农业科学—基础兽医学] O633.22[农业科学—兽医学]