Article
Conformational conversion of prion protein in prion diseases.
Acta biochimica et biophysica Sinica - 1 Jun 2013
Zhou Zheng, Xiao Gengfu
Abstract excerpt
Prion diseases are a group of infectious fatal neurodegenerative diseases. The conformational conversion of a cellular prion protein (PrP(C)) into an abnormal misfolded isoform (PrP(Sc)) is the key event in prion diseases pathology. Under normal conditions, the high-energy barrier separates PrP(C) from PrP(Sc) isoform. However, pathogenic mutations, modifications as well as some cofactors, such as...
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