NR AYDL
AU Sakaguchi,S.
TI [Mechanisms of prion transmission]
QU Nippon Rinsho. Japanese Journal of Clinical Medicine 2007 Aug; 65(8): 1391-5
PT english abstract; journal article; review
AB Prions, the causative agents of prion diseases, consist of the abnormal isoform of prion protein, PrPsc. PrPsc is generated by conformational conversion of the normal isoform of prion protein, PrPc, a glycosyl-phosphatidyl-inositol-anchored glycoprotein abundantly expressed on the surface of neurons. Prions or PrPsc having invaded the body interact with PrPc and induce changes in structure of the interacting PrPc into that of PrPsc, leading to prion replication. At the same time, this constitutive conversion causes the detrimental accumulation of PrPsc in the brain tissue. Here, I will introduce the structural and biochemical properties of PrPc and PrPsc and discuss the nature of prions and the mechanisms of prion replication in more detail.
ZR 22
MH Animals; Brain/metabolism; Humans; PrPc Proteins/chemistry/metabolism; PrPsc Proteins/*metabolism/*pathogenicity; Prion Diseases/*etiology/*transmission
AD Division of Molecular Neurobiology, The Institute for Enzyme Research, The University of Tokushima.
SP japanisch
PO Japan