Infection and intracellular dynamics of prions

Takeshi Yamasaki, Motohiro Horiuchi

Laboratory of Veterinary Hygiene, Graduate School of Veterinary Medicine, Hokkaido University

In prion diseases, once prions appear in the central nervous system, prions continuously propagate and spread cell to cell and finally cause neurodegeneration. Clarifying of the mechanism how prions infect neurons and propagate in cells is important to understand pathobiology of prion diseases. In 1990s, cellular mechanism of prion propagation was mainly investigated with cell biochemical techniques by the detection of abnormal isoform of prion protein (PrP^Sc), a major component of prions, as an indicator of the presence of prions. Based on the findings provided by earlier studies, after middle 2000s, new findings on the cellular mechanism of prion propagation have been accumulating due to the improvement of the immunocytochemical detection of PrP^Sc as well as molecular and cell biological techniques. Here we review the mechanisms of prion infection and propagation in cells as well as cell-to-cell transmission of prions by including recent achievements.

Address correspondence to Dr. Motohiro Horiuchi, Laboratory of Veterinary Hygiene, Graduate School of Veterinary Medicine, Hokkaido University (Kita-18, Nishi-9, Kita-ku, Sapporo 060-0818, Japan)