Scrapie prion proteins are synthesized in neurons

Scrapie is a slow degenerative encephalopathy of animals caused by unusual infectious particles termed prions. A cDNA encoding the only apparent component of the prion, a protein designated PrP 27-30, has recently been cloned and sequenced. By measuring mRNA levels using in situ hybridization with the PrP cDNA, the authors found that prion proteins are synthesized almost exclusively within neurons. The levels of PrP mRNA varied among different types of neurons, but did not change during scrapie infection. A cDNA encoding glial fibrillary acidic protein (GFAP) was a positive control; GFAP mRNA was confined to astrocytes. Our finding of PrP mRNA in neurons may explain the degeneration and vacuolation that occurs in these cells during scrapie infection.