Prion-Protein Immunoreactivity in Human Transmissible Dementias

Abstract

To the Editor: Creutzfeldt–Jakob disease, kuru, and the Gerstmann–Sträussler syndrome are transmissible encephalopathies of humans caused by unusual slow infectious pathogens^{1 , 2} that have been labeled "prions" by one of us (S.B.P.).^{3 , 4} Scrapie of animals, a similar disease, has been intensively studied. Prions are composed in part, if not entirely, of a protease-resistant membrane protein that can polymerize into amyloid filaments.⁵ Collections of these filaments within the extracellular space of the brain form plaques that stain with Congo red dye and exhibit green–gold birefringence.^{6 , 7} These findings stimulated renewed interest with respect to the possible role of scrapie prions or similar molecules . . .