Research NewsNeurobiology

Ironing Out the Wrinkles in the Prion Strain Problem

Science  20 Dec 1996:
Vol. 274, Issue 5295, pp. 2010
DOI: 10.1126/science.274.5295.2010


One of the biggest stumbling blocks to accepting the prion hypothesis—the notion that naked protein particles can cause neurodegenerative diseases like Creutzfeldt-Jakob disease of humans and mad cow disease—has been the existence of multiple prion strains with different, and apparently inheritable, characteristics. New work, reported on page 2079, now provides compelling evidence for the idea that the different strains consist of the same protein misfolded in different ways, with each able to impose its brand of misfolding on the normal prion protein by simple contact, thereby transmitting disease with unique strain characteristics. (See also the Special News Report, “Putting Prions to the Test,” Science, 12 July 1996, p. 184.)