Tethered prions

The spongiform encephalopathies, which include the human neurodegenerative diseases Creutzfeldt-Jakob disease, mad cow disease, and scrapie in sheep, are caused by an errant protein known as a prion. Prions can anchor themselves to cell membranes via a glycosylphosphatidylinositol, GPI, group and then transform normal healthy proteins into copies of themselves. Now, Christian Becker at the TU Munich, Germany, and Peter Seeberger at the ETH Zurich, Switzerland, and their colleagues have built a synthesized a GPI-anchored prion in the laboratory, which they say could help researchers model the activity of prions. Their anchoring technique will also be generally applicable to tethering other proteins involved in unrelated diseases.