Science and TechnologyCreutzfeldt-Jakob diseaseBreaking down the barrierA glimmer of hope for a drug that treats disease caused by prionsTHE epidemic of mad-cow disease in people that some forecast in the 1990s has not, fortunately, come to pass.But Creutzfeldt-Jakob disease (CJD), to give its proper name, is still a nasty illness that humanity would be better off without.It is also a strange illness.CJD and a handful of similar neurological conditions are caused by the misfolding of a particular protein that is found in the membranes of certain nerve cells.The strangeness is that the misfolded protein, known as a prion, somehow catalyses other molecules of the protein to misfold in the same way. The result is a chain reaction in which more and more protein ends up as prions.Nerve cells containing the prions stop working. The sufferer endures memory loss, personality changes and spontaneous, jerky bodily movements.Eventually, the disease kills him.A drug to treat CJD would therefore be welcome. And chemicals that seem either to prevent the misfolding, or to help the body clear away misfolded molecules, do, indeed, exist. The problem is turning at least one of those chemicals into an effective medicine.Adam Renslo of the University of California, San Francisco, and his colleagues (who include Stanley Prusiner, the Nobel laureate who discovered prions) have been trying to do so.
