Prion Trio

Three prion related research stories that have come out lately:

1/ Regulation of Embryonic Cell Adhesion by the Prion Protein in PLoS Biology this week.

Researchers in Germany have shown that the regular Prion protein (the one everyone has, that isn't infectious and doesn't cause problems) plays a beneficial role for the organism by helping cells communicate with one another during embryonic development. Even though we have known that a normal prion protein in the brain can turn harmful and cause deadly illnesses like Creutzfeldt-Jakob disease (CJD) in humans, and bovine spongiform encephalopathy (BSE) in cattle we did not, until this paper, know why large amounts of this normal protein are produced by our bodies in the first place.

2/ Cellular prion protein mediates impairment of synaptic plasticity by amyloid-beta oligomers in Nature a couple of weeks ago.

The regular prion protein may contribute to nerve damage if it becomes entangled with a protein fragment that scientists consider a chief suspect as a cause for Alzheimer's disease. The prion protein, if it is involved in Alzheimer's, is probably in its normal form. There's no evidence that the disease somehow releases infectious prions.

3/ Safety and efficacy of quinacrine in human prion disease (PRION-1 study): a patient preference trial in the April issue of Lancet Neurology.

The anti-malaria drug quinacrine does not appear to extend the lives of people with the human form of mad cow disease, despite encouraging results from experiments with mice. Currently there are no drugs that prevent or reverse the disease, though quinacrine has shown promise in treating prion-infected mouse cells because it can penetrate the blood-brain barrier and the drug has been effective in treating these prion-infected mouse cells by blocking the conversion of normal proteins into the abnormal disease-causing form.