Screening could prevent spread of infectious proteins through transfusions
A new blood test can detect even tiny amounts of infectious proteins called prions, two new studies show.
Incurable prion diseases, such as mad cow disease (BSE) in cattle and variant Creutzfeldt-Jakob disease (vCJD) in people, result from a normal brain protein called PrP twisting into a disease-causing “prion” shape that kills nerve cells in the brain. As many as 30,000 people in the United Kingdom may be carriers of prions that cause vCJD, presumably picked up by eating BSE-tainted beef. Health officials worry infected people could unwittingly pass prions to others through blood transfusions. Four such cases have already been recorded. But until now, there has been no way to screen blood for the infectious proteins.
In the test, described December 21 in Science Translational Medicine, magnetic nanobeads coated with plasminogen — a protein that prions grab onto — trap prions. Washing the beads gets rid of the rest of the substances in the blood. Researchers then add normal PrP to the beads. If any prions are stuck to the beads, the infectious proteins will convert PrP to the prion form, which will also stick to the beads. After many rounds, the researchers could amplify the signal enough to detect vCJD prions in all the people in the studies known to have the disease.
No healthy people or people with other degenerative brain diseases (including Alzheimer’s and Parkinson’s) in either study had evidence of the infectious proteins in their blood. And only one of 83 people with a sporadic form of Creutzfeld-Jakob disease tested positive. Those results indicate that the test is specific to the vCJD prion form, so a different test is needed to detect the sporadic disease.
In two cases, researchers detected prions in frozen blood samples collected 31 months and 16 months before people developed vCJD symptoms.
D. Bougard et al. Detection of prions in the plasma of presymptomatic and symptomatic patients with variant Creutzfeldt-Jakob disease. Science Translational Medicine. Vol. 8, December 21, 2016, p.370ra182. doi: 10.1126/scitranslmed.aag1257.
L. Concha-Marambio et al. Detection of prions in blood from patients with variant Creutzfeldt-Jakob disease. Science Translational Medicine. Vol. 8, December 21, 2016, p.370ra183. doi: 10.1126/scitranslmed.aaf6188.
T.H. Saey. Prion disease gets personal. Science News. Vol. 189, March 5, 2016, p. 4.
T.H. Saey. A protein variant can provide protection from deadly brain-wasting. Science News. Vol. 188, July 11, 2015, p. 11.
T.H. Saey. California mad cow case no reason for panic. Science News Online, April 30, 2012.
C. Brownlee. Better Blood: New tool removes agent of brain disease. Science News. Vol. 171, January 6, 2007, p. 4.