Researchers are on their way to developing a test that can rapidly diagnose fatal brain conditions like mad cow disease in cattle and Creutzfeldt-Jakob disease in humans.
By developing a fast and highly sensitive way to detect and measure the infectious particles – or prions – that cause these brain damaging disorders, researchers at NIH’s National Institute of Allergy and Infectious Disease (NIAID) hope for some kind of early treatment.
Difficult to diagnose, untreatable, and fatal, prion diseases are also known as transmissible spongiform encephalopathies (TSEs) and is characterized by dead tissue that leaves sponge-like holes in the brain.
Scientists believe that the disease-causing prions are abnormal infectious clusters of normal prion molecules, which are unclustered and found in every mammal.
A fast test is so important because prion infections can be around for decades before symptoms appear. Prions can be found in a wide variety of tissues and bodily fluids from infected mammals, including blood, breast milk, saliva, urine, and nasal fluids – but their miniscule amounts make it hard to detect.
So Byron Caughey from Rocky Mountain Laboratories in Montana and his colleagues found a way to detect when large amounts of normal prion proteins are converted into abnormal forms in test tube. They call this real time quaking induced conversion assay (RT-QuIC).
They used RT-QuIC to detect prion infections in deer with chronic wasting disease, sheep with scrapie, and hamsters infected with scrapie.
“Although relatively rare in humans and other animals, prion diseases are devastating to those infected and can have huge economic impacts,” says NIAID director Anthony S. Fauci. “Scientists have promising concepts for developing therapies for people infected with prion diseases, but treatments only are helpful if it is known who needs them. This detection model could eventually bridge that gap.”
The researchers said related approaches might be used to diagnose other protein related diseases in the brain such as Alzheimer’s, Huntington’s and Parkinson’s diseases.
Image: brain of patient with Creutzfeldt-Jakob disease via Wikimedia Commons