Results from a couple recent studies show progress in understanding, and ultimately preventing, prion-associated diseases such as bovine spongiform encephalopathy (BSE). One study explores the use of an antihistamine to reduce prion aggregation, while the other tests new culturing and testing methods that could significantly reduce the time required to conduct experiments on prion diseases.
In a study reported in the journal Proceedings of the National Academy of Sciences, researchers led by a team at Michigan State University explored the process through which proteins clump, or aggregate, prior to causing neurodegenerative diseases such as BSE in cattle, chronic wasting disease (CWD) in cervids or Creutzfeldt–Jakob disease (CJD) in humans. Experimenting with hamsters and rabbits, they found that pH plays a key role in protein aggregation. They also tested the effects of drugs and found that an antihistamine, astemizole, effectively speeds up protein self-interactions and prevents prion clumping. Astemizole, according to the researchers, was once used to treat allergies, but it was removed from the market due to rare but sometimes fatal side effects. The antihistamine has, however, shown promise in some Alzheimer’s research.
This research was funded in part by the National Institutes of Health.
In another study, researchers at Iowa State University developed a highly sensitive method of screening for these diseases that involves intact, living brain tissue from model animals and maintaining them as live cultures. Their paper, titled “Integrated Organotypic Slice Cultures and RT-QuIC (OSCAR) Assay: Implications for Translational Discovery in Protein Misfolding Diseases,” appears in the journal Scientific Reports.
Prion diseases have notoriously long incubation periods, which hamper the efforts of scientists to study the disease process in live models. However, lead researcher Anumantha Kanthasamy, Distinguished Professor and chair of the ISU Department of Biomedical Sciences, says the new screening method can start showing the disease progression in as little as seven days and can be repeated with greater speed than using live models, allowing generation of higher throughput data.
“This has a lot of potential to help us understand the disease process,” Kanthasamy said. “We can test potential new therapies and study how they work. We expect this technique to catch on quickly among scientists looking at these diseases.”