Tests on brain organoids suggest the disease-causing prions face a tough barrier to infect people, but ruling out transmission is a difficult task.
Chronic wasting disease has been spreading among deer in the United States, which has raised concerns that the fatal neurological illness might make the leap to people. But a recent study suggests that the disease has a tough path to take to get into humans.
“This was a model that could really help tell us … whether or not it was a real risk,” says Bradley Groveman, a biologist at the National Institutes of Health’s Rocky Mountain Laboratories in Hamilton, Mont. Concern about the danger that deer, elk and moose prions pose to people has been rising, in part because of the disease’s persistent spread across North America. On April 5, CWD was. To date, the disease has been identified in wildlife in 34 U.S. states as well as parts of Canada, South Korea and northern Europe — a considerable increase since the first known case appeared in 1967 in a captive deer from Colorado.
News reports in April put the spotlight on a case report presented at the annual American Academy of Neurology meeting in Denver that described a fatal neurological disease in two hunters. The individuals had a history of consuming deer meat from a population known to have chronic wasting disease. But both likely died of a different prion ailment, Creutzfeldt-Jakob disease, which can arise sporadically, according to both the report and the U.S. Centers for Disease Control and Prevention.
Prion strains, which twist PrP proteins in different ways, can also come into play. How prions misfold is one factor that can prevent the proteins from infecting a new species, Groveman says. Figuring out the differences in how deer prions bend and curl could help expose which might attach to PrP from people or other animals.
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