20 May NIH Study Suggests Prion Disease Unlikely to Spread from Deer to Humans
MedicalResearch.com Interview with:
Dr. Haigh
Cathryn Haigh, Ph.D.
Chief Prion Cell Biology Unit
Laboratory of Neurological Infections and Immunity
National Institute of Allergy and Infectious Diseases
Division of Intramural Research, Rocky Mountain Laboratories
National Institutes of Health
Hamilton, MT 59840
MedicalResearch.com: What is the background for this study, ie what are prions/prion-related diseases? Where are prions found?
Response: Prion diseases are infectious neurodegenerative diseases of humans and animals. In humans these diseases often manifest as rapidly progressing dementias but are rarely caused by a known exposure to the infectious agents (prions). More commonly they are sporadic (no known cause) or hereditary.
One form of human disease is believed to have arisen from eating beef contaminated with bovine spongiform encephalopathy (as known as mad cow disease). This has resulted in concerns that chronic wasting disease (CWD), a prion disease affecting deer, elk and moose, might also have the potential to cross the species barrier and cause disease in humans. To date, transmissions of CWD prions to cynomolgus macaques have been negative, a good sign that crossing the species barrier would not be easy, but macaques are not human so we wanted to test whether CWD could infect human brain tissue.
To do this we used a human cerebral organoid model (mini human brain tissues grown from skin cells in a laboratory) and directly exposed the organoids to prions from the brains of animals that had died of CWD.
MedicalResearch.com: What are the main findings?
Response: Our study showed CWD prions from white tailed deer, mule deer and elk did not infect human cerebral organoid cultures.
MedicalResearch.com: What should readers take away from your report?
Response: This supports that there is a strong species barrier against CWD infecting humans and, therefore, transmission to humans is unlikely.
MedicalResearch.com: What recommendations do you have for future research as a results of this study?
Response: While we believe our results are promising that CWD will not infect humans, there are many factors we cannot control for in our experiments (such as emergence of a new strain or a subset of the human population that are especially susceptible). Therefore, the field should continue to be diligent in monitoring cases of human disease closely.
Our research will continue to look for whether there are factors that might make CWD crossing the species barrier more likely, such as a subset of vulnerable humans, and if new strains are identified we will also test these in the organoid system.
None of the authors have any disclosures.
Citation: Groveman BR, Williams K, Race B, Foliaki S, Thomas T, Hughson AG, et al. Lack of transmission of chronic wasting disease prions to human cerebral organoids. Emerg Infect Dis. 2024 Jun [date cited]. https://doi.org/10.3201/eid3006.231568
DOI: 10.3201/eid3006.231568
Original Publication Date: May 17, 2024
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Last Updated on May 20, 2024 by Marie Benz MD FAAD