小黄书 experts address Chronic Wasting Disease potential risk in humans
Contact: Vanessa Beeson
STARKVILLE, Miss.鈥擬ississippi State scientists and leading experts in deer and prion research are weighing in on recent news about deer diseases and potential risk to human health.
A recent brief published in the journal stated that in 2022, two hunters from the same hunting lodge were afflicted with sporadic Creutzfeldt-Jakob disease, or CJD. Steve Demarais, Mississippi State鈥檚 Taylor Chair in Applied Big Game Research and Instruction discussed the abstract and what hunters and the public should know.
Both the human CJD disease and Chronic Wasting Disease (CWD)鈥攁ffecting deer, elk and moose鈥攁re caused by a misfolded protein called a prion.
Demarais, co-director of the 小黄书 Deer Lab and scientist in the university鈥檚 Forest and Wildlife Research Center, has studied the ecology of white-tailed deer for more than 40 years. Much of his recent research focuses on CWD, similar to CJD. Since the first case of CWD was reported in Mississippi in 2016, he has been the state鈥檚 foremost expert driving CWD research, including pioneering environmental testing of the disease鈥檚 presence across the landscape. He also leads outreach programs informing hunters and the public about this disease.
鈥淚t鈥檚 important for people to think critically when considering this news. This was a synopsis presented at a meeting and Neurology published it. Since CJD is so rare, there is cause for concern but there are still so many unknowns regarding this case. We know the two subjects had a history of consuming venison, but we don鈥檛 know if they ingested meat that tested positive for CWD. The scientists who published the brief themselves noted that more research is needed. At this point, research has not demonstrably proven that CJD was caused by CWD,鈥 Demarais explained.
While there are normal proteins in all mammals that follow a normal cycle of life鈥攕erving a function, then degrading and shedding from the body鈥攚hen a misfolded prion encounters normal protein, it causes the latter to refold into the prion鈥檚 abnormal shape. These misfolded prions can鈥檛 break down, so they build up and damage neural tissue, ultimately leading to mortality.
Assistant Professor Galen Collins in 小黄书鈥檚 Department of Biochemistry, Molecular Biology, Entomology and Plant Pathology, is an expert in intracellular protein degradation. Prior to joining 小黄书, he spent 12 years as a cell biology research fellow at Harvard Medical School. Collins discussed the research.
鈥淭he occurrence of sporadic CJD is about one in a million. The synopsis records two people located in the same space and time that came down with this disease. This is very unusual, and I think they鈥檙e right to raise concern over this. That said, more information is needed to reach a definitive conclusion about the case,鈥 he said.
Collins, a scientist in the Mississippi Agricultural and Forestry Experiment Station, explained more about CJD in humans.
鈥淣ormal proteins are a globular shape known as the alpha helix with many turns and twists that almost look like a spiral staircase. Misfolded proteins, or prions, are long wavy sheets called beta sheets that are very stable and stick well together,鈥 he explained.
Collins said there is a genetic form of CJD called familial fatal insomnia and there was also an increase in spontaneous incidences of the disease in Europe in the 1990s, when 200 people died due to the consumption of cows that had the prion that caused bovine spongiform encephalopathy (BSE), commonly referred to as Mad Cow Disease.
鈥淭ypically, rare spontaneous cases tend to occur in old age because it takes a long time for the alpha helix to turn into the beta sheet. But when you eat infected meat, you鈥檙e basically accelerating the process,鈥 he said.
Collins also noted the prions that cause CWD are not killed through typical cooking methods.
William T. McKinley, deer program coordinator for the Mississippi Department of Wildlife, Fisheries and Parks (MDWFP), encourages hunters to be vigilant in testing for CWD.
鈥淢DWFP continues to recommend that hunters submit samples from deer harvested in areas where CWD has been found and to not consume the meat from deer that tested positive for CWD. MDWFP operates over 55 CWD drop off freezers and works with over 75 cooperating taxidermists across the state.聽There is no cost to the hunter to get a deer tested,鈥 he said.
Demarais echoes the sentiment to test harvested deer.
鈥淢ake informed decisions and look for collective input from scientists and health professionals. Testing for CWD is critical and easy. I鈥檝e been a hunter all my life and as hunters we feel compelled to eat what we harvest; however, it is important to test your deer to ensure the safety of yourself and your family,鈥 Demarais said.
For more on the 小黄书 Deer Lab, visit . To view the lab鈥檚 comprehensive video series about CWD, visit .
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