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Friday, May 17, 2024
Cerebral organoid studies strengthen evidence for substantial species barrier
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New research on prion disease using a human cerebral organoid model suggests there is a substantial species barrier that prevents transmission of chronic wasting disease (CWD) from cervids such as deer, elk and elk to humans I am doing it. The findings, published by scientists at the National Institutes of Health and published in the journal Emerging Infectious Diseases, follow similar studies conducted over decades using animal models at the NIH's National Institute of Allergy and Infectious Diseases (NIAID). Consistent with research.
Prion diseases are degenerative diseases found in some mammals. These diseases primarily involve a decline in brain function, but can also affect the eyes and other organs. Disease and death occur when the abnormal protein folds, aggregates, recruits other prion proteins to do the same, and ultimately destroys the central nervous system. Currently, there is no prevention or treatment for prion diseases.
CWD is a type of prion disease found in deer, which are popular game animals. Although CWD has never been found in humans, questions about whether people who eat meat from deer infected with CWD can develop prion disease and its transmission potential have persisted for decades. I am. From the mid-1980s to the mid-1990s, another prion disease, bovine spongiform encephalopathy (BSE) or mad cow disease, occurred in cattle in the United Kingdom (UK), and cases were detected in cattle in other countries. The issue is important. Countries including the United States. Over the next 10 years, 178 people in the UK who were believed to have eaten BSE-infected beef developed variant Creutzfeldt-Jakob disease, a new human prion disease, and died. Researchers later determined that the disease was spread among cattle through feed contaminated with infectious prion protein. The route of transmission of the disease from feed to cattle to humans frightened the British population and alarmed the world against other prion diseases transmitted from animals to humans, including CWD. CWD is the most transmissible member of the prion disease family and has been shown to transmit very efficiently between cervids.
Historically, scientists have used mice, hamsters, squirrel monkeys, and cynomolgus monkeys to mimic human prion diseases and have monitored the animals for signs of CWD for more than a decade. In 2019, NIAID scientists at Rocky Mountain Research Institute in Hamilton, Montana, developed a human cerebral organoid model of Creutzfeldt-Jakob disease to evaluate potential treatments and study certain human prion diseases. was developed.
Human cerebral organoids are tiny spheres of human brain cells that range in size from a poppy seed to a pea. Scientists grow organoids from human skin cells in culture dishes. The organization, structure, and electrical signaling of brain organoids are similar to brain tissue. These are currently the closest available laboratory models to the human brain. Scientists use organoids to study neurological diseases over time because they can survive for months in a controlled environment. Cerebral organoids have been used as models to study other diseases such as Zika virus infection, Alzheimer's disease, and Down syndrome.
In the new CWD study (mostly conducted in 2022 and 2023), the research team successfully infected human cerebral organoids with human CJD prions (a positive control) to validate the study model. Then, using the same laboratory conditions, healthy human cerebral organoids were directly exposed to high concentrations of CWD prions from white-tailed deer, mule deer, elk, and normal brain material (negative control) for 7 days. The researchers then observed the organoids for up to six months, but none were infected with CWD.
According to the researchers, this indicates that human central nervous system tissue has substantial resistance or barrier to transmission of infection even after direct exposure to CWD prions. The authors acknowledge the study's limitations, including the possibility that a small number of people have unexplained genetic susceptibilities and the possibility that new strains may emerge with lower barriers to infection. They are optimistic that, based on these current data, it is highly unlikely that humans will become infected with prion disease by inadvertently eating meat from CWD-infected deer.
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B Groveman and K Williams et al. Lack of transmission of chronic wasting disease prions to human cerebral organoids. Emerging Infectious Diseases DOI: 10.3201/eid3006.231568 (2024).
B Groveman and NC Ferreira et al. Human cerebral organoids as a therapeutic drug screening model for Creutzfeldt-Jakob disease. Scientific Report DOI: 10.1038/s41598-021-84689-6 (2021).
Lack of transmission of chronic wasting disease to cynomolgus monkeys. Journal of Virology DOI: 10.1128/JVI.00550-18 (2018).
Susceptibility of nonhuman primates to chronic wasting disease. Emerging Infectious Diseases DOI: 10.3201/eid1509.090253 (2009).
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Dr. Kathryn Haig, director of the Prion Cell Biology Unit in NIAID's Institute of Neuroinfectious Diseases and Immunology, was available to comment on the study.
NIAID conducts and conducts research at the NIH, throughout the United States, and around the world to study the causes of infectious and immune-mediated diseases and to develop better ways to prevent, diagnose, and treat these diseases. We support you. News releases, fact sheets, and other NIAID-related materials are available on the NIAID website.
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