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Media Advisory
Friday, June 14, 2019
What
Scientists from the National Institutes of Health have used human skin cells to create what they believe to be the first brain organoid system, or "mini-brain," intended for the study of Creutzfeldt's disease -Jakob (CJD) sporadic. CJD is a fatal neurodegenerative brain disorder in humans, which could be caused by an infectious prion protein. It affects about 1 million people. Researchers at the NIH's National Institute of Allergy and Infectious Diseases (NIAID) hope the human organoid model will allow them to evaluate potential treatments for CJD and provide more details on the subtypes of prion diseases than rodent and non-human primate models currently in use.
Human brain organoids are small balls of human brain cells that range in size from poppy seed to pea. Their organization, structure and electrical signaling are similar to those of brain tissue. Since these brain organoids can survive for months in a controlled environment, diseases of the nervous system can be studied over time. Cerebral organoids have been used as models for studying Zika virus infection, Alzheimer's disease and Down's syndrome.
In a new study published in Acta Neuropathologica Communications, scientists at NIAID's Rocky Mountain Laboratories discovered how to infect five-month-old brain organoids with prions using samples taken from two deceased patients of two different CJD subtypes, MV1 and MV2. It took about a month to confirm the infection, and scientists monitored for more than six months changes in health indicators, such as metabolism, in organoids. At the end of the study, scientists observed that seeding activity, an indication of the infectious spread of prions, was present in all organoids exposed to CJD samples. However, seeding was more important in organoids infected with the MV2 sample than in the MV1 sample. They also reported that MV1-infected organoids were more damaging than MV2-infected organoids.
Scientists have also noted other differences between the evolution of MV1 and MV2 infections in organelles. They plan to further investigate these differences in the hope of identifying how different subtypes of CJD affect brain cells. In the end, they hope to learn how to prevent cell damage and restore the function of cells damaged by prion infection. The new system also offers the opportunity to test potential treatments for CJD in a tissue model that mimics the human brain.
article
B Groveman et al. Sporadic Creutzfeldt-Jakob Disease prion infection of human brain organoids. Acta Neuropathologica Communications DOI: 10.1186 / s40478-019-0742-2 (2019).
who
Cathryn Haigh, Ph.D., a researcher at the NIAID Laboratory for Persistent Viral Diseases, is available to comment on this study.
Contact
To schedule interviews, please contact Ken Pekoc at (301) 402-1663, [email protected].
This press release describes basic research. Basic research increases our understanding of human behavior and biology, which is fundamental to advancing new and improved methods of disease prevention, diagnosis and treatment. Science is an unpredictable and progressive process – every breakthrough in research builds on past discoveries, often unexpectedly. Most clinical progress would not be possible without the knowledge of basic fundamental research.
NIAID conducts and supports research – at NIH, in the United States, and around the world – to investigate the causes of infectious and immune-mediated diseases and to develop better ways to prevent, diagnose and treat these diseases. NIAID press releases, fact sheets and other documents are available on the NIAID website.
About the National Institutes of Health (NIH):
The NIH, the country's medical research agency, has 27 institutes and centers and is part of the US Department of Health and Human Services. NIH is the lead federal agency that leads and supports basic, clinical and translational medical research. She studies causes, treatments and treatments for common and rare diseases. For more information on NIH and its programs, visit www.nih.gov.
NIH … transforming discovery into health®
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