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Scientists develop models & # 39; mini brain & # 39; human prion disease


Scientists from the United States National Institutes of Health have used human skin cells to create the first brain organoid system, or & # 39; mini brain & # 39; which can be used to study sporadic Creutzfeldt-Jakob disease (CJD), a pathology The fatal neurodegenerative brain caused by prion proteins is contagious and affects 1 in 1 million people.

Organoids The human brain is a small sphere of human brain cells of varying size, from poppy seeds to small peas. The electrical organization, structure and signaling are similar to brain tissue. Because they can survive in a controlled environment for months, diseases of the nervous system can be learned from time to time. Cerebral organisms have been used as models to study Zika virus infections, Alzheimer's disease and Down syndrome.

In their new study, published in the journal & # 39; Acta Neuropathologica Communications & # 39; scientists found a way to infect five-month-old brain organoid with prions using a sample of two patients who died because two different subtypes were sporadic Creutzfeldt-Jakob disease, MV1 and MV2.

The infection takes about a month to be confirmed, and scientists monitor organoids for changes in health indicators, such as metabolism, for more than six months. At the end of the study, the scientists observed that hatchery activity, an indication of the spread of infectious prions, is present in all organoids exposed to CJD samples. However, it was higher in organoids infected with MV2 samples than in MV1 samples. Organoid infected with MV1 showed more damage than those infected with MV2.

The scientists also observed another difference between the evolution of MV1 and MV2 infections in organoids. They plan to investigate these differences further in the hope of identifying how the various subtypes of Jakob Creutzfeldt disease affect brain cells.

In the end, they hope to learn how to prevent cell damage and restore cell function damaged by prion infection. The new system also provides an opportunity to test potential therapies for Creutzfeldt-Jakob disease in tissue models that mimic the human brain.

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