Thursday, January 19, 2023

Cerebral Organoid Model in NIAID Study Reveals New Understanding of Fatal Familial Insomnia

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Thursday, Jan. 19, 2023

Cerebral Organoid Model in NIAID Study Reveals New Understanding of Fatal Familial Insomnia

FFI organoid

This image shows differences in brain waves, illustrating brain cell dysfunction in organoid models of fatal familial insomnia compared to controls. (Credit: NIAID)

NIAID scientists used a human cerebral organoid model to observe brain cells exposed to prion protein carrying the mutation that causes fatal familial insomnia. Surprisingly, they did not observe spontaneous change in shape or spread of the mutated protein to additional prion protein – typically a trademark of prion disease. Most changes, they found, were caused by the presence of the mutation rather than interacting with prion protein lacking the mutation. They also found that neurons in the FFI organoid model were impaired because of damaged mitochondria – interactions that are the focus of upcoming study.

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