Fatal Familial Insomnia Project
Fatal Familial Insomnia, or FFI, is a transmissible spongiform encephalopathy caused by a mutation in the PRNP gene resulting in a D178N mutation in the PrP protein. FFI can be developed through mutations during life, inherited from parents who develop mutations, or by being exposed to the mutated PrP proteins of an infected individual. The disease causes protein aggregation and subsequent deterioration of the brain, particularly within the thalamus. This results in insomnia and sympathetic hypersensitivity, and typically causes death within eighteen months of diagnosis. Little is known about the mechanisms by which FFI causes protein aggregation. Mutated PrP proteins are being simulated with various drugs in order to determine possible inhibitors for FFI.
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