| fatal familial insomnia |
Fatal familial insomnia (FFI) is a very rare, autosomal dominant inherited, brain disease. It is caused by a mutation in a protein called prion protein (PrP): asparagine-178 is replaced by aspartic acid. The mutation changes the shape of PrP so that it becomes a prion and makes other, normal PrP molecules change to the abnormal shape. This causes amyloid plaques in the thalamus, the region of the brain responsible for regulation of sleep patterns. ...
Ãâó: en.wikipedia.org/wiki/Fatal_familial_insomnia
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| fatal familial insomnia |
ABBR: FFI. An inherited, rapidly progressive prion disease of middle or later life. Signs and symptoms include intractable insomnia, autonomic dysfunction, endocrine disturbances, dysarthria, myoclonus, coma, and death.
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