Fatal familial insomnia(FFI) is a very rare prion disease of the brain. FFI has no known cure and involves progressively worsening insomnia, which eventually leads to hallucinations, delirium, and dementia-like symptoms. The average age of diagnosis is about 40 years of age and the average life span after diagnosis is 18 months. FFI is not caused by an infection, but is inherited in an autosomal dominant fashion. Knowing its inheritance pattern AND knowing it is a prion disease, which of the following is accurate?
A. Individuals with FFI have inherited a gene that encodes a mutant form of a prion protein that folds abnormally.
B. The abnormal prion protein gradually converts the normal prion proteins of the brain into abnormal prion proteins.
C. The abnormal proteins do not get degraded and they form aggregates in brain cells.
D. An individual who inherits one copy of the mutant allele will show the FFI phenotype.
E. All of these are correct.
E. All of these are correct.
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