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Familial British dementia (FBD) is an autosomal dominant condition caused by a point mutation in the stop codon of the BRI gene. This mutation extends the normal precursor protein (PP) of 266 amino acids to the next stop codon, which is at amino acid 277. Kim and colleagues demonstrated in vitro that furin can process both the normal protein BriPP and the extended protein ABriPP to produce C-terminal...
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