Article
The Familial British Dementia Mutation Promotes Formation of Neurotoxic Cystine Cross-linked Amyloid Bri (ABri) Oligomers.
The Journal of biological chemistry - 3 Jul 2015
Cantlon Adam, Frigerio Carlo Sala, Freir Darragh B, Boland Barry, Jin Ming, Walsh Dominic M
Abstract excerpt
Familial British dementia (FBD) is an inherited neurodegenerative disease believed to result from a mutation in the BRI2 gene. Post-translational processing of wild type BRI2 and FBD-BRI2 result in the production of a 23-residue long Bri peptide and a 34-amino acid long ABri peptide, respectively, and ABri is found deposited in the brains of individuals with FBD. Similarities in the neuropathology and clinical...
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