The scientific highlight of the Prof. Henry Wisniewski memorial symposium (12 July) was an informative lecture about familial British dementia (FBD) delivered by Blas Frangione. His studies with the 34 amino acid long ABri protein (abstracts 262 and 871) suggest that amyloid production alone is sufficient to initiate a cascade culminating in dementia. Antibodies against ABri recognize both parenchymal and vascular deposits and are accompanied with neurofibrillary changes very similar (both morphologically and biochemically) to those found in AD. Moreover, analysis of the Bri gene in a Danish kindred showed a 10 nucleotide duplication insertion which produces a frame shift and subsequent production of a 34 amino acid long amyloidogenic peptide (ADan). This second Bri mutation also causes dementia and presents with similar neuropathological changes. Thus, it appears that different amyloidogenic peptides can precipitate similar neurodegenerative processes and that amyloidogenesis is a key initiation event in these processes.—Dominic Walsh


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