World Alzheimer Conference 2000: Amyloid Is Necessary and Sufficient to Cause Dementia
17 July 2000. 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.