Abstract
Background: Familial Danish dementia (FDD) is a rare autosomal dominant neurodegenerative disorder that shares pathological features with Alzheimer's Disease. It results from a decamer duplication in the BRI gene, giving rise to a precursor protein that is 11 residues longer than the wildtype (Vidal et al., 2000). Furin cleavage releases a 34-residue peptide Adan, which is deposited in the brains of the Danish patients. Objectives: To explore the properties of Adan. Methods: We synthesised Adan in its reduced and oxidised (cyclic) forms. AFM, SDS-PAGE, gel filtration, electrospray mass spectrometry and electron microscopy showed that Adan readily forms soluble and insoluble aggregates (Gibson et al., 2004); The aggregates of both reduced and oxidised Adan were toxic to human neuroblastoma cells in culture, inducing pan-caspase activation which was indicative of apoptosis.
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