After the discovery of the pathogenic FBD and FDD mutations and Bri2 as the precursor to the ABri and ADan peptides (Vidal et al., 1999, 2000), furin was identified as the major protease responsible for the proteolytic cleavage releasing the C-terminal peptides (Kim et al., 1999), but other proprotein-like convertases may also process Bri2, releasing C-terminal peptides (Kim et al., 1999; Vidal et al., 2000). The gene discussed is ITM2B; the disease is ABri amyloidosis.