The BRICHOS family consists of a variety of proteins linked to major diseases, each containing a 100 amino acid BRICHOS domain that is thought to have a chaperone function. These include BRI2, which is related to familial British and Danish dementia (FBD and FDD); Chondromodulin-I, related to chondrosarcoma; CA11, related to stomach cancer; and surfactant protein C (SP-C), related to respiratory distress syndrome (RDS).
Role in amyloid formation The BRICHOS domain of Bri2 (Bri2 BRICHOS) has been studied for its effect on amyloid formation, including in pancreatic islets. Bri2 BRICHOS domain is a molecular chaperone that can prevent the formation of amyloid-β (Aβ) and islet amyloid polypeptide (IAPP) fibrils. Experiments with IAPP show that the Bri2 BRICHOS domain prevents IAPP fibril formation and promotes amorphous aggregate formation instead of IAPP amyloid fibrils. In a study of human stem cell-derived islets exposed to metabolic stress in vitro, overexpression of the Bri2 BRICHOS domain reduces IAPP amyloid formation and partially protects β-cell function.
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