Protein domain
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).
Further reading
Johansson H, Eriksson M, Nordling K, Presto J, Johansson J (June 2009). "The Brichos domain of prosurfactant protein C can hold and fold a transmembrane segment" . Protein Sci . 18 (6): 1175–82. doi :10.1002/pro.123 . PMC 2774428 . PMID 19472327 . Johansson H, Nordling K, Weaver TE, Johansson J (July 2006). "The Brichos domain-containing C-terminal part of pro-surfactant protein C binds to an unfolded poly-val transmembrane segment" . J. Biol. Chem . 281 (30): 21032–9. doi :10.1074/jbc.M603001200 . PMID 16709565 . Mulugeta S, Nguyen V, Russo SJ, Muniswamy M, Beers MF (June 2005). "A surfactant protein C precursor protein BRICHOS domain mutation causes endoplasmic reticulum stress, proteasome dysfunction, and caspase 3 activation" . Am. J. Respir. Cell Mol. Biol . 32 (6): 521–30. doi :10.1165/rcmb.2005-0009OC . PMC 2715321 . PMID 15778495 . References
Sánchez-Pulido L, Devos D, Valencia A (July 2002). "BRICHOS: a conserved domain in proteins associated with dementia, respiratory distress and cancer". Trends Biochem. Sci . 27 (7): 329–32. doi :10.1016/s0968-0004(02)02134-5 . PMID 12114016 .
Hedlund J, Johansson J, Persson B (2009). "BRICHOS - a superfamily of multidomain proteins with diverse functions" . BMC Res Notes . 2 : 180. doi :10.1186/1756-0500-2-180 . PMC 2751770 . PMID 19747390 .
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Human SP-C BRICHOS domain, extracted monomer (PDB: 2yad)