BRICHOS family

BRICHOS domain
Human SP-C BRICHOS domain, extracted monomer (PDB: 2yad​)
Identifiers
Symbol	BRICHOS
Pfam	PF04089
InterPro	IPR007084
PROSITE	PDOC50869
CATH	2yadA00
Available protein structures: Pfam   structures / ECOD   PDB RCSB PDB; PDBe; PDBj PDBsum structure summary

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.