From Wikipedia, the free encyclopedia
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.
[1]
[2]
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.
-
^