BRICHOS family

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BRICHOS domain
Human SFTPC BRICHOS 2yad.png
Human SP-C BRICHOS domain, extracted monomer ( PDB: 2yad )
Identifiers
SymbolBRICHOS
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. [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

Related Research Articles

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<span class="mw-page-title-main">Surfactant protein C</span> Protein-coding gene in the species Homo sapiens

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<span class="mw-page-title-main">Dedicator of cytokinesis protein 3</span> Protein found in humans

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Surfactant metabolism dysfunction is a condition where pulmonary surfactant is insufficient for adequate respiration. Surface tension at the liquid-air interphase in the alveoli makes the air sacs prone to collapsing post expiration. This is due to the fact that water molecules in the liquid-air surface of alveoli are more attracted to one another than they are to molecules in the air. For sphere-like structures like alveoli, water molecules line the inner walls of the air sacs and stick tightly together through hydrogen bonds. These intermolecular forces put great restraint on the inner walls of the air sac, tighten the surface all together, and unyielding to stretch for inhalation. Thus, without something to alleviate this surface tension, alveoli can collapse and cannot be filled up again. Surfactant is essential mixture that is released into the air-facing surface of inner walls of air sacs to lessen the strength of surface tension. This mixture inserts itself among water molecules and breaks up hydrogen bonds that hold the tension. Multiple lung diseases, like ISD or RDS, in newborns and late-onsets cases have been linked to dysfunction of surfactant metabolism.

<span class="mw-page-title-main">TMEM106A</span> Protein-coding gene in the species Homo sapiens

TMEM106A is a gene that encodes the transmembrane protein 106A (TMEM106A) in Homo sapiens. It is located at 17q21.31 on the plus strand next to cancer-related genes NBR1 and BRCA1. The TMEM106A gene contains a domain of unknown function, DUF1356.

References

  1. 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.
  2. 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.