BRICHOS: a conserved domain in proteins associated with dementia, respiratory distress and cancer
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Updated: Sep 30, 2026

Quantifying Tissue-Specific Proteostatic Decline in Caenorhabditis elegans
Published on: September 7, 2021
Luis Sánchez-Pulido1, Damien Devos, Alfonso Valencia
1Protein Design Group, Centro Nacional de Biotecnología (CNB-CSIC), Cantoblanco E-28049, Madrid, Spain.
Insights
A newly discovered BRICHOS domain appears in proteins linked to major diseases like dementia and cancer. This domain may play a role in protein processing after their initial creation.
Area of Science:
- Molecular Biology
- Genetics
- Protein Science
Background:
- A novel BRICHOS domain of ~100 amino acids has been identified.
- This domain is present in proteins associated with major diseases.
Purpose of the Study:
- To identify and characterize the novel BRICHOS domain.
- To investigate the potential role of the BRICHOS domain in disease-related proteins.
Main Methods:
- Bioinformatic analysis to identify the BRICHOS domain across protein families.
- Comparative analysis of protein sequences and structures.
- Review of existing experimental data on related proteins.
Main Results:
- The BRICHOS domain is conserved in proteins linked to familial British/Danish dementia (BRI2), chondrosarcoma (Chondromodulin-I), stomach cancer (CA11), and respiratory distress syndrome (surfactant protein C).
- In many cases, the BRICHOS domain is located in the propeptide region, suggesting involvement in post-translational modification.
- Experimental data hint at a role in complex protein processing.
Conclusions:
- The BRICHOS domain is a significant finding in proteins associated with diverse major diseases.
- Its location and conservation suggest a critical function in protein maturation and processing.
- Further research is warranted to elucidate the precise mechanisms by which the BRICHOS domain influences protein function and disease pathology.
Abstract:
A novel domain (the BRICHOS domain) of approximately 100 amino acids has been identified in several previously unrelated proteins that are linked to major diseases. These include BRI(2), which is related to familial British and Danish dementia (FBD and FDD); Chondromodulin-I (ChM-I), related to chondrosarcoma; CA11, related to stomach cancer; and surfactant protein C (SP-C), related to respiratory distress syndrome (RDS). In several of these, the conserved BRICHOS domain is located in the propeptide region that is removed after proteolytic processing. Experimental data suggest that the role of this domain could be related to the complex post-translational processing of these proteins.
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