BRICHOS: a conserved domain in proteins associated with dementia, respiratory distress and cancer

Trends Biochem Sci. 2002 Jul;27(7):329-32. doi: 10.1016/s0968-0004(02)02134-5.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Motifs
  • Amino Acid Sequence
  • Animals
  • Dementia / genetics*
  • Dementia / metabolism
  • Humans
  • Infant, Newborn
  • Intercellular Signaling Peptides and Proteins / chemistry
  • Intercellular Signaling Peptides and Proteins / genetics
  • Intercellular Signaling Peptides and Proteins / metabolism
  • Membrane Proteins*
  • Molecular Sequence Data
  • Neoplasm Proteins / chemistry
  • Neoplasm Proteins / genetics
  • Neoplasm Proteins / metabolism
  • Neoplasms / genetics*
  • Neoplasms / metabolism
  • Protein Structure, Tertiary*
  • Pulmonary Surfactants / chemistry
  • Pulmonary Surfactants / metabolism
  • Respiratory Distress Syndrome, Newborn / genetics*
  • Respiratory Distress Syndrome, Newborn / metabolism
  • Sequence Alignment


  • Intercellular Signaling Peptides and Proteins
  • Membrane Proteins
  • Neoplasm Proteins
  • Pulmonary Surfactants
  • CNMD protein, human