cystatin-B (liver thiol protease inhibitor, CPI-B, stefin-B)

Function

• intracellular thiol proteinase inhibitor
• tightly binding reversible inhibitor of cathepsins L, H & B
• able to form dimers stabilized by noncovalent forces

Structure

belongs to the cystatin family

Compartment

cytoplasm. nucleus

Pathology

• defects in CSTB are the cause of progressive myoclonic epilepsy type 1

More general terms

• cystatin

Database

• UniProt: http://www.uniprot.org/uniprot/P04080.html
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:1476
• OMIM: https://mirror.omim.org/entry/254800
• OMIM: https://mirror.omim.org/entry/601145
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=1476