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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
Related
CST6, STFB, CSTB or EPM1 gene
General
cystatin
Properties
SIZE: entity length = 98 aa
MW = 11 kD
COMPARTMENT: cytoplasm
cell nucleus
MOTIF: acetylation site
SITE: 1-1
EFFECTOR-BOUND: acetyl
glycine residue {4}
subtrate contact {46-50}
binding site
FOR-BINDING-OF: cysteine proteinase
INHIBITS: cysteine proteinase
Database Correlations
OMIM correlations
UniProt P04080
Pfam PF00031
Entrez Gene 1476
Kegg hsa:1476
References
- UniProt :accession P04080
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=CSTB