cystatin b


Summary: An intracellular cystatin subtype that is found in a broad variety of cell types. It is a cytosolic enzyme inhibitor that protects the cell against the proteolytic action of lysosomal enzymes such as CATHEPSINS.

Top Publications

  1. Lalioti M, Scott H, Buresi C, Rossier C, Bottani A, Morris M, et al. Dodecamer repeat expansion in cystatin B gene in progressive myoclonus epilepsy. Nature. 1997;386:847-51 pubmed
    ..Two point mutations in the cysteine proteinase inhibitor gene cystatin B (CSTB), proved that this gene is responsible for EPM1 (ref. 3)...
  2. Genton P. Unverricht-Lundborg disease (EPM1). Epilepsia. 2010;51 Suppl 1:37-9 pubmed publisher
  3. Ulbrich L, Cozzolino M, Marini E, Amori I, De Jaco A, Carri M, et al. Cystatin B and SOD1: protein–protein interaction and possible relation to neurodegeneration. Cell Mol Neurobiol. 2014;34:205-13 pubmed
    b>Cystatin B (CSTB), an inhibitor of the cysteine proteases, belongs to the cathepsin family and it is known to interact with a number of proteins involved in cytoskeletal organization...