Alternative Names
CPI-B; CST6cystatin B (liver thiol proteinase inhibitor)10STFBcystatin-B; CSTB; cystatin B (stefin B); Cystatin B; EPM1; Liver thiol proteinase inhibitor; PME; Stefin B; stefin-B
Background
Cystatin B, also called stefin B or liver thiol proteinase inhibitor, is a member of family 1 of the cystatin superfamily. Like Cystatin A, it is an intracellular inhibitor regulating the activities of cysteine proteases of the papain family such as cathepsins B, H and L. Defects in Cystatin-B / CSTB are the cause of progressive myoclonic epilepsy type 1 (EPM1) which is an autosomal recessive disorder characterized by severe, stimulus-sensitive myoclonus and tonic-clonic seizures.
Note
For Research Use Only , Not for Diagnostic Use.
