Reactivities: | Human |
Presku: | AAP59177 |
Size: | 100ug |
Weight: | 11kDa |
Gene: | 1476 |
Format: | Lyophilized powder |
Target: | CSTB is a stefin that functions as an intracellular thiol protease inhibitor. The protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. The protein is thought to play a role in protecting against the proteases leaking from lysosomes. Evidence indicates that mutations in CSTB gene are responsible for the primary defects in patients with progressive myoclonic epilepsy a stefin that functions as an intracellular thiol protease inhibitor. The protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. The protein is thought to play a role in protecting against the proteases leaking from lysosomes. Evidence indicates that mutations in this gene are responsible for the primary defects in patients with progressive myoclonic epilepsy. |
Alternative names: | CST6; EPM1; PME; STFB |