cystatin B

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This gene encodes 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 (EPM1). One type of mutation responsible for EPM1 is the expansion in the promoter region of this gene of a CCCCGCCCCGCG repeat from 2-3 copies to 30-78 copies.

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Anti-CSTB Neutralizing Antibody (V3S-0822-YC926) (CAT#: V3S-0822-YC926)

Target: CSTB

Host Species: Mouse

Target Species: Human,

Application: Neut,WB,