Cystatin B/Stefin B Products

Description

The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 cystatins (stefins), type 2 cystatins and kininogens. 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).

Bioinformatics

Entrez	1476 Human 25308 Rat
Uniprot	AAH03370 Human AAH10532 Human NM_000100 Human NP_000091 Human NP_000091.1 Human P04080 Human Q62426 Mouse
Product By Gene ID	1476
Alternate Names	CPI-B CST6cystatin B (liver thiol proteinase inhibitor)10STFBcystatin-B cystatin B (stefin B) EPM1 Liver thiol proteinase inhibitor PME stefin B stefin-B Stefin-B

Research Areas for Cystatin B/Stefin B

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Cancer