Products

Enzyme-linked Immunoabsorbent Assay

Western Blot (Recombinant protein)

Antibody Production

Protein Array

Entrez GeneID

GeneBank Accession#

Protein Accession#

Gene Name

CSTB

Gene Alias

CST6, EPM1, PME, STFB

Gene Description

cystatin B (stefin B)

Omim ID

Gene Ontology

Gene Summary

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). [provided by RefSeq

Other Designations

CPI-B|cystatin B|liver thiol proteinase inhibitor|stefin B