商品介绍

gene_symbol：Stefin B
Synonyms：CPI-B; CST6; EPM1; EPM1A; PME; STFB; ULD
clone_name：OTI5F2
immunogen：Human recombinant protein fragment corresponding to amino acids 1-98 of human CSTB (NP_000091) produced in E.coli.
predicted_size：11 kDa
background：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). 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. [provided by RefSeq, Jul 2016]
buffer：PBS (pH 7.3) containing 1% BSA, 50% glycerol and 0.02% sodium azide.
purification：Purified from mouse ascites fluids or tissue culture supernatant by affinity chromatography (protein A/G)
isotype：IgG1
host：Mouse
applications：IHC, WB
Recommend Dilution：WB 1:500, IHC 1:500
reactivities：Human
storage：Store at -20°C as received.