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.
Applications:Suitable for use in Western Blot, Immunohistochemistry, Immunofluorescence, and ELISA.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000Immunohistochemistry (paraffin): 1:100-1:300Immunofluorescence: 1:200-1:1000ELISA: 1:20,000Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：30ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, 0.5% BSA, 0.02% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of human Cystatin B. Species Crossreactivity: rat
Isotype：IgG
Calc Applications Abbrev：E IF IHC WB
Calc Crossreactivity：Hu Rt
Immunogen：Synthetic peptide corresponding to aa20-100 of human Cystatin B.
