CSTB is 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 CSTB gene are responsible for the primary defects in patients with progressive myoclonic epilepsy 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.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).
Applications:Suitable for use in Immunohistochemistry, Western Blot.
Other applications not tested.
Recommended Dilution:Optimal 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：100ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by affinity chromatography.
Form：Supplied as a liquid in PBS, 2% sucrose, 0.09% sodium azide.
Specificity：Recognizes human CSTB.
Isotype：IgG
Calc Applications Abbrev：IHC WB
Calc Crossreactivity：Hu
Immunogen：Synthetic peptide corresponding to the N-terminal region of human CSTB
