84-5449-19　［取扱停止］Cystatin B 100ul　535356

特徴

• 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 Immunohistochemistry, ELISA.
• Other applications not tested.
• Recommended Dilution:Immunohistochemistry (paraffin): 1:50-1:200ELISA: 1:10,000-1:20,000Optimal 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.