Cystatin B Monoclonal Antibody

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.

Description
Cystatin B Monoclonal Antibody. Unconjugated. Raised in: Rabbit.

Formulation
Buffer: PBS containing 50% glycerol and 0.05% BSA, preserved with proclin300 or sodium azide, pH 7.3.

Specificity
Human, Mouse, Rat

Isotype
IgG

Uniprot ID
P04080

Purification
Affinity Purified

Immunogen
Synthetic peptide. Please contact EpigenTek for more information.

Storage
Store at -20°C. Avoid freeze / thaw cycles.

Alternative Names
PME; ULD; CST6; EPM1; STFB; CPI-B; EPM1A; Cystatin B

Application
WB, ELISA; Recommended dilution: WB, 1:500 - 1:1000; ELISA, Recommended starting concentration is 1 µg/mL. Please optimize the concentration based on your specific assay requirements.