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Description
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). [provided by RefSeq
Specifications
Specifications
| Antigen | CSTB |
| Applications | ELISA, Western Blot |
| Classification | Monoclonal |
| Clone | 2F1 |
| Conjugate | Unconjugated |
| Description | Mouse monoclonal antibody raised against full length recombinant CSTB. |
| Dilution | Western Blot (1:1000-1:3000) The optimal working dilution should be determined by the end user. |
| Formulation | In PBS, pH 7.4 (0.09% sodium azide) |
| Gene | CSTB |
| Gene Accession No. | NM_000100 |
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