Cystatin B Proteins, Antibodies, cDNA Clones, ELISA Kits Research Reagents

All Cystatin B reagents are produced in house and quality controlled, including 4 Cystatin B Antibody, 1 Cystatin B ELISA, 30 Cystatin B Gene, 1 Cystatin B IP Kit, 1 Cystatin B Protein, 1 Cystatin B qPCR. All Cystatin B reagents are ready to use.

Cystatin B Protein (1)

    Cystatin B Antibody (4)

      Cystatin B ELISA Kit & Match Antibody ELISA Pair Set (1)

      Cystatin B cDNA Clone (30)

      Cystatin B qPCR Primer (1)

      Cystatin B Background

      Cystatin-B, also known as CPI-B, Liver thiol proteinase inhibitor, Stefin-B, CSTB and CST6, is a cytoplasm and nucleus protein which belongs to thecystatin family. Cystatin-B / CSTB is an intracellular thiol proteinase inhibitor. Tightly binding reversible inhibitor of cathepsins L, H and B. Cystatin-B / CSTB is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. Cystatin-B / CSTB is also thought to play a role in protecting against the proteases leaking from lysosomes. Defects in Cystatin-B / CSTB are the cause of progressive myoclonic epilepsy type 1 (EPM1) which is an autosomal recessive disorder characterized by severe, stimulus-sensitive myoclonus and tonic-clonic seizures. The cystatins are a family of cysteine protease inhibitors with homology to chicken cystatin. Cystatins are physiological inhibitors of cysteine proteinases which are widely distributed in human tissues and fluids. Cystatins typically comprise about 115 amino acids, are largely acidic, contain four conserved cysteine residues known to form two disulfide bonds. Cystatins may be glycosylated and / or phosphorylated, with similarity to fetuins, kininogens, stefins, histidine-rich glycoproteins and cystatin-related proteins. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired inhibitory activity. Cystatins mainly inhibit peptidases belonging to peptidase families C1 (papain family) and C13 (legumain family).

      Cystatin B References

      • Ritonja A., et al.,(1985), Amino acid sequence of the intracellular cysteine proteinase inhibitor cystatin B from human liver. Biochem. Biophys. Res. Commun. 131:1187-1192.
      • Pennacchio L.A., et al., (1996), Mutations in the gene encoding cystatin B in progressive myoclonus epilepsy (EPM1).Science 271:1731-1734.
      • Hattori M., et al.,(2000), The DNA sequence of human chromosome 21.Nature 405:311-319.

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