Anti-Stefin B/CSTB Antibody Picoband® fluoro550 Vervoeging

Cystatin B (CSTB), also called STFB, is a small protein that is a member of the superfamily of cysteine protease inhibitors. It has been isolated from human spleen and liver and its amino acid sequence has been fully determined. The cystatin B gene is located on 21q22.3. It is widely distributed and is localized mostly intracellularly, but has been found extracellularly. The protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. Its role is though to be as a protector against the proteinases leaking from lysosomes. A cystatin B multiprotein complex might have a specific cerebellar function, and that the loss of this function might contribute to the etiopathogenesis of EPM1. Upon differentiation to myotubes, CSTB becomes excluded from the nucleus and lysosomes, suggesting that the subcellular distribution of CSTB is dependent on the differentiation status of the cell.

Cystatin-B; CPI-B; Liver thiol proteinase inhibitor; Stefin-B; CSTB; CST6, STFB

CSTB

1476

P04080

Human

No cross-reactivity with other proteins

A synthetic peptide corresponding to a sequence in the middle region of human Stefin B.

Flow Cytometry

Cell Biology, Protease Inhibitors, Proteolysis/Ubiquitin

Immunogen affinity purified.

Liquid

This is an intracellular thiol proteinase inhibitor. Tightly binding reversible inhibitor of cathepsins L, H and B.

1. Alakurtti, K., Weber, E., Rinne, R., Theil, G., de Haan, G.-J., Lindhout, D., Salmikangas, P., Saukko, P., Lahtinen, U., Lehesjoki, A.-E. Loss of lysosomal association of cystatin B proteins representing progressive myoclonus epilepsy, EPM1, mutations. Europ. J. Hum. Genet. 13: 208-215, 2005. Note: Erratum: Europ. J. Hum. Genet. 13: 264 only, 2005. 2. Antonarakis, S. Personal Communication. Geneva, Switzerland 4/8/1997. 3. Bespalova, I. N., Adkins, S., Pranzatelli, M., Burmeister, M. Novel cystatin B mutation and diagnostic PCR assay in an Unverricht-Lundborg progressive myoclonus epilepsy patient. Am. J. Med. Genet. 74: 467-471, 1997.

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

11 kDa

No cross reactivity with other proteins.

6

Cystatin-B

Cytoplasm . Nucleus .

Belongs to the cystatin family.

Cystatin-B

Rabbit IgG

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.