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Anti-Stefin B CSTB Antibody Picoband® (monoclonal, 2B6) Fluoro594 Conjugated

Product Specifications

Background

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 thought 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.

Synonyms

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

Gene Name

CSTB

Gene ID

1476

UniProt

P04080

Host

Mouse

Reactivity

Human

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

E. coli-derived human Stefin B recombinant protein (Position: M1-F98) . Human Stefin B shares 78.6 % amino acid (aa) sequence identity with both mouse and rat Stefin B.

Clonality

Monoclonal

Clone

Clone: 2B6

Tissue Specificity

Expressed in all adult and fetal tissues except brain and skin. More abundant in fetal tissues especially liver.

Applications

Flow Cytometry

Field of Research

Cell Biology, Protease Inhibitors, Proteolysis/Ubiquitin

Purification

Immunogen affinity purified.

Form

Liquid

Function

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

References & Citations

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.

Storage Conditions

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

Specificity

No cross reactivity with other proteins.

Applications Notes

6

Gene Name Synonym

Cystatin B (stefin B)

Subcellular Location

Cytoplasm

Isotype

Mouse IgG1

Contents

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

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