Polyclonal Anti-Cystatin B

Price

200.00 USD

Size

100ug/vial

Reactivities

Mouse, Rat

Format

Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3.

References

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.\n2. Antonarakis, S. Personal Communication. Geneva, Switzerland 4/8/1997.\n3. 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.\n

Storage Temp

At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time.Avoid repeated freezing and thawing.

Scientific 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.