antibody

Anti-Stefin B antibody

MBS175615

0.1 mg

245 USD

polyclonal

rabbit

nonconjugated

mouse, rat

Antibody

Anti-Stefin B antibody

Stefin B

Cystatin-B; cystatin B(stefin B); CHROW21 antibody; CPI B antibody; CPI-B antibody; CST6 antibody; CSTB antibody; Cystatin B antibody; Cystatin-B antibody; CYTB antibody; CYTB_HUMAN antibody; EPM1 antibody; EPM1A antibody; Liver thiol proteinase inhibitor antibody; PME antibody; Stefin-B antibody; STFB antibody; ULD antibody

Cystatin-B; Cystatin-B; cystatin-B; stefin-B; cystatin B; Stefin-B

CSTB

Cstb; Cstb; Stfb; AA960480; Cst6; Stfb

CYTB_MOUSE

Polyclonal

IgG

Rabbit

Mouse, Rat

98

Immunogen affinity purified.

Lyophilized

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.

Western Blot (WB)

Immunogen: A synthetic peptide corresponding to a sequence in the middle region of mouse Stefin B (62-77aa DKCVHLRVFQPLPHEN), different from the related rat sequence by two amino acids.

Contents: Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3. Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Description: Rabbit IgG polyclonal antibody for Cystatin-B(CSTB) detection. Tested with WB in Mouse, Rat. 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 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.

2494022

Q62426.1

Q62426

11,046 Da

CSTB: This is an intracellular thiol proteinase inhibitor. Tightly binding reversible inhibitor of cathepsins L, H and B. Defects in CSTB are the cause of progressive myoclonic epilepsy type 1 (EPM1). EPM1 is an autosomal recessive disorder characterized by severe, stimulus-sensitive myoclonus and tonic-clonic seizures. The onset, occurring between 6 and 13 years of age, is characterized by convulsions. Myoclonus begins 1 to 5 years later. The twitchings occur predominantly in the proximal muscles of the extremities and are bilaterally symmetrical, although asynchronous. At first small, they become late in the clinical course so violent that the victim is thrown to the floor. Mental deterioration and eventually dementia develop. Belongs to the cystatin family. Protein type: Inhibitor. Cellular Component: extracellular space; cytoplasm; nucleolus; intracellular; nucleus. Molecular Function: protease binding; protease inhibitor activity; endopeptidase inhibitor activity; cysteine protease inhibitor activity. Biological Process: negative regulation of proteolysis; adult locomotory behavior; negative regulation of peptidase activity

0.1 mg

245 USD