CSTB Antibody

• Background:

  CSTB is a stefin that functions as an intracellular thiol protease inhibitor. The protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. The protein is thought to play a role in protecting against the proteases leaking from lysosomes. Evidence indicates that mutations in CSTB gene are responsible for the primary defects in patients with progressive myoclonic epilepsy a stefin that functions as an intracellular thiol protease inhibitor. The protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. The protein is thought to play a role in protecting against the proteases leaking from lysosomes. Evidence indicates that mutations in this gene are responsible for the primary defects in patients with progressive myoclonic epilepsy.The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 cystatins (stefins), type 2 cystatins and kininogens. This gene encodes a stefin that functions as an intracellular thiol protease inhibitor. The protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. The protein is thought to play a role in protecting against the proteases leaking from lysosomes. Evidence indicates that mutations in this gene are responsible for the primary defects in patients with progressive myoclonic epilepsy (EPM1) .

• NCBI Gene ID:

  1476

• Swiss Prot:

  P04080

• Accession Number:

  NP_000091

• Host:

  Rabbit

• Reactivity:

  Human

• Immunogen:

  Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human CSTB.

• Clonality:

  Polyclonal

• Conjugation:

  Unconjugated

• Type:

  Primary Antibodies

• Field of Research:

  Other

• Purification:

  Antibody is purified by peptide affinity chromatography method.

• Positive Control:

  Tranfected 293T Cell Lysate

• Concentration:

  Batch dependent

• Buffer:

  Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

• Modification:

  None

• Shipping Conditions:

  Blue Ice

• Storage Conditions:

  For short periods of storage (days) store at 4˚ C. For longer periods of storage, store CSTB antibody at -20˚ C. As with any antibody avoid repeat freeze-thaw cycles.

• Calculated Molecular Weight:

  11 kDa

• Applications Notes:

  CSTB antibody can be used for detection of CSTB by ELISA at 1:12500. CSTB antibody can be used for detection of CSTB by western blot at 0.25 μ g/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.

• Symbol:

  CSTB

• NCBI Official Name:

  Cystatin B (stefin B)

• NCBI Organism:

  Homo sapiens

• Background Reference 01:

  Kenig, M., (2006) Proteins 62 (4), 918-927.

• Other Product Names:

  CSTB, CST6, EPM1, PME, STFB, ULD, EPM1A

• Tested Applications:

  ELISA, WB, IHC

• Protein ID:

  4503117

• Physical Properties:

  Liquid