Overview

Description

  • Nature
    Synthetic
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      C-QTNKAKHDELTYF
    • Amino acids
      86 to 98

Associated products

Specifications

Our Abpromise guarantee covers the use of ab23292 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    Blocking - Blocking peptide for Anti-Stefin B antibody (ab15970)

  • Form
    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

General Info

  • Alternative names
    • CHROW21
    • CPI B
    • CPI-B
    • CST 6
    • CST6
    • CSTB
    • Cystatin B
    • Cystatin B (stefin B)
    • Cystatin-B
    • CYTB
    • CYTB_HUMAN
    • EPM1
    • EPM1A
    • Liver thiol proteinase inhibitor
    • PME
    • Stefin-B
    • STF B
    • STFB
    • ULD
    see all
  • Function
    This is an intracellular thiol proteinase inhibitor. Tightly binding reversible inhibitor of cathepsins L, H and B.
  • Involvement in disease
    Defects in CSTB are the cause of progressive myoclonic epilepsy type 1 (EPM1) [MIM:254800]. 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.
  • Sequence similarities
    Belongs to the cystatin family.
  • Cellular localization
    Cytoplasm. Nucleus.
  • Information by UniProt

References

ab23292 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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