Name: Anti-CSTB antibody
Price: 297.00 USD

Overview
- 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). One type of mutation responsible for EPM1 is the expansion in the promoter region of this gene of a CCCCGCCCCGCG repeat from 2-3 copies to 30-78 copies.
  
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Properties
- Host
  
  rabbit
  
  Antibody Type
  
  polyclonal
  
  Isotype
  
  IgG
  
  Reactivity
  
  human
  
  Immunogen
  
  Recombinant fusion protein containing a sequence corresponding to amino acids 1-98 of human CSTB (NP_000091. 1).
  
  Purification
  
  Affinity purification
  
  Conjugate
  
  Unconjugated
  
  Formulation
  
  PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  
  Storage
  
  Store at -20°C and avoid freeze-thaw cycles.
  
  Regulatory Status
  
  RUO
  
  Antigen
  
  Cystatin-B; Cpi-B; Liver Thiol Proteinase Inhibitor; Stefin-B (Gene ID:1476; UniProt:P04080)
  
  * For research use only