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CSTB Protein (AA 1-98, full length) (GST tag)

CSTB Origine: Humain Hôte: Escherichia coli (E. coli) Recombinant 95 % SDS, WB, ELISA
N° du produit ABIN7479584
  • Antigène Voir toutes CSTB Protéines
    CSTB (Cystatin B (Stefin B) (CSTB))
    Type de proteíne
    Recombinant
    Attributs du protein
    AA 1-98, full length
    Origine
    • 5
    • 3
    • 3
    • 3
    • 1
    • 1
    • 1
    • 1
    • 1
    Humain
    Source
    • 9
    • 7
    • 1
    • 1
    • 1
    Escherichia coli (E. coli)
    Purification/Conjugué
    Cette CSTB protéine est marqué à la GST tag.
    Application
    SDS-PAGE (SDS), Western Blotting (WB), ELISA
    Séquence
    MMCGAPSATQ PATAETQHIA DQVRSQLEEK ENKKFPVFKA VSFKSQVVAG TNYFIKVHVG DEDFVHLRVF QSLPHENKPL TLSNYQTNKA KHDELTYF
    Pureté
    95 %
    Top Product
    Discover our top product CSTB Protéine
  • Restrictions
    For Research Use only
  • Format
    Lyophilized
    Buffer
    PBS buffer, 20mM GSH.
    Stock
    4 °C
  • Antigène
    CSTB (Cystatin B (Stefin B) (CSTB))
    Autre désignation
    Cystatin-B (CSTB Produits)
    Synonymes
    CST6 Protein, EPM1 Protein, EPM1A Protein, PME Protein, STFB Protein, ULD Protein, CSTB Protein, Cyb Protein, Epm1 Protein, Stfb Protein, MGC189005 Protein, pme Protein, cst6 Protein, epm1 Protein, stfb Protein, AA960480 Protein, ARABIDOPSIS THALIANA PHYTOCYSTATIN 6 Protein, ATCYS6 Protein, ATCYSB Protein, cystatin B Protein, LOC100008600 Protein, LOC100136235 Protein, CPI1 Protein, cystatin B Protein, cystatin B (stefin B) Protein, cystatin 14a, tandem duplicate 2 Protein, cystatin-B Protein, CSTB Protein, Cstb Protein, cstb Protein, cst14a.2 Protein, CYSB Protein, cpi1 Protein, LOC101123265 Protein
    Sujet
    Background: 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) [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. Synonyms: CPI-B Liver thiol proteinase inhibitor Stefin-B.
    Poids moléculaire
    38.5 kD
    NCBI Accession
    NM_000100
    UniProt
    P04080
    Pathways
    Response to Water Deprivation
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