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GLA Protein (His tag)

GLA Spezies: Maus Wirt: HEK-293 Cells Recombinant > 95 % as determined by SDS-PAGE Active
Produktnummer ABIN7320092
  • Target Alle GLA Proteine anzeigen
    GLA (Galactosidase, alpha (GLA))
    Protein-Typ
    Recombinant
    Biologische Aktivität
    Active
    Spezies
    • 9
    • 2
    • 2
    • 1
    Maus
    Quelle
    • 7
    • 2
    • 2
    • 1
    • 1
    • 1
    HEK-293 Cells
    Aufreinigungstag / Konjugat
    Dieses GLA Protein ist gelabelt mit His tag.
    Verwendungszweck
    Recombinant Mouse alpha-Galactosidase A/GLA Protein (His Tag)(Active)
    Sequenz
    Met1-Arg421
    Produktmerkmale
    A DNA sequence encoding the mouse Gla (Q8BGZ6) (Met1-Arg421) was expressed with a C-terminal polyhistidine tag.
    Reinheit
    > 95 % as determined by SDS-PAGE
    Endotoxin-Niveau
    < 1.0 EU per μg of the protein as determined by the LAL method.
    Biological Activity Comment
    Measured by its ability to hydrolyze 4-methylumbelliferyl-α-D-galactopyranoside.The specific activity is > 400 pmoles/min/μg.
    Top Product
    Discover our top product GLA Protein
  • Beschränkungen
    Nur für Forschungszwecke einsetzbar
  • Format
    Lyophilized
    Rekonstitution
    Please refer to the printed manual for detailed information.
    Buffer
    Lyophilized from sterile PBS, pH 7.4
    Lagerung
    4 °C,-20 °C,-80 °C
    Informationen zur Lagerung
    Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80°C. Reconstituted protein solution can be stored at 4-8°C for 2-7 days. Aliquots of reconstituted samples are stable at < -20°C for 3 months.
  • Target
    GLA (Galactosidase, alpha (GLA))
    Andere Bezeichnung
    alpha-Galactosidase A/GLA (GLA Produkte)
    Synonyme
    GALA Protein, Ags Protein, zgc:101584 Protein, MGC130872 Protein, SMU.877 Protein, SCF11.21 Protein, AO090005000217 Protein, alpha-GAL Protein, galactosidase alpha Protein, galactosidase, alpha Protein, galactosidase alpha S homeolog Protein, alpha-galactosidase Protein, aga Protein, alpha-galactosidase A Protein, GLA Protein, Gla Protein, gla Protein, gla.S Protein, agaN Protein, aga Protein, agaL Protein, SCO0541 Protein, rafA Protein, melA Protein, galA Protein, ANI_1_2528074 Protein, ANI_1_1502124 Protein, AOR_1_390174 Protein, CpipJ_CPIJ002066 Protein, MCYG_00962 Protein, MCYG_00791 Protein, Tsp_02909 Protein, Tsp_02508 Protein
    Hintergrund

    Background: Alpha-galactosidase A, also known as Alpha-D-galactoside galactohydrolase, Alpha-D-galactosidase A, Melibiase and GLA, is a member of the glycosyl hydrolase 27 family. GLA is used as a long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease. Defects in GLA are the cause of Fabry disease (FD) which is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Deficiency of GLA leads to the accumulation of glycosphingolipids in the vasculature leading to multiorgan pathology. In addition to well-described microvascular disease, deficiency of GLA is also characterized by premature macrovascular events such as stroke and possibly myocardial infarction.

    Synonym: Ags

    Molekulargewicht
    45.6 kDa
    UniProt
    Q8BGZ6
    Pathways
    SARS-CoV-2 Protein Interaktom
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