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ProductsBack/Galactosidase alpha antibody [N1C2]

Galactosidase alpha antibody [N1C2]

GTX101178
GeneTex
ApplicationsImmunoPrecipitation, Western Blot, ImmunoHistoChemistry, ImmunoHistoChemistry Paraffin
DownloadDatasheet
Product group Antibodies
TargetGLA
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Overview

  • Supplier
    GeneTex
  • Product Name
    Galactosidase alpha antibody [N1C2]
  • Delivery Days Customer
    9
  • Application Supplier Note
    WB: 1:500-1:3000. IHC-P: 1:100-1:1000. IP: 1:100-1:500. *Optimal dilutions/concentrations should be determined by the researcher.Not tested in other applications.
  • Applications
    ImmunoPrecipitation, Western Blot, ImmunoHistoChemistry, ImmunoHistoChemistry Paraffin
  • Certification
    Research Use Only
  • Clonality
    Polyclonal
  • Concentration
    0.16 mg/ml
  • Conjugate
    Unconjugated
  • Gene ID2717
  • Target name
    GLA
  • Target description
    galactosidase alpha
  • Target synonyms
    agalsidase alfa; alpha-D-galactosidase A; alpha-D-galactoside galactohydrolase 1; alpha-gal A; alpha-galactosidase A; GALA; galactosylgalactosylglucosylceramidase GLA; melibiase
  • Host
    Rabbit
  • Isotype
    IgG
  • Protein IDP06280
  • Protein Name
    Alpha-galactosidase A
  • Scientific Description
    This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties. [provided by RefSeq]
  • Storage Instruction
    -20°C or -80°C,2°C to 8°C
  • UNSPSC
    12352203

References

  • Modeling of Fabry disease nephropathy using patient derived human induced pluripotent stem cells and kidney organoid system.
    Read more
  • Rapid Clathrin-Mediated Uptake of Recombinant alpha-Gal-A to Lysosome Activates Autophagy. Ivanova MM et al., 2020 May 30, Biomolecules
    Read more
  • The lysosomal enzyme alpha-Galactosidase A is deficient in Parkinsons disease brain in association with the pathologic accumulation of alpha-synuclein. Nelson MP et al., 2018 Feb, Neurobiol Dis
    Read more

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Figure 1. Western blot analysis of Galactosidase alpha/Gla using anti-Galactosidase alpha/Gla antibody (A01135-2). Electrophoresis was performed on a 5-20% SDS-PAGE gel at 70V (Stacking gel) / 90V (Resolving gel) for 2-3 hours. The sample well of each lane was loaded with 50ug of sample under reducing conditions. Lane 1: human HELA whole cell lysates, Lane 2: human MCF-7 whole cell lysates, Lane 3: human HEK293 whole cell lysates. After Electrophoresis, proteins were transferred to a Nitrocellulose membrane at 150mA for 50-90 minutes. Blocked the membrane with 5% Non-fat Milk/ TBS for 1.5 hour at RT. The membrane was incubated with rabbit anti-Galactosidase alpha/Gla antigen affinity purified polyclonal antibody (Catalog # A01135-2) at 0.5 microg/mL overnight at 4°C, then washed with TBS-0.1%Tween 3 times with 5 minutes each and probed with a goat anti-rabbit IgG-HRP secondary antibody at a dilution of 1:5000 for 1.5 hour at RT. The signal is developed using an Enhanced Chemiluminescent detection (ECL) kit (Catalog # EK1002) with Tanon 5200 system. A specific band was detected for Galactosidase alpha/Gla at approximately 49KD. The expected band size for Galactosidase alpha/Gla is at 49KD.
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