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Galactosidase alpha antibody [N1C2]

GTX101178
GeneTex
ApplicationsImmunoPrecipitation, Western Blot, ImmunoHistoChemistry, ImmunoHistoChemistry Paraffin
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
    GALA, alpha-galactosidase A, agalsidase alfa, alpha-D-galactosidase A, alpha-D-galactoside galactohydrolase 1, alpha-gal A, 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

  • Cui S, Fang X, Lee H, et al. Modeling of Fabry disease nephropathy using patient derived human induced pluripotent stem cells and kidney organoid system. J Transl Med. 2023,21(1):138. doi: 10.1186/s12967-023-03992-0
    Read this paper
  • Ivanova MM, Dao J, Kasaci N, et al. Rapid Clathrin-Mediated Uptake of Recombinant α-Gal-A to Lysosome Activates Autophagy. Biomolecules. 2020,10(6). doi: 10.3390/biom10060837
    Read this paper
  • Nelson MP, Boutin M, Tse TE, et al. The lysosomal enzyme alpha-Galactosidase A is deficient in Parkinson's disease brain in association with the pathologic accumulation of alpha-synuclein. Neurobiol Dis. 2018,110:68-81. doi: 10.1016/j.nbd.2017.11.006
    Read this paper

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