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ProductsBack/LYAG antibody [C2C3], C-term

LYAG antibody [C2C3], C-term

GTX109821
GeneTex
ApplicationsWestern Blot, ImmunoHistoChemistry, ImmunoHistoChemistry Paraffin
DownloadDatasheet
Product group Antibodies
TargetGAA
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Overview

  • Supplier
    GeneTex
  • Product Name
    LYAG antibody [C2C3], C-term
  • Delivery Days Customer
    9
  • Application Supplier Note
    WB: 1:500-1:3000. IHC-P: 1:100-1:1000. *Optimal dilutions/concentrations should be determined by the researcher.Not tested in other applications.
  • Applications
    Western Blot, ImmunoHistoChemistry, ImmunoHistoChemistry Paraffin
  • Certification
    Research Use Only
  • Clonality
    Polyclonal
  • Concentration
    0.76 mg/ml
  • Conjugate
    Unconjugated
  • Gene ID2548
  • Target name
    GAA
  • Target description
    alpha glucosidase
  • Target synonyms
    acid maltase; aglucosidase alfa; glucosidase alpha, acid; LYAG; lysosomal alpha-glucosidase
  • Host
    Rabbit
  • Isotype
    IgG
  • Protein IDP10253
  • Protein Name
    Lysosomal alpha-glucosidase
  • Scientific Description
    This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompes disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene. [provided by RefSeq]
  • Storage Instruction
    -20°C or -80°C,2°C to 8°C
  • UNSPSC
    12352203

References

  • Rare Variants in Autophagy and Non-Autophagy Genes in Late-Onset Pompe Disease: Suggestions of Their Disease-Modifying Role in Two Italian Families. Napolitano F et al., 2021 Mar 31, Int J Mol Sci
    Read more
  • Efficient therapy for refractory Pompe disease by mannose 6-phosphate analogue grafting on acid alpha-glucosidase. Basile I et al., 2018 Jan 10, J Control Release
    Read more

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Figure 1. Western blot analysis of GAA using anti-GAA antibody (A01548). 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 A549 whole cell lysates, Lane 2: human HepG2 whole cell lysates, Lane 3: human HEK293 whole cell lysates, Lane 4: human PC-3 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-GAA antigen affinity purified polyclonal antibody (Catalog # A01548) 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 1:10000 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 GAA at approximately 110,95,76,70KD. The expected band size for GAA is at 110,95,76,70KD.
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