Overview

  • Product name

    Anti-GAA antibody [EPR4716(2)]
    See all GAA primary antibodies
  • Description

    Rabbit monoclonal [EPR4716(2)] to GAA
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WBmore details
    Unsuitable for: Flow Cyt,ICC,IHC-P or IP
  • Species reactivity

    Reacts with: Mouse, Rat, Human
  • Immunogen

    Synthetic peptide corresponding to amio acids in Human GAA (UniProt ID: P10253).

  • Positive control

    • 293T cell lysates, fetal liver and Human placenta tissue lysates
  • General notes

     

     

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab137068 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB 1/1000 - 1/10000. Detects a band of approximately 110,95,76 kDa (predicted molecular weight: 105 kDa).
  • Application notes
    Is unsuitable for Flow Cyt,ICC,IHC-P or IP.
  • Target

    • Function

      Essential for the degradation of glygogen to glucose in lysosomes.
    • Involvement in disease

      Defects in GAA are the cause of glycogen storage disease type 2 (GSD2) [MIM:232300]; also called acid alpha-glucosidase (GAA) deficiency or acid maltase deficiency (AMD). GSD2 is a metabolic disorder with a broad clinical spectrum. The severe infantile form, or Pompe disease, presents at birth with massive accumulation of glycogen in muscle, heart and liver. Cardiomyopathy and muscular hypotonia are the cardinal features of this form whose life expectancy is less than two years. The juvenile and adult forms present as limb-girdle muscular dystrophy beginning in the lower limbs. Final outcome depends on respiratory muscle failure. Patients with the adult form can be free of clinical symptoms for most of their life but finally develop a slowly progressive myopathy.
    • Sequence similarities

      Belongs to the glycosyl hydrolase 31 family.
      Contains 1 P-type (trefoil) domain.
    • Post-translational
      modifications

      The different forms of acid glucosidase are obtained by proteolytic processing.
      Phosphorylation of mannose residues ensures efficient transport of the enzyme to the lysosomes via the mannose 6-phosphate receptor.
    • Cellular localization

      Lysosome. Lysosome membrane.
    • Information by UniProt
    • Database links

    • Alternative names

      • 70 kDa lysosomal alpha-glucosidase antibody
      • Acid alpha glucosidase antibody
      • Acid maltase antibody
      • Aglucosidase alfa antibody
      • Alpha glucosidase antibody
      • GAA antibody
      • Glucosidase alpha acid (Pompe disease glycogen storage disease type II) antibody
      • Glucosidase alpha acid antibody
      • Glucosidase alpha antibody
      • LYAG antibody
      • LYAG_HUMAN antibody
      • Lysosomal alpha glucosidase antibody
      see all

    Images

    • All lanes : Anti-GAA antibody [EPR4716(2)] (ab137068) at 1/1000 dilution

      Lane 1 : 293T cell lysate
      Lane 2 : Human Fetal liver tissue lysate
      Lane 3 : Human placenta tissue lysate

      Lysates/proteins at 10 µg per lane.

      Predicted band size: 105 kDa
      Observed band size: 110,75,95 kDa
      why is the actual band size different from the predicted?

    References

    This product has been referenced in:

    • Han SO  et al. Salmeterol with Liver Depot Gene Therapy Enhances the Skeletal Muscle Response in Murine Pompe Disease. Hum Gene Ther N/A:N/A (2019). Read more (PubMed: 30803275) »
    • Sakamaki JI  et al. Bromodomain Protein BRD4 Is a Transcriptional Repressor of Autophagy and Lysosomal Function. Mol Cell 66:517-532.e9 (2017). WB . Read more (PubMed: 28525743) »
    See all 4 Publications for this product

    Customer reviews and Q&As

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    Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
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