Overview

  • Product nameAnti-GAA antibody
    See all GAA primary antibodies
  • Description
    Rabbit polyclonal to GAA
  • Tested applicationsSuitable for: WB, IHC-Pmore details
  • Species reactivity
    Reacts with: Mouse, Human
  • Immunogen

    Full length protein, corresponding to amino acids 1-952 of Human GAA.

  • Positive control
    • Mouse intestine tissue lysate. GAA transfected 293T cell lysate. IHC-P: Human placenta FFPE tissue sections

Properties

Applications

Our Abpromise guarantee covers the use of ab102815 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 1/500 - 1/1000. Predicted molecular weight: 105 kDa.
IHC-P Use a concentration of 5 µg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.

Target

  • FunctionEssential for the degradation of glygogen to glucose in lysosomes.
  • Involvement in diseaseDefects 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 similaritiesBelongs 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 localizationLysosome. 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

Anti-GAA antibody images

  • Anti-GAA antibody (ab102815) at 1/500 dilution + Mouse intestine lysate at 50 µg

    Predicted band size : 105 kDa
  • All lanes : Anti-GAA antibody (ab102815) at 1/500 dilution

    Lane 1 : GAA transfected 293T cell line
    Lane 2 : Non transfected 293T cell line

    Lysates/proteins at 25 µg per lane.


    Predicted band size : 105 kDa
  • IHC image of GAA staining in human placenta formalin fixed paraffin embedded tissue section, performed on a Leica BondTM system using the standard protocol F. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20 mins. The section was then incubated with ab102815, 5µg/ml, for 15 mins at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.

    For other IHC staining systems (automated and non-automated) customers should optimize variable parameters such as antigen retrieval conditions, primary antibody concentration and antibody incubation times.

References for Anti-GAA antibody (ab102815)

ab102815 has not yet been referenced specifically in any publications.

Product Wall

That seems like a good idea.

I would possible also suggest reducing the washing conditions used slightly, using 3x5 minute washes with TBST rather than extended periods.

Please do let me know how you get on.

Thank you for contacting us.

I now have some further information in regards to how the western blot presented on the datasheet of Anti-GAA antibody (ab102815) was performed.

5% non-fat milk in PBST (0.2%) was used to block the membr...

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I w...

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I am sorry to hear you are experiencing difficulties with one of our products. We take product complaints very seriously, and investigate every product that we feel may not be performing correctly.

I a...

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