Anti-GAA Antibody Picoband™

Product Name

Anti-GAA Antibody Picoband™

View all LYAG/GAA Antibodies

SKU/Catalog Number

A01548

Size

100 μg/vial

Form

Lyophilized

Description

Boster Bio Anti-GAA Antibody Picoband™ catalog # A01548. Tested in IHC, WB applications. This antibody reacts with Human.

Storage & Handling

Store at -20˚C for one year from date of receipt. After reconstitution, at 4˚C for one month. It can also be aliquotted and stored frozen at -20˚C for six months. Avoid repeated freeze-thaw cycles.

Cite This Product

Anti-GAA Antibody Picoband™ (Boster Biological Technology, Pleasanton CA, USA, Catalog # A01548)

Host

Rabbit

Contents

Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.

Clonality

Polyclonal

Isotype

Rabbit IgG

Immunogen

A synthetic peptide corresponding to a sequence in the middle region of human GAA, different from the related mouse sequence by eight amino acids, and from the related rat sequence by six amino acids.

*Blocking peptide can be purchased. Costs vary based on immunogen length. Contact us for pricing.

Cross-reactivity

No cross-reactivity with other proteins

Reactive Species

A01548 is reactive to GAA in Human

Applications

A01548 is guaranteed for IHC, WB Boster Guarantee

Observed Molecular Weight

110 kDa, 95kDa, 76kDa, 70 kDa

Calculated molecular weight

105.324kDa

Background of LYAG/GAA

Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene. This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Alternative splicing results in multiple transcript variants.

Antibody Validation

Boster validates all antibodies on WB, IHC, ICC, Immunofluorescence, and ELISA with known positive control and negative samples to ensure specificity and high affinity, including thorough antibody incubations.

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Reconsitution

Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Assay Dilutions Recommendation

The recommendations below provide a starting point for assay optimization. The actual working concentration varies and should be decided by the user.

Immunohistochemistry (Paraffin-embedded Section), 0.5-1μg/ml, Human, By Heat
Western blot, 0.1-0.5μg/ml, Human

Validation Images & Assay Conditions

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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 μg/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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Figure 2. IHC analysis of GAA using anti-GAA antibody (A01548).
GAA was detected in paraffin-embedded section of human liver cancer tissues. Heat mediated antigen retrieval was performed in citrate buffer (pH6, epitope retrieval solution) for 20 mins. The tissue section was blocked with 10% goat serum. The tissue section was then incubated with 1μg/ml rabbit anti-GAA Antibody (A01548) overnight at 4°C. Biotinylated goat anti-rabbit IgG was used as secondary antibody and incubated for 30 minutes at 37°C. The tissue section was developed using Strepavidin-Biotin-Complex (SABC)(Catalog # SA1022) with DAB as the chromogen.

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Figure 3. IHC analysis of GAA using anti-GAA antibody (A01548).
GAA was detected in paraffin-embedded section of human prostatic cancer tissues. Heat mediated antigen retrieval was performed in citrate buffer (pH6, epitope retrieval solution) for 20 mins. The tissue section was blocked with 10% goat serum. The tissue section was then incubated with 1μg/ml rabbit anti-GAA Antibody (A01548) overnight at 4°C. Biotinylated goat anti-rabbit IgG was used as secondary antibody and incubated for 30 minutes at 37°C. The tissue section was developed using Strepavidin-Biotin-Complex (SABC)(Catalog # SA1022) with DAB as the chromogen.

Gene/Protein Information For GAA (Source: Uniprot.org, NCBI)

Alternative Names

Acid alpha-Glucosidase; Acid Maltase; Aglucosidase alfa; EC 3.2.1.20; GAA; glucosidase, alpha; acid; LYAG; Lysosomal alphaGlucosidase; Lysosomal alpha-Glucosidase GAA LYAG alpha glucosidase lysosomal alpha-glucosidase|acid maltase|aglucosidase alfa|glucosidase alpha, acid

For more info on GAA, check out the GAA Infographic

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In this infographic, you will see the following information for GAA: database IDs, superfamily, protein function, synonyms, molecular weight, chromosomal locations, tissues of expression, subcellular locations, post-translational modifications, and related diseases, research areas & pathways. If you want to see more information included, or would like to contribute to it and be acknowledged, please contact [email protected].

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