Mouse Anti-GAA Monoclonal Antibody – Cat# MBS6152607

Western Blot detection against Immunogen (36.96kD).

GAA Antibody – Mouse Monoclonal

Name: GAA Antibody; Monoclonal; Mouse; Human-Reactive; MyBioSource
Brand: MyBioSource
SKU: MBS6152607
Price: 1140 USD
Availability: InStock
Rating: 5 (10 reviews)

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS6152607

MBS6152607 is an antibody targeting GAA. Also known as GAA (Lysosomal alpha-glucosidase, Acid Maltase, Aglucosidase alfa) (HRP). Reactive in Human. Host: Mouse. Essential for the degradation of glygogen to glucose in lysosomes. More details

$1140 / 0.1 mL +1 FREE 8GB USB

Product Documentation

Sample Manual Insert View PDF

Testing Data View PDF

Certificate of Analysis (COA) View PDF

QC Report View PDF

Material Safety Data Sheet View PDF

Technical Datasheet View PDF

Protocol View PDF

Product Overview

Online Datasheet in PDF

Product Name

GAA, Monoclonal Antibody

Full Product Name

GAA (Lysosomal alpha-glucosidase, Acid Maltase, Aglucosidase alfa) (HRP)

Product Gene Name

anti-GAA antibody

Clonality

Monoclonal

Isotype

IgG1,k

Clone Number

3C6

Host

Mouse

Species Reactivity

Human

Form/Format

Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with horseradish peroxidase (HRP).

Purity/Purification

Purified by Protein A Affinity Chromatography.

Specificity

Recognizes human GAA.

Preparation and Storage

Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Note: Sodium azide is a potent inhibitor of peroxidase and should not be added to HRP conjugates. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Immunogen

Partial recombinant corresponding to aa851-952 from human Gaa(AAH40431) with GST tag. MW of the GST tag alone is 26kD.

Immunogen Sequence

GEARGELFWDDGESLEVLERGAYTQVIFLARNNTIVNELVRVTSEGAGLQLQKVTVLGVATAPQQVLSNGVPVSNFTYSPDTKVLDICVSLLMGEQFLVSWC

Conjugate

HRP

Product Categories/Family for anti-GAA antibody

Antibodies; Enzymes

Applications Tested/Suitable for anti-GAA antibody

ELISA (EIA), Western Blot (WB)

Application Notes for anti-GAA antibody

Applications are based on unconjugated antibody.

Product References and Citations for anti-GAA antibody

1. Discovery of a novel non-iminosugar acid alpha glucosidase chaperone series. Xiao J, Westbroek W, Motabar O, Lea WA, Hu X, Velayati A, Zheng W, Southall N, Gustafson AM, Goldin E, Sidransky E, Liu K, Simeonov A, Ribes A, Matalonga L, Ferrer M, Marugan JJ, Tamargo RJ.J Med Chem. 2012 Jul 26. 2. Glycosylation-related gene expression is linked to differentiation status in glioblastomas undifferentiated cells. Cheray M, Petit D, Forestier L, Karayan-Tapon L, Maftah A, Jauberteau MO, Battu S, Gallet FP, Lalloue F.Cancer Letters (2011), doi: 10.1016/ j.canlet.2011.07.027

Other Notes

Small volumes of anti-GAA antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Additional Details

NCBI/Uniprot data below describe general gene information for GAA. It may not necessarily be applicable to this product.

NCBI GI #

26251856

NCBI GeneID

2548

NCBI Accession #

BC040431

NCBI Related Accession #

AAH40431

UniProt Related Accession #

P10253

NCBI Official Full Name

Homo sapiens glucosidase, alpha; acid, mRNA

NCBI Official Synonym Full Names

glucosidase alpha, acid

NCBI Official Symbol

GAA

NCBI Official Synonym Symbols

LYAG

Protein Family

Lysosomal alpha-glucosidase

NCBI Protein Information

lysosomal alpha-glucosidase

NCBI Summary for GAA

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. [provided by RefSeq, Jan 2016]

Research Articles on GAA

1. Through blind predictions of residual enzymatic activity in human acid alpha-glucosidase (GAA) mutants, data demonstrate that gene-specific features can provide clues for investigating origin of variant pathogenicity, particularly for variants that are poorly predicted by existing methods.

Precautions

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

Disclaimer

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.

Product Note

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual, it is intended to serve as an example only. Please refer to the instructions For Use provided with the assay kit for precise details.