LYAG/GAA Antibody (3C6)

Images

 
Western Blot: LYAG/GAA Antibody (3C6) [H00002548-M01] - Western Blot detection against Immunogen (36.96 KDa)
ELISA: LYAG/GAA Antibody (3C6) [H00002548-M01] - Detection limit for recombinant GST tagged GAA is 0.3 ng/ml as a capture antibody.

Product Details

Summary
Reactivity HuSpecies Glossary
Applications WB, ELISA
Clone
3C6
Clonality
Monoclonal
Host
Mouse
Conjugate
Unconjugated

Order Details

LYAG/GAA Antibody (3C6) Summary

Immunogen
GAA (AAH40431 851 a.a. - 952 a.a.) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. GEARGELFWDDGESLEVLERGAYTQVIFLARNNTIVNELVRVTSEGAGLQLQKVTVLGVATAPQQVLSNGVPVSNFTYSPDTKVLDICVSLLMGEQFLVSWC
Localization
Lysosome
Specificity
GAA - glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II)
Isotype
IgG1 Kappa
Clonality
Monoclonal
Host
Mouse
Gene
GAA
Purity
IgG purified
Innovator's Reward
Test in a species/application not listed above to receive a full credit towards a future purchase.

Applications/Dilutions

Dilutions
  • Western Blot
  • ELISA
Application Notes
Antibody reactivity against Recombinant Protein with GST tag on ELISA and WB. GST tag alone is used as a negative control.
Control
LYAG/GAA Knockout 293T Cell Lysate
Publications
Read Publications using H00002548-M01.

Packaging, Storage & Formulations

Storage
Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles.
Buffer
PBS (pH 7.4)
Preservative
No Preservative
Purity
IgG purified

Notes

Quality control test: Antibody Reactive Against Recombinant Protein.

This product is produced by and distributed for Abnova, a company based in Taiwan.

Alternate Names for LYAG/GAA Antibody (3C6)

  • Acid alpha-Glucosidase
  • Acid Maltase
  • Aglucosidase alfa
  • EC 3.2.1.20
  • GAA
  • glucosidase, alpha; acid
  • LYAG
  • Lysosomal alphaGlucosidase
  • Lysosomal alpha-Glucosidase

Background

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 Pompe's 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.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

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Publications for LYAG/GAA Antibody (H00002548-M01)(3)


Showing Publications 1 - 3 of 3.
Publications using H00002548-M01 Applications Species
Cheray M, Petit D, Forestier L et al. Glycosylation-related gene expression is linked to differentiation status in glioblastomas undifferentiated cells. Cancer Letters Jul 21 2011
Xiao J, Westbroek W, Motabar O et al. Discovery of a novel non-iminosugar acid alpha glucosidase chaperone series. J Med Chem. 2012 Jul 26 [PMID: 22834902]
Cheray M, Petit D, Forestier L et al. Glycosylation-related gene expression is linked to differentiation status in glioblastomas undifferentiated cells. Cancer Letters. 2011 [PMID: 21899947]

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Product General Protocols

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Video Protocols

WB Video Protocol

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Control Lysate(s)

Secondary Antibodies

 

Isotype Controls

Other Available Formats

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Bioinformatics

Gene Symbol GAA
Entrez
OMIM
Uniprot