LYAG/GAA Antibody (3C6)

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.

• Reactivity: Hu
• Applications: WB, ELISA
• Clone: 3C6
• Clonality: Monoclonal
• Host: Mouse
• Conjugate: Unconjugated

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

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

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.

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

Publications using H00002548-M01

• 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]

Bioinformatics

• Gene Symbol: GAA
• Entrez:
  • 2548(Human)
• OMIM:
  • 232(Human)
• Uniprot:
  • AAH40431(Human)