LYAG/GAA Antibody (2489C) [Janelia Fluor® 669]

• Reactivity: Hu
• Applications: WB, IHC
• Clone: 2489C
• Clonality: Monoclonal
• Host: Rabbit
• Conjugate: Janelia Fluor 669

LYAG/GAA Antibody (2489C) [Janelia Fluor® 669] Summary

• Additional Information: Recombinant Monoclonal Antibody.
• Immunogen: Human embryonic kidney cell, HEK293-derived human Lysosomal alpha -Glucosidase protein
  Ala70-Cys952
  Accession # P10253
• Specificity: Detects human Lysosomal alpha -Glucosidase protein in direct ELISAs.
• Isotype: IgG
• Clonality: Monoclonal
• Host: Rabbit
• Purity: Protein A or G purified from cell culture supernatant

Applications/Dilutions

• Dilutions:
  • Immunohistochemistry
  • Western Blot
• Application Notes: Optimal dilution of this antibody should be experimentally determined.

Packaging, Storage & Formulations

• Storage: Store at 4C in the dark.
• Buffer: 50mM Sodium Borate
• Preservative: 0.05% Sodium Azide
• Purity: Protein A or G purified from cell culture supernatant

Notes

Sold under license from the Howard Hughes Medical Institute, Janelia Research Campus.

Alternate Names for LYAG/GAA Antibody (2489C) [Janelia Fluor® 669]

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

Background

GAA 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. [provided by RefSeq]

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.