LYAG/GAA Antibody (2489C) [Alexa Fluor® 647]

• Reactivity: Hu
• Applications: WB, IHC
• Clone: 2489C
• Clonality: Monoclonal
• Host: Rabbit
• Conjugate: Alexa Fluor 647

LYAG/GAA Antibody (2489C) [Alexa Fluor® 647] Summary

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

Applications/Dilutions

• Dilutions:
  • Immunohistochemistry
  • Western Blot

Packaging, Storage & Formulations

• Storage: Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied
• Buffer: Supplied 0.2mg/ml in 1X PBS with RDF1 and 0.09% Sodium Azide

Notes

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Alternate Names for LYAG/GAA Antibody (2489C) [Alexa Fluor® 647]

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

Background

Acid alpha-glucosidase (GAA) is an enzyme that is essential in the degradation of glycogen to glucose in the lysosome (1). Defects in GAA are the cause of glycogen storage disease II, also known as Pompe's disease, which is a rare autosomal recessive metabolic disorder that damages muscle and nerve cells throughout the body, primarily due to the accumulation of glycogen in the lysosome (2). Pompe disease occurs in babies, children, and adults who inherit a defective GAA gene and affects an estimated 5,000 to 10,000 people worldwide (3). Enzyme replacement therapy (ERT) is used to treat patients with this disease (4, 5).

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.