GAA Rabbit Monoclonal Antibody for WB, ELISA - P10253

Core Product Specifications and Parameters

Biological Background: Lysosomal alpha-glucosidase (GAA) Function and Localization

• Lysosomal alpha-glucosidase (GAA), also known as acid maltase or aglucosidase alfa, is a hydrolase essential for glycogen degradation in lysosomes.
  Related references:
  PMID:14695532 PMID:18429042 PMID:1856189

• The enzyme exhibits highest activity toward alpha-1,4-linked glucosidic bonds and can also hydrolyze alpha-1,6-linked glucans, facilitating complete glycogen breakdown.
  Related references:
  PMID:29061980

• GAA is localized to the lysosome membrane, where it acts on glycogen particles delivered to lysosomes.

• Post-translational modifications, including N-linked glycosylation and phosphorylation, are critical for enzyme folding, stability, and lysosomal targeting.

• Mutations in GAA cause glycogen storage disease type II (Pompe disease), characterized by progressive glycogen accumulation in lysosomes, leading to muscle weakness and respiratory failure.

• The precursor protein contains a signal peptide and undergoes proteolytic processing to generate mature, active enzyme forms.

Experimental Guidance and Technical Tips

• The immunogen is a synthetic peptide spanning amino acids 100–200 of human GAA, allowing detection of denatured protein by WB. For native conditions, further validation may be needed.

• In WB experiments, consider using human cell lines (e.g., HeLa or HepG2) or tissue lysates as positive controls, and validate with relevant negative controls.

• For ELISA, the antibody can be applied as a capture or detection antibody. Optimizing pairing with another GAA-specific antibody may enhance assay sensitivity.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio specializes in providing diagnostic manufacturers and research laboratories with comprehensive access to IVD raw materials, from early-stage development to clinical application. This Anti-GAA monoclonal antibody supports glycogen metabolism research and Pompe disease studies, and we can supply validated antibody pairs, optimized monoclonal and polyclonal antibodies, bulk ancillary reagents, and rare target sourcing solutions tailored to your needs.