Anti-GAA Rabbit Polyclonal Antibody for WB, IF/ICC, ELISA - P10253

Core Product Specifications and Parameters

Biological Background: GAA Function and Localization

• Lysosomal alpha-glucosidase (GAA), also known as acid maltase or alglucosidase alfa, is a glycoside hydrolase belonging to the glycosidase family.
• Essential for the degradation of glycogen in lysosomes. GAA deficiency causes glycogen storage disease type II (Pompe disease). Related references: PMID:14695532 PMID:18429042 PMID:1856189
• Hydrolyzes both alpha-1,4 and alpha-1,6 glycosidic linkages in glycogen, with highest activity on alpha-1,4-linked glucans. Related references: PMID:29061980
• Subcellular localization is primarily in the lysosome lumen and associated with the lysosomal membrane.
• Polymorphisms and mutations in the GAA gene are directly linked to Pompe disease, a lysosomal storage disorder (keyword: Disease variant, Glycogen storage disease).
• GAA undergoes post-translational modifications including glycosylation (glycoprotein) and phosphorylation (phosphoprotein), and contains structural disulfide bonds.
• Synthesized with a signal peptide that directs it to the lysosome, where proteolytic processing generates the mature enzyme.
• High-resolution 3D structure has been determined, and the protein has been identified by direct protein sequencing and mass spectrometry-based proteomics (keywords: 3D-structure, Direct protein sequencing, Proteomics identification).

Experimental Guidance and Technical Tips

• The immunogen (residues 350–450) lies within a conserved region of human GAA; verify cross-reactivity for non-human samples by sequence alignment.
• For Western blot, the predicted full-length molecular weight is 105 kDa; processed lysosomal forms may appear as lower bands. Use appropriate protease inhibitors during sample preparation.
• As a polyclonal antibody, batch-to-batch consistency should be monitored; consider titrating the antibody for each new lot.
• In immunofluorescence, co-stain with a lysosomal marker (e.g., LAMP1) to confirm specific lysosomal localization.
• For ELISA applications, assess binding to coated recombinant GAA protein under optimized conditions before use in quantitative assays.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio simplifies IVD raw material procurement by offering access to high-quality antibodies like this anti-GAA polyclonal antibody, relevant for lysosomal storage disorder research. Our supply network includes validated antibody pairs, bulk ancillary reagents, and custom sourcing for rare targets. Whether you need reagents for glycogen degradation studies or diagnostic kit development, we bridge your needs from concept to clinic.