The GAA Monoclonal Antibody (CAB19234) is a high-quality antibody developed for reliable detection and analysis of target proteins. This antibody, raised in rabbits, is highly specific for human GAA samples and is validated for use in various applications, including Western blot and immunohistochemistry.GAA plays a crucial role in glycogen metabolism, specifically in lysosomes where it breaks down glycogen into glucose. Mutations in the GAA gene can lead to glycogen storage diseases, where glycogen accumulates in various tissues causing damage and dysfunction.
This antibody is validated for use in WB, ELISA applications and has demonstrated reactivity against Human samples.
Product Name:
GAA Monoclonal Antibody
SKU:
CAB19234
Size:
20μL, 100μL
Reactivity:
Human
Clone Number:
ARC2392
Conjugate:
Unconjugated
Immunogen:
Synthetic peptide. This information is considered to be commercially sensitive.
Recommended starting concentration is 1 μg/mL. Please optimize the concentration based on your specific assay requirements.
Synonyms:
LYAG, GAA
Positive Sample:
293T, HepG2, DU145
Cellular Localization:
Lysosome, Lysosome Membrane.
Calculated MW:
105kDa
Observed MW:
76kDa/105kDa
This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. 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. Alternative splicing results in multiple transcript variants.
Purification Method
Affinity purification
Gene ID
2548
Buffer Information
Store at -20℃. Avoid freeze / thaw cycles. Buffer: PBS containing 50% glycerol and 0.05% BSA, preserved with proclin300 or sodium azide, pH 7.3.
Western blot analysis of various lysates using GAA Rabbit mAb (CAB19234) at 1:1000 dilution. Secondary antibody: HRP-conjugated Goat anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25μg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (AbGn00020). Exposure time: 10s.
