GAA antibody (AA 494-527)

Catalog No. ABIN5647392

This Rabbit Polyclonal antibody specifically detects GAA in WB and IHC (p). It exhibits reactivity toward Human and Rat.

Quick Overview for GAA antibody (AA 494-527) (ABIN5647392)

Target: GAA (Glucosidase, Alpha, Acid (GAA))

Reactivity: Human, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GAA antibody is un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Product Details anti-GAA Antibody

Binding Specificity: AA 494-527

Purification: Antigen affinity purified

Immunogen: Amino acids 494-527 (TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR-human) were used as the immunogen for the GAA antibody.

Isotype: IgG

Application Details

Application Notes: Optimal dilution of the GAA antibody should be determined by the researcher.\. Western blot: 0.5-1 μg/mL,Immunohistochemistry (FFPE): 1-2 μg/mL

Restrictions: For Research Use only

Handling

Buffer: 0.5 mg/mL if reconstituted with 0.2 mL sterile DI water

Storage: -20 °C

Storage Comment: After reconstitution, the GAA antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.

Target Details for GAA

Target: GAA (Glucosidase, Alpha, Acid (GAA))

Alternative Name: GAA / Glucosidase alpha acid

Background: Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene. 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.

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process