GAA antibody

Catalog No. ABIN7880880

The Mouse Monoclonal anti-GAA antibody is suitable to detect GAA in samples from Human. It has been validated for WB, IHC (p) and IF.

Quick Overview for GAA antibody (ABIN7880880)

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

Reactivity: Human

Host: Mouse

Clonality: Monoclonal

Conjugate: This GAA antibody is un-conjugated

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

Clone: 2G7

Product Details anti-GAA Antibody

Purpose: Glucosidase Alpha Acid Antibody / GAA

Sequence: TALAWWEDMV AEFHDQVPFD GMWIDMNEPS NFIR

Purification: Affinity purified

Immunogen: Amino acids TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR from the human protein were used as the immunogen for the Glucosidase Alpha Acid antibody.

Isotype: IgG2b

Application Details

Application Notes: Optimal dilution of the Glucosidase Alpha Acid antibody should be determined by the researcher.

Restrictions: For Research Use only

Handling

Format: Lyophilized

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

Storage: 4 °C,-20 °C

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

Target Details for GAA

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

Alternative Name: 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