Glucosidase Alpha Acid Antibody / GAA

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.

Product Specifications

CAS Number

9007-83-4

Specifications

Western blot: 0.5-1 µg/mL, Immunohistochemistry: 1-2 µg/mL, Immunofluorescence: 2-4 µg/mL

UniProt

P10253

Host

Mouse

Reactivity

Human

Immunogen

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

Clonality

Monoclonal

Isotype

IgG2b

Clone

2G7

Applications

WB, IHC-P, IF

Purity

Affinity purified

Format

Purified

Buffer

Lyophilized from 1X PBS with 2% Trehalose and 0.025% sodium azide

Reconstitution

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.

Limitations

This Glucosidase Alpha Acid antibody is available for research use only.

Storage Conditions

After reconstitution, the Glucosidase Alpha Acid 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.

Formulation

0.5 mg/mL if reconstituted with 0.2ml sterile DI water

Applications Notes

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

Location

Cytoplasmic, membranous

Image Legend

IHC staining of FFPE human prostate cancer with Glucosidase Alpha Acid antibody. HIER: boil tissue sections in pH8 EDTA for 20 min and allow to cool before testing.