GAA Antibody / Glucosidase alpha acid

This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. 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. Three transcript variants encoding the same protein have been found for this gene.

Product Specifications

CAS Number

9007-83-4

Specifications

Western blot: 1:500-1:2000, Immunohistochemistry (FFPE) : 1:25

UniProt

P10253

Host

Rabbit

Reactivity

Human

Immunogen

A portion of amino acids 174-203 from the human protein were used as the immunogen for the GAA antibody.

Clonality

Polyclonal

Isotype

Applications

WB, IHC-P

Purity

Antigen affinity purified

Format

Purified

Buffer

In 1X PBS, pH 7.4, with 0.09% sodium azide

Reconstitution

Aliquot the GAA antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.

Limitations

This GAA antibody is available for research use only.

Storage Conditions

Aliquot the GAA antibody and store frozen at -20°C or colder. Avoid repeated freeze-thaw cycles.

Formulation

In 1X PBS, pH 7.4, with 0.09% sodium azide

Applications Notes

The stated application concentrations are suggested starting points. Titration of the GAA antibody may be required due to differences in protocols and secondary/substrate sensitivity.