GAA antibody (AA 174-203)

Catalog No. ABIN7871285

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

Quick Overview for GAA antibody (AA 174-203) (ABIN7871285)

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

Reactivity: Human

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 174-203

Purpose: GAA Antibody / Glucosidase alpha acid

Purification: Antigen affinity purified

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

Isotype: Ig Fraction

Application Details

Application 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.

Restrictions: For Research Use only

Handling

Format: Liquid

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

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: -20 °C

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

Target Details for GAA

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

Alternative Name: GAA

Background: 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.

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process