GAA antibody (Middle Region) (Fluoro488)

Catalog No. ABIN7993501

The Rabbit Polyclonal anti-GAA antibody is suitable to detect GAA in samples from Human. It has been validated for FACS.

Quick Overview for GAA antibody (Middle Region) (Fluoro488) (ABIN7993501)

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

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GAA antibody is conjugated to Fluoro488

Application: Flow Cytometry (FACS)

Product Details anti-GAA Antibody (Fluoro488)

Binding Specificity: Middle Region

Purpose: Anti-GAA Antibody Fluoro488 Conjugated

Specificity: No cross reactivity with other proteins.

Cross-Reactivity (Details): No cross-reactivity with other proteins

Predicted Reactivity: different from the related mouse sequence by eight amino acids,and from the related rat sequence by six amino acids.

Purification: Immunogen affinity purified.

Immunogen: A synthetic peptide corresponding to a sequence in the middle region of human GAA, different from the related mouse sequence by eight amino acids, and from the related rat sequence by six amino acids.

Isotype: IgG

Application Details

Application Notes: Flow Cytometry, Optimal dilutions should be determined by end users.

Restrictions: For Research Use only

Handling

Format: Liquid

Buffer: Each vial contains 50 % glycerol, 0.9 % NaCl, 0.2 % Na2HPO4, 0.02 % 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: At -20°C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

Expiry Date: 12 months

Target Details for GAA

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

Alternative Name: GAA

Background:

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.

Gene Full Name: alpha glucosidase

Gene ID: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process