GAA antibody (Middle Region)

Catalog No. ABIN7993520

The Mouse Monoclonal anti-GAA antibody (Clone 2G7) (ABIN7993520) specifically detects GAA in WB, IHC, ICC and IF. The antibody is reactive with Human samples.

Quick Overview for GAA antibody (Middle Region) (ABIN7993520)

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

Reactivity: Human

Host: Mouse

Clonality: Monoclonal

Conjugate: This GAA antibody is un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (IHC), Immunocytochemistry (ICC), Immunofluorescence (IF)

Grade: Carrier-free

Clone: 2G7

Product Details anti-GAA Antibody

Binding Specificity: Middle Region

Purpose: Anti-GAA Antibody (monoclonal, 2G7)

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.

Characteristics: Anti-GAA Antibody.(monoclonal, 2G7). Tested in IF, IHC, ICC, WB applications. This antibody reacts with Human.

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: IgG2b

Application Details

Application Notes: Western blot, 0.1-0.5 μg/mL, Human Immunohistochemistry (Paraffin-embedded Section), 0.5-1 μg/mL, Human Immunocytochemistry/Immunofluorescence, 2 μg/mL, Human

Restrictions: For Research Use only

Handling

Format: Lyophilized

Reconstitution: Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.

Concentration: 500 μg/mL

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.01 mg 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: 4 °C,-20 °C

Storage Comment: Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.

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

Molecular Weight: 110 kDa, 95 kDa, 76 kDa

Gene ID: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process