GAA antibody (Middle Region)

Catalog No. ABIN4886596

Product Details anti-GAA Antibody

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

Binding Specificity: AA 494-527, Middle Region

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GAA antibody is un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (IHC)

Purpose: Anti-GAA Antibody Picoband®

Sequence: TALAWWEDMV AEFHDQVPFD GMWIDMNEPS NFIR

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

Characteristics: Anti-GAA Antibody Picoband® (ABIN4886596). Tested in IHC, WB applications. This antibody reacts with Human. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

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: Immunohistochemistry (Paraffin-embedded Section), 0.5-1 μg/mL, Human
Western blot, 0.1-0.5 μg/mL, Human
1. "Entrez Gene: GAA glucosidase, alpha, acid (Pompe disease, glycogen storage disease type II)". 2. Donald J. Voet, Judith G. Voet, Charlotte W. Pratt (2008). "Additional Pathways in Carbohydrate Metabolism". Principles of Biochemistry, Third edition. Wiley. p. 538. 3. Reuser AJ, Kroos MA, Hermans MM, et al. (1995). "Glycogenosis type II (acid maltase deficiency).". Muscle Nerve. 3: S61-9.

Comment:

Antibody can be supported by chemiluminescence kit ABIN921124 in WB, supported by ABIN921231 in IHC(P).

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, 0.05 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.

Handling Advice: Avoid repeated freezing and thawing.

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.

Target Details for GAA

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

Alternative Name: GAA (GAA Products)

Background:

Synonyms: Lysosomal alpha-glucosidase,3.2.1.20,Acid maltase,Aglucosidase alfa,76 kDa lysosomal alpha-glucosidase,70 kDa lysosomal alpha-glucosidase,GAA,

Tissue Specificity: Most abundant in heart, brain, liver, skeletal muscle and testis but absent in thymus and peripheral blood leukocytes.

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.

Molecular Weight: 110 kDa, 95kDa, 76kDa, 70 kDa

Gene ID: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process