Anti-GAA Antibody Picoband® (monoclonal, 2G7)

SKU: M01548

Size: 100 μg

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

Form: Lyophilized

Reactivity: Human

Applications: IF,IHC,ICC,WB

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

Gene Name: GAA

Specificity: No cross reactivity with other proteins.

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.

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.

Clonality: Monoclonal

Contents: Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4, 0.01mg NaN3.

Purification: Immunogen affinity purified.

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

Reference: 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.

Uniprot ID: P10253

Host: Mouse

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate: This is the unconjugated antibody. It is also available with 10 conjugation options, including Biotin, Cy3, Fluoro488, Fluoro550, Fluoro594, FITC, HRP, APC, PE, and Fluoro647. A carrier-free (BSA-free) version is also available. Additional charges may apply for some options. Please contact us for a quote

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Mouse IgG2b

Phospho_site:

Clone Number: Clone: 2G7

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

Calculated Molecular Weight:

Gene ID: 2548

Protein Name: Lysosomal alpha-glucosidase

Gene Full Name: alpha glucosidase

Synonyms: Acid maltase; Aglucosidase alfa; GAA; glucosidase; alpha; acid; LYAG; Lysosomal alpha glucosidase