WH0002548M1

Monoclonal Anti-GAA antibody produced in mouse

clone 3C6, purified immunoglobulin, buffered aqueous solution

• Synonym(s): Anti-LYAG, Anti-glucosidase, α acid (Pompe disease, glycogen storage disease type II)
• UNSPSC Code: 12352203
• NACRES: NA.41

Properties

• biological source: mouse
• conjugate: unconjugated
• antibody form: purified immunoglobulin
• antibody product type: primary antibodies
• clone: 3C6, monoclonal
• form: buffered aqueous solution
• species reactivity: human
• technique(s): indirect ELISA: suitable; western blot: 1-5 μg/mL
• isotype: IgG1κ
• GenBank accession no.: BC040431
• UniProt accession no.: P10253
• shipped in: dry ice
• storage temp.: −20°C
• target post-translational modification: unmodified
• Gene Information: human ... GAA(2548)

Description

General description

Lysosomal α-glucosidase (GAA), a glycoprotein and member of glycoside hydrolase family GH31, comprises a trefoil type-P domain, catalytic GH31 domain, distal, proximal, and an N-terminal β-sheet domain. The GAA gene is mapped to human chromosome 17q25.3. It undergoes various proteolytical and N-glycan processing in the late endosomal/lysosomal compartment to become an active form.

Immunogen

GAA (AAH40431, 851 a.a. ~ 952 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Sequence
GEARGELFWDDGESLEVLERGAYTQVIFLARNNTIVNELVRVTSEGAGLQLQKVTVLGVATAPQQVLSNGVPVSNFTYSPDTKVLDICVSLLMGEQFLVSWC

Biochem/physiol Actions

Lysosomal α-glucosidase (GAA) uses mannose-6-phosphate receptors for its localization on the lysosomes. It mediates the hydrolysis of glycogen to glucose. Mutations in the GAA gene impair acid alpha-glucosidase enzyme activity. Deficiency of GAA leads to a rare lysosomal storage disease namely Pompe disease.

Physical form

Solution in phosphate buffered saline, pH 7.4

Legal Information

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Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Safety Information

• Storage Class Code: 10 - Combustible liquids
• Flash Point(F): Not applicable
• Flash Point(C): Not applicable
• Personal Protective Equipment: dust mask type N95 (US), Eyeshields, Gloves