SAB2100872

Anti-GAA antibody produced in rabbit

affinity isolated antibody

• Synonym(s): Anti-Glucosidase, α; acid, Anti-LYAG
• UNSPSC Code: 12352203
• NACRES: NA.41

Properties

• biological source: rabbit
• Quality Level: 100
• conjugate: unconjugated
• antibody form: affinity isolated antibody
• antibody product type: primary antibodies
• clone: polyclonal
• form: buffered aqueous solution
• mol wt: 98 kDa
• species reactivity: dog, guinea pig, human, rat, mouse, bovine
• concentration: 0.5 mg - 1 mg/mL
• technique(s): immunohistochemistry: suitable; western blot: suitable
• UniProt accession no.: P10253
• shipped in: wet ice
• storage temp.: −20°C
• target post-translational modification: unmodified
• Gene Information: human ... GAA(2548)

Description

General description

Lysosomal alpha-glucosidase (GAA) is a glycoprotein that belongs to the glycoside hydrolase family GH31. It comprises a catalytic GH31 (β/α)8 barrel domain, trefoil type-P domain, an N-terminal β-sheet domain. The GAA gene is mapped to human chromosome location 17q25.3.

Immunogen

Synthetic peptide directed towards the N terminal region of human GAA

Application

Anti-GAA antibody produced in rabbit has been used western blotting (1:100)

Biochem/physiol Actions

GAA is acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. 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. This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. 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. Three transcript variants encoding the same protein have been found for this gene.

Sequence

Synthetic peptide located within the following region: FGVIVRRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQYITGLAEHLSPL

Physical form

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

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: 10 - Combustible liquids
• wgk_germany: WGK 3
• flash_point_f: Not applicable
• flash_point_c: Not applicable