WH0002548M1

Monoclonal Anti-GAA antibody produced in mouse

clone 3C6, purified immunoglobulin, buffered aqueous solution

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

Eigenschaften

• Biologische Quelle: mouse
• Konjugat: unconjugated
• Antikörperform: purified immunoglobulin
• Antikörper-Produkttyp: primary antibodies
• Klon: 3C6, monoclonal
• Form: buffered aqueous solution
• Speziesreaktivität: human
• Methode(n): indirect ELISA: suitable; western blot: 1-5 μg/mL
• Isotyp: IgG1κ
• GenBank-Hinterlegungsnummer: BC040431
• UniProt-Hinterlegungsnummer: P10253
• Versandbedingung: dry ice
• Lagertemp.: −20°C
• Posttranslationale Modifikation Target: unmodified
• Angaben zum Gen: human ... GAA(2548)

Beschreibung

Allgemeine Beschreibung

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. Wirkung

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.

Physikalische Form

Solution in phosphate buffered saline, pH 7.4

Rechtliche Hinweise

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Haftungsausschluss

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.

Sicherheitshinweise

• Lagerklassenschlüssel: 10 - Combustible liquids
• Flammpunkt (°F): Not applicable
• Flammpunkt (°C): Not applicable
• Persönliche Schutzausrüstung: Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)