WH0002548M1

Monoclonal Anti-GAA antibody produced in mouse

clone 3C6, purified immunoglobulin, buffered aqueous solution

• Synonim(y): Anti-LYAG, Anti-glucosidase, α acid (Pompe disease, glycogen storage disease type II)
• Kod UNSPSC: 12352203
• NACRES: NA.41

Właściwości

• pochodzenie biologiczne: mouse
• Poziom jakości: 100
• białko sprzężone: unconjugated
• forma przeciwciała: purified immunoglobulin
• rodzaj przeciwciała: primary antibodies
• klon: 3C6, monoclonal
• Postać: buffered aqueous solution
• reaktywność gatunkowa: human
• metody: indirect ELISA: suitable; western blot: 1-5 μg/mL
• izotyp: IgG1κ
• numer dostępu GenBank: BC040431
• numer dostępu UniProt: P10253
• Warunki transportu: dry ice
• temp. przechowywania: −20°C
• docelowa modyfikacja potranslacyjna: unmodified
• informacje o genach: human ... GAA(2548)

Opis

Opis ogólny

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

Działania biochem./fizjol.

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.

Postać fizyczna

Solution in phosphate buffered saline, pH 7.4

Informacje prawne

GenBank is a registered trademark of United States Department of Health and Human Services

Oświadczenie o zrzeczeniu się odpowiedzialności

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.

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Informacja o środkach bezpieczeństwa

• Kod klasy składowania: 10 - Combustible liquids
• Temperatura zapłonu (°F): Not applicable
• Temperatura zapłonu (°C): Not applicable
• Środki ochrony indywidualnej: Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)