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GAA Recombinant Antibody, AbBy Fluor™ 488 Conjugated

Product Specifications

Background

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. [provided by RefSeq, Jul 2008].

Synonyms

70 kDa lysosomal alpha-glucosidase antibody, Acid alpha glucosidase antibody, Acid maltase antibody, Aglucosidase alfa antibody, Alpha glucosidase antibody, GAA antibody, Glucosidase alpha acid (Pompe disease glycogen storage disease type II) antibody, Glucosidase alpha acid antibody, Glucosidase alpha antibody, LYAG antibody, LYAG_HUMAN antibody, Lysosomal alpha glucosidase antibody

Gene ID

2548

Swiss Prot

P10253

Cellular Locus

Lysosome, Lysosome membrane

Host

Rabbit

Cross Reactivity

Human

Target

GAA

Clonality

Monoclonal

Isotype

IgG

Clone

5H49

Conjugation

AbBy Fluor™ 488

Source

Synthetic peptide within Human GAA.

Applications

WB, FCM

Purification

Purified by Protein A.

Excitation Emission

499nm/519nm

Concentration

Lot dependent

Dilution

WB (1:300-5000), FCM (1:20-100)

Buffer

Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Modification

Unmodified

Storage Conditions

Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Gene ID URL

2548

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