GAA Polyclonal Antibody, APC 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; Acid alpha glucosidase; Acid maltase; Aglucosidase alfa; Alpha glucosidase; GAA; Glucosidase alpha acid Pompe disease glycogen storage disease type II; Glucosidase alpha acid; Glucosidase alpha; LYAG; LYAG_HUMAN; Lysosomal alpha glucosidase.

Gene ID

2548

Swiss Prot

P10253

Cellular Locus

Cytoplasm, Cell membrane

Host

Rabbit

Cross Reactivity

Human, Mouse

Immunogen

541-640/952

Target

GAA

Clonality

Polyclonal

Isotype

IgG

Conjugation

APC

Source

KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha

Applications

WB, IF (IHC-P), IF (IHC-F), IF (ICC)

Purification

Purified by Protein A.

Excitation Emission

650nm/660nm

Concentration

1µg/µl

Dilution

WB (1:300-5000), IF (IHC-P) (1:50-200), IF (IHC-F) (1:50-200), IF (ICC) (1:50-200)

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

Predicted Cross Reactivity

Rat, Dog, Sheep, Pig, Horse

Curated Selection

Explore Other Products

Discover premium biology products from our extensive collection of 20M+ items

NBP2-99087H 0.1 mL

Rabbit Polyclonal E2F6 Antibody [HRP]

Ask

View Details

STJ117191 100 µl

Anti-ARCN1 antibody

Ask

View Details

ELI-21217h 96 Tests

Human OSBPL10 ELISA KIT

Ask

View Details