GAA Polyclonal Antibody, AbBy Fluor™ 647 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
AbBy Fluor™ 647
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/665nm
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
Available Sizes
Curated Selection
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