GAA Polyclonal Antibody, APC-Cy5.5 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-Cy5.5
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/694nm
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
QP7877-ec-50ug 50ug
Recombinant Human BTB/ POZ domain-containing protein KCTD15 Protein, GST, E.coli-50ug
Ask
View Details 
QP7879-ec-50ug 50ug
Recombinant Human Mitochondrial cardiolipin hydrolase Protein, His-SUMO, E.coli-50ug
Ask
View Details MBS6259419-02 5x 0.1 mL
Chondroitin Sulfate Proteoglycan (NG2, CSPG) (MaxLight 490)
Ask
View Details 