GAA Recombinant Antibody, FITC 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
FITC
Source
Synthetic peptide within Human GAA.
Applications
WB, FCM
Purification
Purified by Protein A.
Excitation Emission
494nm/518nm
Concentration
Lot dependent
Dilution
WB (1:300-5000), FCM (1:20-100)
Buffer
Aqueous buffered solution containing 0.01M TBS (pH 7.4) 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
Curated Selection
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