Product Detail
Product Name Alpha Glucosidase Antibody FITC Conjugated
Host SpeciesRabbit
ClonalityPolyclonal
IsotypeIgG
PurificationPurified by Protein A.
ApplicationsICC IF
Species ReactivityHu Ms Rt
Immunogen DescKLH conjugated synthetic peptide derived from human GAA Glucosidase alpha
Target NameGAA
ConjugateFITC
Excitation Emission494nm 518nm
Other Names70 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.
Accession No
NCBI Gene ID2548
Uniprot
P10253
Gene ID
2548;
Concentration1mg ml
Formulation0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
StorageShipped at 4˚C. Store at -20˚C for one year. Avoid repeated freeze/thaw cycles.
Application Details
ICC=1:50-200 IF=1:50-200
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].
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