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You are here:Home » Antibodies » Abs to Enzymes, Glycosidase » Anti -GAA, NT (GAA, Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76kD lysosomal alpha-glucosidase, 70kD lysosomal alpha-glucosidase)

Anti -GAA, NT (GAA, Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76kD
lysosomal alpha-glucosidase, 70kD lysosomal alpha-glucosidase)

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Specifications

Clone Host Grade Applications
Polyclonal Rabbit Affinity Purified E B IH
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.
Catalog #035832
ApplicationsSuitable for use in Western Blot, Immunohistochemistry, ELISA
Recommended DilutionELISA: 1:1,000
Western Blot: 1:100-500
Immunohistochemistry: 1:10-50
Storage and StabilityMay be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Clone TypePolyclonal
IsotypeIgG
HostRabbit
SourceHuman
ConcentrationAs reported
FormSupplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.
PurityPurified by Protein A affinity chromatography.
ImmunogenGAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 173-203 amino acids from the N-terminal region of human GAA.
SpecificityHuman
Important NoteThis product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.
Alternate namesGAA, Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76kD lysosomal alpha-glucosidase, 70kD lysosomal alpha-glucosidase


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