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Monoclonal Antibody

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Rabbit anti-Human GAA Monoclonal Antibody

Cat NoBMA-1846
Conjugate
Type重组兔多抗
SourceRabbit
Size50 uL
ApplicationImmunogen
FormatLiquid
ConcentrationPlease refer to the vial lable for the specific concentration.
BufferSupplied in PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.
SpeciesHuman
StorageStore at -20℃. Avoid freeze / thaw cycles.
SynonymsGAA;LYAG;glucosidase alpha;acid
PurificationAffinity purification
MolecularWeight76KDa/105KDa
Description
BackgroundThis gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. Defects in this gene are the cause of glycogen storage disease II, also known as Pompes disease, which is an autosomal recessive disorder with a broad clinical spectrum. Alternative splicing results in multiple transcript variants.
We can offer labeled proteins/antibodies using a broad range of intensely fluorescent dyes and labels including FITC, Biotin, Alexa Fluor, Rhodamine B, Cy, Co-Au.