Anti-GAA Antibody

Rs. 45,619.00
SKU stj29987

General Information

Product name Anti-GAA Antibody
Short Description Rabbit polyclonal to GAA
Description This 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 Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Alternative splicing results in multiple transcript variants.
Applications WB
Dilution range WB 1:500 - 1:2000
Protein Name Anti-GAA Antibody
Immunogen A synthetic peptide corresponding to a sequence within amino acids 350-450 of human GAA (NP_000143.2).
Storage Instruction Store at -20℃. Avoid freeze / thaw cycles.
Type of Usage For Research Use Only (RUO).

Product Properties

Host Rabbit
Clonality Polyclonal
Reactivity
Conjugation Unconjugated
Purification Affinity purification
Isotype IgG
Formulation Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Target

Gene ID
Gene Symbol
Molecular Weight 105.3 kDa
Database Links
Alternative Names GAALysosomal alpha-glucosidaseGAA
Function Essential for the degradation of glycogen in lysosomes.
Cellular Localization Lysosome
Tissue Specificity
Swiss-Prot Key

 

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