GAA Rabbit Polyclonal Antibody

CAT#: TA376424

GAA Rabbit polyclonal Antibody



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热销推荐

CNY 1,999.00

CNY 3,280.00


货期*
2周

规格
    • 100 ul

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Specifications

Product Data
Applications ICC/IF, IP, WB
Recommend Dilution WB,1:500 - 1:2000
IF,1:50 - 1:200
IP,1:50 - 1:200
Reactivity Human, Mouse, Rat
Modifications Unmodified
Host Rabbit
Clonality Polyclonal
Immunogen A synthetic peptide corresponding to a sequence within amino acids 350-450 of human GAA (NP_000143.2).
Formulation PBS with 0.05% proclin300,50% glycerol,pH7.3.
Concentration lot specific
Purification Affinity purification
Conjugation Unconjugated
Storage Condition Store at -20℃. Avoid freeze / thaw cycles.
Predicted Protein Size 105kDa
Gene Name glucosidase alpha, acid
Background 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.
Synonyms LYAG
Reference Data
*Delivery time may vary from web posted schedule. Occasional delays may occur due to unforeseen complexities in the preparation of your product. International customers may expect an additional 1-2 weeks in shipping.
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