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  2. Monoclonal

A19234

GAA Rabbit monoclonal antibody

Size

POA

SKU:
A19234
Additional Names:
GAA|LYAG
Application:
ELISA, WB
Molecular Weight:
76kDa/105kDa
Species Reactivity:
Human
Purification:
Affinity Purified
Storage Conditions:
-20[o]C Avoid freeze/thaw cycles.
Supplier:
Abclonal
Host:
Rabbit
Reactivities:
Human
Immunogen:
Synthetic peptide. This information is considered to be commercially sensitive.
Formulation:
Unmodified
Sequence:
QEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPHV
Uniprot:
P10253
Synonyms:
acid maltase;aglucosidase alfa;glucosidase alpha, acid;LYAG;lysosomal alpha-glucosidase
Extra Details:
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.
Shipping Conditions:
Blue Ice