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E-AB-65554

GAA Polyclonal Antibody

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£195.30

War £217.00

SKU:
E-AB-65554
Zusätzliche Namen:
GAA,LYAG
Anwendung:
WB
Konzentration:
1 mg/ml
Aufreinigung:
Affinity Purified
Lagerbedingungen:
-20[o]C Avoid freeze/thaw cycles.
Hersteller:
Elabscience
Host:
Rabbit
Reaktivitäten:
Human, Mouse, Rat
Buffer:
Phosphate buffered solution, pH 7.4, containing 0.05% stabilizer and 50% glycerol.
Immunogen:
A synthetic peptide of human GAA (NP_000143.2).
Uniprot:
P10253
Synonyme:
acid maltase;aglucosidase alfa;glucosidase alpha, acid;LYAG;lysosomal alpha-glucosidase
Weitere 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.
Versandbedingungen:
Blue Ice