E-AB-65554
GAA Polyclonal Antibody
Size
£195.30
Was £217.00
- SKU:
- E-AB-65554
- Additional Names:
- GAA,LYAG
- Application:
- WB
- Concentration:
- 1 mg/ml
- Purification:
- Affinity Purified
- Storage Conditions:
- -20[o]C Avoid freeze/thaw cycles.
- Supplier:
- Elabscience
- Host:
- Rabbit
- Reactivities:
- 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
- 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