A19234
GAA Rabbit monoclonal antibody
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- SKU:
- A19234
- Zusätzliche Namen:
- GAA|LYAG
- Anwendung:
- ELISA, WB
- Molekulargewicht:
- 76kDa/105kDa
- Spezies-Reaktivität:
- Human
- Aufreinigung:
- Affinity Purified
- Lagerbedingungen:
- -20[o]C Avoid freeze/thaw cycles.
- Hersteller:
- Abclonal
- Host:
- Rabbit
- Reaktivitäten:
- Human
- Immunogen:
- Synthetic peptide. This information is considered to be commercially sensitive.
- Formulierung:
- Unmodified
- Sequenz:
- QEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPHV
- 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