Acid alpha-glucosidase (83-99), human
Ref. 3D-CRB1001203
| 1mg | 298.00 € | ||
| 500µg | 218.00 € |
Product Information
- H-DVPPNSRFDCAPDKAIT-OHDVPPNSRFDCAPDKAIT-acidH-Asp-Val-Pro-Pro-Asn-Ser-Arg-Phe-Asp-Cys-Ala-Pro-Asp-Lys-Ala-Ile-Thr-OHAlpha-gliadin (58-73)
Acid α-glucosidase (83-99) (human) is derived from the exogenous enzyme which degrades glycogen, maltose and isomaltose through targeting alpha -1,4 and alpha -1,6 linkages. Once synthesised in its precursor form, within the Golgi it is glycosylated and acquires mannose 6-phosphate residues. This allows it to be transported to the Lysosome in a multistep process.Pompe disease, also known as glycogen storage disease type II, can be diagnosed through the absence of acid α-glucosidase activity within patients. Therefore glycogen degradation in the lysosome is inhibited by this autosomal recessive disorder. This results in the accumulation of glycogen and tissue destruction, hence contributing to the pathologies of muscle weakness and respiratory failure, associated with infantile onset and adult onset Pompe disease.
Chemical properties
Technical inquiry about: 3D-CRB1001203 Acid alpha-glucosidase (83-99), human
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