Lysosomal α-Glucosidase
Lysosomal α-Glucosidase (EC:3.2.1.20) is a lysosomal α-Glucosidase that degrades glycogen into glucose and catalyzes the hydrolysis of α-1,4 and α-1,6 glycosidic linkages in glycogen, as well as that of natural and synthetic oligoglucosides. Deficiency of Lysosomal α-Glucosidase causes type II glycogen storage disease (Pompe disease), which is characterized by lysosomal glycogen accumulation in mammals and birds. Lysosomal α-Glucosidase can be used in research related to type II glycogen storage disease.
For research use only. We do not sell to patients.
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Storage:
Please store the product under the recommended conditions in the Certificate of Analysis.
Biological Activity
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α‑glucosidase |
| NCT Number | Sponsor | Condition | Start Date |
Phase
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|---|---|---|---|---|
| NCT01329991 | Plexxikon| | 2011-05 | PHASE1 |
Chemical Information
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SMILES
[Lysosomal a-Glucosidase]
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Synonyms
EC:3.2.1.20; GAA
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Shipping
Room temperature in continental US; may vary elsewhere.
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Storage
Please store the product under the recommended conditions in the Certificate of Analysis.
Purity & Documentation
References
[1]. Hermans MM, et al. Human lysosomal alpha-glucosidase: functional characterization of the glycosylation sites. The Biochemical journal. 1993 Feb 01;289 ( Pt 3)(Pt 3):681-6. [Content Brief]
[2]. Hoefsloot LH, et al. Primary structure and processing of lysosomal alpha-glucosidase; homology with the intestinal sucrase-isomaltase complex. The EMBO journal. 1988 Jun;7(6):1697-704. [Content Brief]
Calculators
Concentration (start) × Volume (start) = Concentration (final) × Volume (final)