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Cat No | BMA-1846 |
Conjugate | |
Type | 重组兔多抗 |
Source | Rabbit |
Size | 50 uL |
Application | Immunogen |
Format | Liquid |
Concentration | Please refer to the vial lable for the specific concentration. |
Buffer | Supplied in PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3. |
Species | Human |
Storage | Store at -20℃. Avoid freeze / thaw cycles. |
Synonyms | GAA;LYAG;glucosidase alpha;acid |
Purification | Affinity purification |
MolecularWeight | 76KDa/105KDa |
Description | |
Background | 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 Pompes disease, which is an autosomal recessive disorder with a broad clinical spectrum. Alternative splicing results in multiple transcript variants. |