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Cat No | BMA-2169 |
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 | AGL;GDE;glycogen debranching enzyme |
Purification | Affinity purification |
MolecularWeight | 175KDa |
Description | |
Background | This gene encodes the glycogen debrancher enzyme which is involved in glycogen degradation. This enzyme has two independent catalytic activities which occur at different sites on the protein: a 4-alpha-glucotransferase activity and a amylo-1,6-glucosidase activity. Mutations in this gene are associated with glycogen storage disease although a wide range of enzymatic and clinical variability occurs which may be due to tissue-specific alternative splicing. Alternatively spliced transcripts encoding different isoforms have been described. |