| Host: | Rabbit |
| Applications: | WB/ELISA |
| Reactivity: | Human |
| Note: | STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
| Clonality : | Monoclonal |
| Clone ID : | S6MR |
| Conjugation: | Unconjugated |
| Isotype: | IgG |
| Formulation: | PBS with 0.02% Sodium Azide, 0.05% BSA, 50% Glycerol, pH 7.3. |
| Purification: | Affinity purification |
| Concentration: | Lot specific |
| Dilution Range: | WB:1:1000-1:6000ELISA:Recommended starting concentration is 1 Mu g/mL. Please optimize the concentration based on your specific assay requirements. |
| Storage Instruction: | Store at-20°C for up to 1 year from the date of receipt, and avoid repeat freeze-thaw cycles. |
| Gene Symbol: | GAA |
| Gene ID: | 2548 |
| Uniprot ID: | LYAG_HUMAN |
| Immunogen Region: | 100-200 |
| Specificity: | A synthetic peptide corresponding to a sequence within amino acids 100-200 of human GAA (P10253). |
| Immunogen Sequence: | QEQCEARGCCYIPAKQGLQG AQMGQPWCFFPPSYPSYKLE NLSSSEMGYTATLTRTTPTF FPKDILTLRLDVMMETENRL HFTIKDPANRRYEVPLETPH V |
| Post Translational Modifications | The different forms of acid glucosidase are obtained by proteolytic processing. Phosphorylation of mannose residues ensures efficient transport of the enzyme to the lysosomes via the mannose 6-phosphate receptor. |
| Function | Essential for the degradation of glycogen in lysosomes. Has highest activity on alpha-1,4-linked glycosidic linkages, but can also hydrolyze alpha-1,6-linked glucans. |
| Protein Name | Lysosomal Alpha-GlucosidaseAcid MaltaseAglucosidase Alfa Cleaved Into - 76 Kda Lysosomal Alpha-Glucosidase - 70 Kda Lysosomal Alpha-Glucosidase |
| Database Links | Reactome: R-HSA-5357609Reactome: R-HSA-6798695Reactome: R-HSA-70221 |
| Cellular Localisation | LysosomeLysosome Membrane |
| Alternative Antibody Names | Anti-Lysosomal Alpha-Glucosidase antibodyAnti-Acid Maltase antibodyAnti-Aglucosidase Alfa Cleaved Into - 76 Kda Lysosomal Alpha-Glucosidase - 70 Kda Lysosomal Alpha-Glucosidase antibodyAnti-GAA antibody |
Information sourced from Uniprot.org