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Antibody Product Overview
| ELK.No | ES15073 |
| Product name | LYAG rabbit pAb |
| Reactivity | Human; Mouse;Rat |
| Applications | WB |
| Other name | Lysosomal alpha-glucosidase (EC 3.2.1.20) (Acid maltase) (Aglucosidase alfa) [Cleaved into: 76 kDa lysosomal alpha-glucosidase; 70 kDa lysosomal alpha-glucosidase] |
| Size | 50μL |
| Unit price ($) | 148 |
| Human gene ID | 2548 |
| Human Swiss-Prot | P10253 |
| Source | Rabbit |
| Isotype | IgG |
| Target | LYAG |
| Fields | >>Galactose metabolism;>>Starch and sucrose metabolism;>>Metabolic pathways;>>Lysosome |
| Gene name | GAA |
| Protein name | LYAG |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 14387 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | P70699 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | 367562 |
| Rat gene link | View Rat Gene |
| Rat Swiss-Prot | Q6P7A9 |
| Rat Swiss link | View Rat Swiss-Prot |
| Immunogen | Synthesized peptide derived from human LYAG AA range: 432-482 |
| Specificity | This antibody detects endogenous levels of LYAG at Human/Mouse/Rat |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.221% sodium azide. |
| Clonality | Polyclonal |
| Dilution | WB 1:500-2000 |
| Purification | The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | |
| Observed band (KD) | 105kD |
| 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 Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2016], |
| Function | catalytic activity:Hydrolysis of terminal, non-reducing (1->4)-linked alpha-D-glucose residues with release of alpha-D-glucose.,caution:The sequence shown here is derived from an Ensembl automatic analysis pipeline and should be considered as preliminary data.,disease:Defects in GAA are the cause of glycogen storage disease type 2 (GSD2) [MIM:232300]; also called acid alpha-glucosidase (GAA) deficiency or acid maltase deficiency (AMD). GSD2 is a metabolic disorder with a broad clinical spectrum. The severe infantile form, or Pompe disease, presents at birth with massive accumulation of glycogen in muscle, heart and liver. Cardiomyopathy and muscular hypotonia are the cardinal features of this form whose life expectancy is less than two years. The juvenile and adult forms present as limb-girdle muscular dystrophy beginning in the lower limbs. Final outcome depends on respiratory muscle fa |
| Subcellular location | Lysosome . Lysosome membrane . |
| Expression | Duodenum,Liver,Placenta,Plasma,Testis,Urine, |
Additional Images
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Western blot analysis of lysates from KB cells, primary antibody was diluted at 1:1000, 4°over night
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: AO-06-ES15073-50
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