Typical Testing Data/Standard Curve (for reference only)
Monkey alpha-Glucosidase ELISA Kit | A-Glu elisa kit
Monkey alpha-Glucosidase ELISA Kit
Typical Testing Data/Standard Curve (for reference only)
Typical Testing Data/Standard Curve (for reference only)
Principle of the Assay: AGLU ELISA kit applies the competitive enzyme immunoassay technique utilizing a monoclonal anti-AGLU antibody and an AGLU-HRP conjugate. The assay sample and buffer are incubated together with AGLU-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the AGLU concentration since AGLU from samples and AGLU-HRP conjugate compete for the anti-AGLU antibody binding site. Since the number of sites is limited, as more sites are occupied by AGLU from the sample, fewer sites are left to bind AGLU-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The AGLU concentration in each sample is interpolated from this standard curve.
NCBI and Uniprot Product Information
NCBI Description
This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. 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. Three transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2008]
Uniprot Description
GAA: Essential for the degradation of glygogen to glucose in lysosomes. Defects in GAA are the cause of glycogen storage disease type 2 (GSD2); 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 failure. Patients with the adult form can be free of clinical symptoms for most of their life but finally develop a slowly progressive myopathy. Belongs to the glycosyl hydrolase 31 family.
Protein type: Contractile; Hydrolase; Carbohydrate Metabolism - galactose; EC 3.2.1.20; Carbohydrate Metabolism - starch and sucrose
Chromosomal Location of Human Ortholog: 17q25.2-q25.3
Cellular Component: membrane; lysosome; lysosomal membrane
Molecular Function: alpha-glucosidase activity; maltase activity; carbohydrate binding
Biological Process: heart morphogenesis; maltose metabolic process; tissue development; glycogen catabolic process; vacuolar sequestering; glucose metabolic process; sucrose metabolic process; locomotory behavior; muscle maintenance; neuromuscular process controlling posture; lysosome organization and biogenesis; diaphragm contraction; neuromuscular process controlling balance; regulation of the force of heart contraction; cardiac muscle contraction
Disease: Glycogen Storage Disease Ii
Research Articles on A-Glu
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Product Notes
The Monkey A-Glu gaa (Catalog #AAA744987) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA744987 ELISA Kit recognizes Monkey A-Glu. It is sometimes possible for the material contained within the vial of "alpha-Glucosidase, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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