Human Alpha Glucosidase ELISA Kit | A Glu elisa kit
Human Alpha Glucosidase ELISA Kit
Typical Testing Data/Standard Curve (for reference only)
INTENDED USE This GAA ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Human GAA. This ELISA kit for research use only!
PRINCIPLE OF THE ASSAY GAA ELISA kit applies the competitive enzyme immunoassay technique utilizing a monoclonal anti-GAA antibody and an GAA-HRP conjugate. The assay sample and buffer are incubated together with GAA-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 GAA concentration since GAA from samples and GAA-HRP conjugate compete for the anti-GAA antibody binding site. Since the number of sites is limited, as more sites are occupied by GAA from the sample, fewer sites are left to bind GAA-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The GAA 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: Carbohydrate Metabolism - starch and sucrose; Contractile; EC 3.2.1.20; Carbohydrate Metabolism - galactose; Hydrolase
Chromosomal Location of Human Ortholog: 17q25.2-q25.3
Cellular Component: membrane; lysosomal membrane; lysosome
Molecular Function: alpha-glucosidase activity; maltase activity; carbohydrate binding
Biological Process: heart morphogenesis; tissue development; maltose metabolic process; 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
