ELISA/assay

Human Alpha Glucosidase ELISA Kit

MBS731326

48-Strip-Wells

470 USD

ELISA Kit

Human Alpha Glucosidase ELISA Kit

Alpha Glucosidase

alpha-glucosidase; Lysosomal alpha-glucosidase; lysosomal alpha-glucosidase; acid maltase; aglucosidase alfa; glucosidase, alpha; acid; Acid maltase; Aglucosidase alfaCleaved into the following 2 chains:76 kDa lysosomal alpha-glucosidase; 70 kDa lysosomal alpha-glucosidase

A Glu

GAA; GAA; LYAG

LYAG_HUMAN

Human

Store all reagents at 2-8 degree C.

Samples: Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate. Assay Type: Competitive. Sensitivity: 1.0 ng/mL.

For samples: Serum, plasma, cell culture supernatants, body fluid and tissue homogenate . 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, not for therapeutic or diagnostic applications! . 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.

114159823

ABI53718.1

105,324 Da

Galactose Metabolism Pathway (82931); Galactose Metabolism Pathway (292); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolic Pathways (132956); Notch-mediated HES/HEY Network Pathway (169347); Starch And Sucrose Metabolism Pathway (82974); Starch And Sucrose Metabolism Pathway (344)

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]

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

48-Strip-Wells

470 USD

96-Strip-Wells

675