catalog number :
MBS720066
products type :
ELISA Kit
products full name :
Rabbit alpha-Glucosidase ELISA Kit
products short name :
alpha-Glucosidase
products name syn :
Rabbit a-Glucosidase ELISA Kit
other names :
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
products gene name :
A-Glu
products gene name syn :
A-Glu
other gene names :
GAA; GAA; LYAG
uniprot entry name :
LYAG_HUMAN
storage stability :
Store all reagents at 2-8 degree C.
products description :
For Samples: Cell culture fluid & body fluid & tissue homogenate Serum or blood plasma. Intended Uses: This A-GLU ELISA kit is intended for laboratory research use only and not for use in diagnostic or therapeutic procedures. The stop solution changes the color from blue to yellow and the intensity of the color is measured at 450 nm using a spectrophotometer. In order to measure the concentration of A-GLU in the sample, this A-GLU ELISA Kit includes a set of calibration standards. The calibration standards are assayed at the same time as the samples and allow the operator to produce a standard curve of Optical Density versus A-GLU concentration. The concentration of in the samples is then determined by comparing the O.D. of the samples to the standard curve. Principle of the Assay: The coated well immunoenzymatic assay for the quantitative measurement of A-GLU utilizes a multiclonal anti-A-GLU antibody and an A-GLU-HRP conjugate. The assay sample and buffer are incubated together with A-GLU-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 A-GLU concentration since A-GLU from samples and A-GLU-HRP conjugate compete for the anti-A-GLU antibody binding site. Since the number of sites is limited, as more sites are occupied by A-GLU from the sample, fewer sites are left to bind A-GLU-HRP conjugate. Standards of known A-GLU concentrations are run concurrently with the samples being assayed and a standard curve is plotted relating the intensity of the color (Optical Density) to the concentration of A-GLU. The A-GLU concentration in each sample is interpolated from this standard curve.
ncbi acc num :
ABI53718.1
ncbi mol weight :
105,324 Da
ncbi pathways :
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
ncbi summary :
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]