Principle of the Assay: This ELISA kit uses Sandwich-ELISA as the method. The micro ELISA plate provided in this kit has been pre-coated with an antigen specific to AGA. Standards or samples are added to the appropriate micro ELISA plate wells and combined with the specific antigen. Then a biotinylated detection antigen specific for AGA and Avidin-Horseradish Peroxidase (HRP) conjugate is added to each micro plate well successively and incubated. Free components are washed away. The substrate solution is added to each well. Only those wells that contain AGA, biotinylated detection antigen and Avidin-HRP conjugate will appear blue in color. The enzyme-substrate reaction is terminated by the addition of a sulphuric acid solution and the color turns yellow. The optical density (OD) is measured spectrophotometrically at a wavelength of 450 nm +/- 2 nm. The OD value is proportional to the concentration of AGA.You can calculate the concentration of AGA in the samples by comparing the OD of the samples to the standard curve.
NCBI and Uniprot Product Information
NCBI Description
Aspartylglucosaminidase is involved in the catabolism of N-linked oligosaccharides of glycoproteins. It cleaves asparagine from N-acetylglucosamines as one of the final steps in the lysosomal breakdown of glycoproteins. The lysosomal storage disease aspartylglycosaminuria is caused by a deficiency in the AGA enzyme. Alternatively spliced transcript variants have been identified. [provided by RefSeq, Jan 2010]
Uniprot Description
AGA: a catabolic enzyme, aspartylglucosaminidase is involved in the catabolism of N-linked oligosaccharides of glycoproteins. It cleaves asparagine from N-acetylglucosamines as one of the final steps in the lysosomal breakdown of glycoproteins. The lysosomal storage disease aspartylglycosaminuria is caused by a deficiency in the AGA enzyme.
Protein type: Glycan Metabolism - other glycan degradation; EC 3.5.1.26; Hydrolase
Chromosomal Location of Human Ortholog: 4q34.3
Cellular Component: lysosome; endoplasmic reticulum
Molecular Function: peptidase activity; protein self-association; N4-(beta-N-acetylglucosaminyl)-L-asparaginase activity
Biological Process: protein maturation; protein deglycosylation; proteolysis
Disease: Aspartylglucosaminuria