Typical Testing Data/Standard Curve (for reference only)
Mouse Alpha-galactosidase A ELISA Kit | Gla elisa kit
Mouse Alpha-galactosidase A ELISA Kit
Typical Testing Data/Standard Curve (for reference only)
Typical Testing Data/Standard Curve (for reference only)
Principle of the Assay: The microtiter plate provided in this kit has been pre-coated with an antibody specific to Alpha-galactosidase A. Standards or samples are then added to the appropriate microtiter plate wells with a biotin-conjugated polyclonal antibody preparation specific for Alpha-galactosidase A and Avidin conjugated to Horseradish Peroxidase (HRP) is added to each microplate well and incubated. Then a TMB substrate solution is added to each well. Only those wells that contain Alpha-galactosidase A, biotin-conjugated antibody and enzyme-conjugated Avidin will exhibit a change in color. The enzyme-substrate reaction is terminated by the addition of a sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450 nm ± 2 nm. The concentration of Alpha-galactosidase A in the samples is then determined by comparing the O.D. of the samples to the standard curve.
NCBI and Uniprot Product Information
Uniprot Description
GLA: Defects in GLA are the cause of Fabry disease (FD). FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities. Belongs to the glycosyl hydrolase 27 family.
Protein type: Lipid Metabolism - glycerolipid; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Carbohydrate Metabolism - galactose; Lipid Metabolism - sphingolipid; EC 3.2.1.22; Hydrolase
Cellular Component: cytoplasm; extracellular region; Golgi apparatus; lysosome
Molecular Function: alpha-galactosidase activity; catalytic activity; galactoside binding; hydrolase activity; hydrolase activity, acting on glycosyl bonds; hydrolase activity, hydrolyzing O-glycosyl compounds; protein homodimerization activity; receptor binding
Biological Process: bone mineralization; carbohydrate metabolic process; glycosylceramide catabolic process; metabolic process; negative regulation of nitric oxide biosynthetic process; negative regulation of nitric-oxide synthase activity; oligosaccharide metabolic process
Research Articles on Gla
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Product Notes
The Mouse Gla gla (Catalog #AAA2885956) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA2885956 ELISA Kit recognizes Mouse Gla. It is sometimes possible for the material contained within the vial of "Alpha-galactosidase A, 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
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
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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