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Human Alpha-Galactosidase A (GLA) ELISA Kit
Human Alpha-Galactosidase A (GLA) ELISA Kit
This ELISA kit is designed to detect Human Alpha-Galactosidase A (Human GLA). The assay plate has been pre-coated with mouse anti-Human GLA monoclonal antibody. When the sample containing GLA is added to the plate, it binds to the antibodies coated on the wells. Then, a horseradish peroxidase conjugated mouse anti-Human GLA Antibody is added to the wells and binds to GLA in the sample. After washing the wells, substrate solutions are added, and the color intensity is directly proportional to the amount of Human GLA present. The reaction is stopped by adding an acidic stop solution, and the absorbance is measured at 450 nm.
Catalog No:
BPE242
Regular price
$754.00 USD
Regular price
$580.00 USD
Sale price
$754.00 USD
Unit price
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per
2 weeks
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Product Details
Species Reactivity
Human
Sensitivity
45.96 pg/mL
Detection Range
203.13-13000 pg/mL
Sample Type
Serum, plasma, cell culture supernates
Incubation(s)
3.5 hour(s)
Research Areas
Cardiovascular, Neuroscience
Background
Alpha-galactosidase A, also known as Alpha-D-galactoside galactohydrolase, Alpha-D-galactosidase A, Melibiase and GLA, is a member of the glycosyl hydrolase 27 family. GLA is used as a long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease. Defects in GLA are the cause of Fabry disease (FD) which 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. Deficiency of GLA leads to the accumulation of glycosphingolipids in the vasculature leading to multiorgan pathology. In addition to well-described microvascular disease, deficiency of GLA is also characterized by premature macrovascular events such as stroke and possibly myocardial infarction.
Shipping Condition
Shipped on cold gel packs.
Storage Condition and Shelf Life
This product can be stored at 2-8C.
Analyte
Alpha-galactosidase A
Regulatory Status
For Research Use Only
