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Human Alpha-Galactosidase A/GLA Antibody ELISA Kit

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Product Details

Standard - EK1260.jpg

BrandMultiSciences
CatNum70-EK1260
ProductNameHuman alpha-Galactosidase A/GLA ELISA Kit
CustomsNameHuman alpha-Galactosidase A/GLA ELISA Kit
ApplicationELISA
ReactivityHuman
Assay TypeSandwich ELISA
Suitable Sample Typeserum, plasma, cell culture supernates
Format96-well strip plate
Storage4℃ (unopened) standard stored at -20℃, others stored at 4℃ (opened)
Shipping Condition4℃
Sample Volume20 μl
Sensitivity53.55 pg/ml
Standard Curve Range500.00 - 32000 pg/ml
Spike Recovery Range73 % - 127 %
Mean Spike Recovery0.93
CV of Intra plate4.9% - 8.8 %
CV of Inter plate3.6% - 8.0%
Components96-well polystyrene microplate coated with a monoclonal antibody against GLA
Human GLA Standard, lyophilized
GLA Detect Antibody
Standard Diluent
Assay Buffer (10×)
Substrate (TMB)
Stop Solution
Washing Buffer (20×)
Plate Covers
DescribtionThis assay employs the quantitative sandwich enzyme immunoassay technique for the quantitative detection of human GLA. The Human alpha-Galactosidase A/GLA ELISA is for research use only. Not for diagnostic or therapeutic procedures.
Alpha-galactosidase A, also known as 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 (FD). Defects in GLA are the cause of 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 globotriaosylceramide (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. In addition, 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.
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