MULTI SCIENCES
Human Alpha-Galactosidase A/GLA Antibody ELISA Kit
Human Alpha-Galactosidase A/GLA Antibody ELISA Kit
SKU:EK1260
Product Details
| Brand | MultiSciences |
|---|---|
| CatNum | 70-EK1260 |
| Product Name | Human alpha-Galactosidase A/GLA ELISA Kit |
| Customs Name | Human alpha-Galactosidase A/GLA ELISA Kit |
| Application | ELISA |
| Reactivity | Human |
| Assay Type | Sandwich ELISA |
| Suitable Sample Type | serum, plasma, cell culture supernates |
| Format | 96-well strip plate |
| Storage | 4℃ (unopened) standard stored at -20℃, others stored at 4℃ (opened) |
| Shipping Condition | 4℃ |
| Sample Volume | 20 μl |
| Sensitivity | 53.55 pg/ml |
| Standard Curve Range | 500.00 - 32000 pg/ml |
| Spike Recovery Range | 73 % - 127 % |
| Mean Spike Recovery | 0.93 |
| CV of Intra plate | 4.9% - 8.8 % |
| CV of Inter plate | 3.6% - 8.0% |
| Components | 96-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 |
| Describtion | This 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. |
