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Human alpha-Galactosidase A / GLA ELISA Pair Set

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Materials provided
Capture Ab:0.5 mg/mL of mouse anti-GLA monoclonal antibody. Dilute to a working concentration of 2 μg/mL in CBS before coating.
Detection Ab:0.5 mg/mL mouse anti-GLA monoclonal antibody conjugated to horseradish-peroxidase (HRP). Dilute to working concentration of 0.25 μg/mL in detection antibody dilution buffer before use.
Standard:Each vial contains 275 ng of recombinant GLA. Reconstitute with 1 mL detection antibody dilution buffer. After reconstitution, store at -20℃ to -80℃ in a manual defrost freezer. A seven-point standard curve using 2-fold serial dilutions in sample dilution buffer, and a high standard of 8 ng/mL is recommended.
Sensitivity
The minimum detectable dose of Human alpha-Galactosidase A / GLA was determined to be approximately 125 pg/ml. This is defined as at least three times standard deviations above the mean optical density of 10 replicates of the zero standard.
Principle of the product
The Human alpha-Galactosidase A / GLA ELISA Pair Set is for the quantitative determination of Human alpha-Galactosidase A / GLA.
This ELISA Pair Set contains the basic components required for the development of sandwich ELISAs.
The Sino Biological ELISA Pair Set is a solid phase sandwich ELISA (Enzyme-Linked Immunosorbent Assay). It utilizes a monoclonal antibody specific for alpha-Galactosidase A / GLA coated on a 96-well plate. Standards and samples are added to the wells, and any alpha-Galactosidase A / GLA present binds to the immobilized antibody. The wells are washed and a horseradish peroxidase conjugated mouse anti-alpha-Galactosidase A / GLA monoclonal antibody is then added, producing an antibody-antigen-antibody “sandwich”. The wells are again washed and TMB substrate solution is loaded, which produces color in proportion to the amount of alpha-Galactosidase A / GLA present in the sample. To end the enzyme reaction, the stop solution is added and absorbances of the microwell are read at 450 nm.
Storage
Capture Antibody: Aliquot and store at -20℃ to -80℃ for up to 6 months from date of receipt. Avoid repeated freeze-thaw cycles.
Detection Antibody: Protect it from prolonged exposure to light. Aliquot and store at -20℃ to -80℃ and for up to 6 months from date of receipt. Avoid repeated freeze-thaw cycles.
Standard: Store lyophilized Standard at -20℃ to -80℃ for up to 6 months from date of receipt. Aliquot and store the reconstituted Standard at -80℃ for up to 1 month. Avoid repeated freeze-thaw cycles.
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.

References
  • Koide T.et al., 1990, FEBS Lett. 259:353-356.
  • Yang C.-C. et al., 2003, Clin. Genet. 63:205-209.
  • Verovnik F. et al.,2004, Eur. J. Hum. Genet. 12:678-681.
  • Nance C.S. et al., 2006, Arch. Neurol. 63:453-457.
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