GLA Matched ELISA Antibody Pair Set,Human
Solid Phase Sandwich ELISA
Quantitative determination of Human GLA
1. Capture Antibody 0.5 mg/mL of mouse anti-Human alpha-Galactosidase A / GLA monoclonal antibody (in PBS, pH 7.4). Dilute to a working concentration of 2 μg/mL in PBS before coating. (Catalog: # 12078-MM08)
2. Detection Antibody 0.2 mg/mL of mouse anti-Human alpha-Galactosidase A / GLA monoclonal antibody conjugated to horseradish-peroxidase (HRP) (in PBS, 50 % HRP-Protector, pH 7.4, store at 4℃). Dilute to working concentration of 0.5 μg/mL in detection antibody dilution buffer before use. (Catalog: # 12078-MM05)
3. Standard Each vial contains 300 ng of recombinant Human alpha-Galactosidase A / 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 10000 pg/mL is recommended.
This GLA Matched ELISA Antibody Pair Set,Human is a solid phase sandwich ELISA for quantitative determination of Human GLA . It contains Human GLA capture antibody, Human GLA detector antibody
and a highly purified
recombinant Human GLA protein. This Pair Set is at affordable price for researchers.
This Matched ELISA Antibody Pair Set is shipped at ambient temperature.
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: Store at 4℃ and protect it from prolonged exposure to light for up to 6 months from date of receipt. DO NOT FREEZE!
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.
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.
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.