GLA / alpha-Galactosidase A Matched ELISA Antibody Pair Set,Human

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GLA / alpha-Galactosidase A Matched ELISA Antibody Pair Set,Human 製品情報

製品名
GLA / alpha-Galactosidase A Matched ELISA Antibody Pair Set,Human
検出原理
Solid Phase Sandwich ELISA
特異性
Quantitative determination of Human GLA / alpha-Galactosidase A
線形範囲
156.25-10000 pg/mL
ELISA 抗体ペアセット(すぐに使用できません)の成分
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.
製品概要
This GLA / alpha-Galactosidase A Matched ELISA Antibody Pair Set,Human is a solid phase sandwich ELISA for quantitative determination of Human GLA / alpha-Galactosidase A . It contains Human GLA / alpha-Galactosidase A capture antibody, Human GLA / alpha-Galactosidase A detector antibody (HRP) and a highly purified HEK293-expressed recombinant Human GLA / alpha-Galactosidase A protein. This Pair Set is at affordable price for researchers.
配送方法
This Matched ELISA Antibody Pair Set is shipped at ambient temperature.
保存条件
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.

GLA / alpha-Galactosidase A Matched ELISA Antibody Pair Set,Human の画像

GLA / alpha-Galactosidase A Matched ELISA Antibody Pair Set,Human: 別名

GALA Matched ELISA Antibody Pair Set, Human

GLA / alpha-Galactosidase A 背景情報

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.
完全な名称
galactosidase, alpha
参考文献
  • 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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