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产品描述:人溶酶体α-葡萄糖苷酶(GAA)酶联免疫试剂盒为双抗夹心法ELISA试剂盒,定量检测血清、血浆、组织匀浆、细胞裂解物样本中的GAA含量。GAA(酸性α-葡萄糖苷酶)是一种关键酶,负责将糖原分解成葡萄糖,其突变导致庞贝病,一种溶酶体储存病。研究GAA机制涉及基因编辑技术构建条件性敲除小鼠模型,以研究GAA基因功能。基因治疗为庞贝病提供了一种有希望的治疗方法。试剂盒检测范围为25 pg/mL-1600 pg/mL,该产品可广泛应用于庞贝氏症发病机制研究、基因治疗或酶替代疗法的体外效果评估、代谢相关疾病模型构建等基础科研方向,为探索糖原贮积症及溶酶体功能调控提供可靠工具。本品仅用于科研,不用于临床诊断,产品具体参数及操作步骤详见产品说明书。
别名:70 kDa lysosomal alpha-glucosidase ELISA Kit; Acid alpha glucosidase ELISA Kit; Acid maltase ELISA Kit; Aglucosidase alfa ELISA Kit; Alpha glucosidase ELISA Kit; GAA ELISA Kit; Glucosidase alpha acid (Pompe disease glycogen storage disease type II) ELISA Kit; Glucosidase alpha acid ELISA Kit; Glucosidase alpha ELISA Kit; LYAG ELISA Kit; LYAG_HUMAN ELISA Kit; Lysosomal alpha glucosidase ELISA Kit
缩写:GAA
Uniprot No.:P10253
种属:Homo sapiens (Human)
样本类型:serum, plasma, tissue homogenates, cell lysates
检测范围:25 pg/mL-1600 pg/mL
灵敏度:6.25 pg/mL
反应时间:1-5h
样本体积:50-100ul
检测波长:450 nm
研究领域:Metabolism
测定原理:quantitative
测定方法:Sandwich
精密度:
Intra-assay Precision (Precision within an assay): CV%<8% | ||||||
Three samples of known concentration were tested twenty times on one plate to assess. | ||||||
Inter-assay Precision (Precision between assays): CV%<10% | ||||||
Three samples of known concentration were tested in twenty assays to assess. | ||||||
线性度:
To assess the linearity of the assay, samples were spiked with high concentrations of human GAA in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay. | ||||||
Sample | Serum(n=4) | |||||
1:1 | Average % | 94 | ||||
Range % | 91-97 | |||||
1:2 | Average % | 84 | ||||
Range % | 81-89 | |||||
1:4 | Average % | 99 | ||||
Range % | 95-104 | |||||
1:8 | Average % | 93 | ||||
Range % | 90-96 |
回收率:
The recovery of human GAA spiked to levels throughout the range of the assay in various matrices was evaluated. Samples were diluted prior to assay as directed in the Sample Preparation section. | ||||||
Sample Type | Average % Recovery | Range | ||||
Serum (n=5) | 87 | 83-90 | ||||
EDTA plasma (n=4) | 86 | 83-89 | ||||
标准曲线:
These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed. | ||||||
pg/mlOD1OD2AverageCorrected16002.3642.5472.4562.3238001.5961.5681.5821.4494001.0011.0351.0180.8852000.6120.6340.6230.4901000.4130.4160.4150.282500.2880.2760.2820.149250.1970.1730.1850.05200.1310.1350.133 |
本试剂盒所含材料:
A micro ELISA plate ---The 96-well plate has been pre-coated with an anti-human GAA antibody.
Six vials standard (5 ml/bottle) ---Dilute a bottle of the standard at dilution series, read the OD values, and then draw a standard curve.
One vial HRP-conjugated GAA antibody (6 ml/bottle) ---Bind to the MSTN in the samples or standards and react with the substrate to make the solution chromogenic.
Substrate A (1 x 7 ml)
Substrate B (1 x 7 ml)
One vial Stop Solution (7ml/bottle) ---Stop the color reaction. The solution color immediately turns from blue to yellow.
本试剂盒不含材料:
A microplate reader capable of measuring absorbance at 450 nm, with the correction wavelength set at 600 nm or 630 nm.
An incubator can provide stable incubation conditions up to 37°C±5°C.
Centrifuge
Vortex
Squirt bottle, manifold dispenser, or automated microplate washer
Absorbent paper for blotting the microtiter plate
货期:3-5 working days
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