Human Alpha-Galactosidase A/GLA ELISA Kit/人α-半乳糖苷酶A / GLA ELISA试剂盒
货号：EH21RB,EH21RBX5,EH21RBX10
规格：96 Tests,5X96 Tests,10X96 tests
价格：6641,29884,57271
产品类型：Platinum ELISA
品牌：Thermo Fisher
物种：人
宿主：其它
抗体亚型：其它
荧光染料：其它
灵敏度 | 45 pg/mL |
| 检测范围 | 45-10,000 pg/mL |
样本类型/体积 | 血清 50 μL 血浆 50 μL 上清液 50 μL |
操作时间 | 1 hr 20 min |
测试时间 | 4 hr 45 min |
Homogenous (no wash) | No |
| 批间变异系数（CV） | <12% |
| 批内变异系数（CV） | <10% |
仪器设备 | Colorimetric Microplate Reader 酶标仪 |
规格 | 5 x 96 Tests |
组份 | - 预包被96孔板 - 标准品 - 测定稀释浓缩液 - 生物素化检测抗体 - SAV-HRP - 洗涤液 - 显色剂 - 终止液 - 胶板盖 |
储存温度 | 2-8℃ |
| Protein name | alpha Galactosidase |
| Protein aliases | Agalsidase, agalsidase alfa, Alpha-D-galactosidase A, Alpha-D-galactoside galactohydrolase, alpha-D-galactoside galactohydrolase 1, alpha-gal A, Alpha-galactosidase A, galactosidase, alpha, Melibiase |
物种（Tested） | 人 |
试剂盒类型 | Sandwich ELISA Kit |
标记（染料） | HRP |
| 检测抗体偶联物 | Biotin |
| Gene aliases | GALA, GLA |
| Gene ID | (Human) 2717 |
| Gene symbol | GLA |
| UniProt ID | (Human) P06280 |
| Human Alpha-Galactosidase A/GLA quantitates human Alpha-Galactosidase A/GLA in serum, plasma, supernatant. The assay will exclusively recognize both natural and recombinant human Alpha-Galactosidase A/GLA.人α-半乳糖苷酶A / GLA定量测定血清，血浆，上清液中的人α-半乳糖苷酶A / GLA。 该检测试剂盒将专门识别天然和重组人α-半乳糖苷酶A / GLA。Target information 靶标信息This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties.该基因编码同二聚体糖蛋白，其水解糖脂和糖蛋白的末端α-半乳糖基部分。 该酶主要水解神经酰胺三己糖苷，并且可以催化蜜二糖水解为半乳糖和葡萄糖。 该基因中的多种突变会影响该酶的合成，加工和稳定性，从而导致法布里病（Fabry病），这是一种罕见的溶酶体贮积病，由无法分解α-D-半乳糖基糖脂部分引起。For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.仅供研究使用。 不用于诊断过程。 未经明确授权不得转售。 |
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