Rabbit Anti-Human ARSB pAb
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产品编号
PA7837
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别名
ARSB, ASB, G4S, MPS6
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规格
- 50ul
- 100ul
| Catalog Number | PA7837 |
| Alias | ARSB, ASB, G4S, MPS6 |
| Size | 50ul, 100ul |
| Product Full Name | Rabbit Anti-Human ARSB Polyclonal Antibody |
| Molecular Name | ARSB |
| Host | Rabbit |
| Clonity | Polyclonal |
| Purification | Affinity Purification |
| Storage Condition | Store the product under sterile conditions after opening at -20℃. Avoid freeze-thaw cycles. |
| Formulation | Supply in PBS with 0.02% sodium azide, 50% glycerol, pH 7.4. |
| Shipping Condition | Shipped on ice packs. |
| Immunogen | Recombinant fusion protein containing a sequence corresponding to a.a. 404-533 of human ARSB (NP_000037.2). |
| Application | WB |
| Product Declaration | 本产品仅用于研究用途(RUO)。不能用于诊断或直接用于人体实验。 |
ARSB(芳基硫酸酯酶B)降解硫酸皮肤素和硫酸软骨素,其缺陷导致黏多糖贮积症VI型(Maroteaux-Lamy综合征),表现为骨骼畸形、角膜混浊及心脏病变。
可以,推荐通过尿液糖胺聚糖电泳或WB检测患者样本中ARSB活性缺失。
经测试无交叉反应,特异性针对ARSB。
样本预处理使用肝素酶消化,减少糖胺聚糖对电泳的影响。
分装后-20℃保存,稀释后4℃保存不超过1周,有效期2年。
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