Overview
| 别名 | Arylsulfatase A; ASA; Cerebroside-sulfatase) [Cleaved into: Arylsulfatase A component B; Arylsulfatase A component C] |
| 基因名 | ARSA |
| UniProt ID | P15289 |
| 反应种属 | Human |
| 应用 | WB |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG2a |
| 克隆号 | 1Y1-J7-I3 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 53 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:1000 |
| 存储缓冲液 | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.09% sodium azide |
| 保存温度 | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| 背景信息 | Arylsulfatase A (ARSA) is a lysosomal enzyme responsible for the hydrolysis of cerebroside 3-sulfate into cerebroside and sulfate, a critical step in glycosphingolipid catabolism. This activity is essential for maintaining the integrity of myelin within the nervous system. Structurally, ARSA requires the post-translational oxidation of an active-site cysteine to formylglycine for catalytic function and coordinates a divalent metal ion to facilitate sulfate ester hydrolysis. Beyond its lysosomal role, ARSA participates in lipid metabolism and sperm-egg binding. Pathogenic loss-of-function variants in the ARSA gene lead to metachromatic leukodystrophy, an autosomal recessive lysosomal storage disorder characterized by sulfatide accumulation, progressive demyelination, and severe neurological decline. |
检测原理