Overview
| 别名 | Alpha-N-acetylgalactosaminidase; Alpha-galactosidase B |
| 基因名 | NAGA |
| UniProt ID | P17050 |
| 反应种属 | Human |
| 应用 | IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 5A2-Z2-E3 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 46 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | IHC-1:200 |
| 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 | Alpha-N-acetylgalactosaminidase (NAGA), also known as nagalase, is a lysosomal exoglycosidase enzyme that specifically removes terminal alpha-N-acetylgalactosamine residues from glycolipids and glycopeptides, playing a crucial role in the degradation of glycolipids and glycoproteins within the lysosome. Structurally, NAGA shares conserved active site residues with the closely related alpha-galactosidase A, and its catalytic mechanism has been elucidated through X-ray crystallography, revealing the basis for substrate specificity and the effects of pathogenic mutations. NAGA is also notable for its potential application in blood group conversion, as it can help produce type O "universal donor" blood from type A blood by removing specific carbohydrate residues. Deficiency or mutations in the NAGA gene lead to Schindler disease, a lysosomal storage disorder characterized by the accumulation of undegraded carbohydrates, resulting in neurological and systemic symptoms. |
检测原理