Overview
| 别名 | Beta-galactosidase; Acid beta-galactosidase; Lactase; Elastin receptor 1 |
| 基因名 | GLB1 |
| UniProt ID | P16278 |
| 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 | 8S8-R2-P4 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 76 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | IHC-1:200-1:250 |
| 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 | Galactosidase beta 1 (GLB1) is a lysosomal enzyme encoded by the GLB1 gene, primarily responsible for breaking down GM1 gangliosides and keratan sulfate, which are critical for neuronal function and connective tissue integrity. It cleaves terminal β-galactose residues from glycoconjugates, enabling cellular recycling processes. Additionally, GLB1 encodes the elastin-binding protein, which forms part of the elastin receptor complex involved in elastic fiber assembly in connective tissues. This dual functionality underscores its importance in both metabolic and structural cellular roles. Mutations in GLB1 can lead to lysosomal storage disorders such as GM1 gangliosidosis and Morquio syndrome type B. These conditions result from impaired enzymatic activity, causing toxic accumulation of substrates like GM1 gangliosides, leading to neurodegeneration, skeletal abnormalities, and other systemic symptoms. |
检测原理