| 别名 | Alglucerase; betaGC; GBA1; GCase; GCB; GLUC; Glucosylceramidase; Imiglucerase; |
| 基因名称 | GBA |
| 蛋白名称 | GBA |
| 推荐应用 | |
| 反应物种 | Human Rat |
| 存储缓冲液 | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Human Gene Link | 2629; |
| Human Swissprot No. | P04062 |
| Mouse Gene ID | 14466; |
| Mouse Swissprot No. | P17439 |
| 特异性 | Endogenous |
| 稀释度 | WB 1:500~1:2000 |
| 参考分子量 | MW(Observed): 60 kDa |
| 预测分子量 | MW(Calculated): 60 kDa |
| 运输及保存条件 | -15°C to -25°C/1 year(Do not lower than -25°C) |
| 宿主 | Rabbit |
| 同种型 | IgG,Kappa |
| 背景介绍 | Defects in GBA are the cause of Gaucher disease (GD) [MIM:230800]; also known as glucocerebrosidase deficiency. GD is the most prevalent lysosomal storage disease, characterized by accumulation of glucosylceramide in the reticulo-endothelial system. |
| 细胞定位 | Lysosome membrane ; Peripheral membrane protein ; Lumenal side . Interaction with saposin-C promotes membrane association (PubMed:10781797) . Targeting to lysosomes occurs through an alternative MPR-independent mechanism via SCARB2 (PubMed:18022370) . . |
| 纯化 | Protein A |
| Clonality | Monoclonal |
| 修饰类型 | Unmodified |
| 细胞定位 | Lysosome membrane ; Peripheral membrane protein ; Lumenal side . Interaction with saposin-C promotes membrane association (PubMed:10781797) . Targeting to lysosomes occurs through an alternative MPR-independent mechanism via SCARB2 (PubMed:18022370) . . |
| 组织表达 | Liver, Spleen, Brain, Gaucher disease tissue, Hepatocellular carcinoma, Glioma |
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