Anti-PEX5 Polyclonal Antibody
品牌:solarbio | 货号:K004208P
| 英文名称 | Anti-PEX5 Polyclonal Antibody |
|---|---|
| 宿主 | Rabbit |
| 别名 | PBD2A;PBD2B;PTS1-BP;PTS1R;PXR1;RCDP5 |
| 应用 | WB IHC IF |
| 稀释比例 | WB 1:500-2000. IHC 1:50-200. IF 1:50-200. |
| 交叉反应 | Human Mouse Rat |
| 蛋白分子量 | 71kDa |
| Gene ID | 5830 |
| 保存 | Store at -20°C. Avoid freeze / thaw cycles. |
| 储存液 | Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| 纯化方法 | Affinity purification |
| 亚型 | IgG |
| 免疫原 | Recombinant protein of human PEX5 |
| 性状 | 液体 |
| Public Immunogen Range | Recombinant protein of human PEX5 |
| Subcellular Locations | Cytoplasm Peripheral membrane protein Peroxisome membrane |
| Swiss Prot | P50542 |
| 克隆类型 | Polyclonal Antibody |
| 背景资料 | The product of this gene binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. Peroxins (PEXs) are proteins that are essential for the assembly of functional peroxisomes. The peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous autosomal recessive, lethal diseases characterized by multiple defects in peroxisome function. The peroxisomal biogenesis disorders are a heterogeneous group with at least 14 complementation groups and with more than 1 phenotype being observed in cases falling into particular complementation groups. Although the clinical features of PBD patients vary, cells from all PBD patients exhibit a defect in the import of one or more classes of peroxisomal matrix proteins into the organelle. Defects in this gene are a cause of neonatal adrenoleukodystrophy (NALD), a cause of Zellweger syndrome (ZWS) as well as may be a cause of infantile Refsum disease (IRD). Alternatively spliced transcript variants encoding different isoforms have been identified. |