徐州威格顿生物科技有限公司
威格顿生物
Weigedun Biotechnology
UBR1 Mouse mAb
规格信息
| 规格 | 货号 |
|---|---|
| 50ul | WGD-Z-2611787 |
| 100ul | WGD-Z-2611787 |
详细参数
| 别名 | E3 ubiquitin-protein ligase UBR1; N-recognin-1; Ubiquitin-protein ligase E3-alpha-1; Ubiquitin-protein ligase E3-alpha-I |
| 基因名 | UBR1 |
| UniProt ID | Q8IWV7 |
| 反应种属 | Human |
| 应用 | WB |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 8F7-K6-D4 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 200 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 | Ubiquitin protein ligase E3 component n-recognin 1 (UBR1) is a large multifunctional E3 ubiquitin ligase that serves as a primary component of the N-end rule pathway. It recognizes and binds proteins with specific destabilizing N-terminal residues, targeting them for ubiquitination and subsequent proteasomal degradation. UBR1 features multiple substrate-binding sites and associates with E2 ubiquitin-conjugating enzymes such as Ubc2/Rad6 and Ubc4. In addition to its role in the N-end rule pathway, it functions in the quality control of misfolded cytosolic proteins, a process facilitated by Hsp70 chaperones. UBR1 is broadly expressed in human tissues, including the brain, liver, and pancreas. Mutations in the UBR1 gene are the established cause of Johanson-Blizzard syndrome, a rare autosomal recessive disorder characterized by exocrine pancreatic insufficiency and various congenital malformations. |