Overview
| 别名 | Lariat debranching enzyme |
| 基因名 | DBR1 |
| UniProt ID | Q9UK59 |
| 反应种属 | Human |
| 应用 | WB,IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 9I9-C4-Z8 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 61 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:4000; IHC-1:100-1:200 |
| 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 | Debranching RNA lariats 1 (DBR1) is an evolutionarily conserved protein that acts as the sole enzyme responsible for hydrolyzing the 2′–5′ phosphodiester bond in lariat RNAs, which are looped byproducts formed during pre-mRNA splicing. DBR1’s catalytic activity is highly specific, and its function is essential for efficient RNA processing, as failure to debranch lariat RNAs causes their abnormal accumulation and disrupts gene expression regulation. Structurally, DBR1 requires cofactors such as Fe²⁺ and Zn²⁺ and interacts with regulatory partners like DRN1 and TTDN1 to enhance its debranching kinetics. The enzyme is predominantly nuclear and its debranching role is conserved from yeast to humans, with >70% of human genes containing introns whose turnover depends on DBR1. Deficiency of DBR1 leads to severe cellular consequences, such as embryonic lethality in animals and plants, and is implicated in human diseases including autosomal recessive brainstem viral-induced encephalitis, lethal congenital ichthyosis, and potentially neurodegenerative conditions like ALS. |
检测原理