Overview
| 别名 | V(D)J recombination-activating protein 1; RAG-1; RING finger protein 74) [Includes: Endonuclease RAG1; E3 ubiquitin-protein ligase RAG1; RING-type E3 ubiquitin transferase RAG1] |
| 基因名 | RAG1 |
| UniProt ID | P15918 |
| 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 | 3M9-P1-C8 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 119 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | 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 | Recombination activating 1 (RAG1) is the catalytic component of the RAG1–RAG2 complex, which mediates the critical DNA cleavage step of V(D)J recombination in developing B and T lymphocytes. This process enables the assembly of diverse immunoglobulin and T cell receptor genes necessary for adaptive immunity. RAG1 binds recombination signal sequences (RSS) to introduce site-specific double-strand breaks and possesses E3 ubiquitin-protein ligase activity that monoubiquitinates histone H3 to facilitate DNA repair. Pathogenic variants in RAG1 lead to a spectrum of primary immunodeficiencies, including classic severe combined immunodeficiency (SCID), Omenn syndrome, and atypical SCID. These conditions result from the graded loss of recombinase activity, which severely impairs lymphocyte development and immune function. |
检测原理