人 RAG1 抗体对
01AB01820
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.
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技术参数
- 靶点基因名
- RAG1
- 基因别名
- RNF74
- 蛋白别名
- recombination activating 1; RAG1
- UniProt
- P15918
- 物种
- Human
产品详情
- 靶点名称
- RAG1
- 蛋白名称
- recombination activating 1
- 蛋白别名
- 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]
- 靶点基因名
- RAG1
- 基因别名
- RNF74
- UniProt
- P15918
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
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.