RAG1 (D36B3) Rabbit mAb #3968

The sequences encoding antigen receptors are split into multiple germline segments, which are then combined by a process called V(D)J recombination during immune cell development. A variable (V) segment is combined with a joining (J) segment, and in some cases, a diversity (D) segment, to create the antigen-binding portion of the receptor. The recombined V(D)J segment is then spliced into exons that encode the constant region to produce mature mRNA (1,2). This essential process required for the development of functional immune T and B cells creates a vast diversity in these receptors (3,4). Initiation of this process follows binding of RAG1 (recombination activating gene 1) and RAG2 to the conserved recombination signal sequences (RSS) and the introduction of a double-strand break between the RSS and the coding sequence (5,6). RAG1 and RAG2 genes are located immediately adjacent to each other in the genome and lack introns in their coding regions in many species. RAG1 and RAG2 are co-expressed only in the B and T cell lineages, and both are required for cleavage activity (7). RAG1 and RAG2 can also function as transposases, contributing to chromosomal translocations and lymphoid malignancy (8,9). Mutations in the RAG genes are associated with a spectrum of combined immune deficiencies in humans (10,11).