Recombination activating gene 2 (RAG2)

Recombination activating gene 2 (RAG2) encodes a pivotal component of the V(D)J recombinase, a complex essential for generating immunological diversity during B and T lymphocyte development[1][2][5][7]. In collaboration with RAG1, RAG2 initiates site-specific cleavage of DNA at recombination signal sequences, enabling antigen receptor gene rearrangement that underlies adaptive immunity[1][4][6]. The protein contains a core domain that binds DNA and a C-terminal plant homeodomain (PHD) finger that interacts with chromatin marked by H3K4me3, positioning recombinase activity at accessible loci[3][5][6]. RAG2 is tightly regulated during the cell cycle, with controlled expression and degradation to prevent inappropriate DNA breaks and preserve genomic stability[6]. Defects or mutations in RAG2 result in profound immunodeficiency (e.g., Omenn syndrome), autoimmunity, and increase the risk of lymphoid malignancies due to aberrant recombination[5][6][7]. No approved drugs directly target RAG2, though it is a topic of study in immunogenetics and gene therapy.

Other names

V(D)J recombination-activating protein 2RAG2RAG-2Recombination activating gene 2

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