RAG2 (V(D)J recombination-activating protein 2) is involved in the initiation of V(D)J recombination during B and T cell development. This protein forms a comple, Xenopus/Amphibian, with the product of the adjacent recombination activating gene 1, this comple, Xenopus/Amphibian, can form double-strand breaks by cleaving DNA at conserved recombination signal sequences. The recombination activating gene 1 component is thought to contain most of the catalytic activity, while the N-terminal of the recombination activating gene 2 component is thought to form a si, Xenopus/Amphibian,-bladed propeller in the active core that serves as a binding scaffold for the tight association of the comple, Xenopus/Amphibian, with DNA. A C-terminal plant homeodomain finger-like motif in this protein is necessary for interactions with chromatin components, specifically with histone H3 that is trimethylated at lysine 4. Mutations in this gene cause Omenn syndrome, a form of severe combined immunodeficiency associated with autoimmune-like symptoms.