RAG2

Recombination activating gene 2 also known as RAG-2 is a is_associated_with::protein that in humans is encoded by the RAG2 is_associated_with::gene.

Function
This gene encodes a protein that is involved in the initiation of is_associated_with::V(D)J recombination during B and T cell development. This protein forms a complex with the product of the adjacent recombination activating gene 1, and this complex can form is_associated_with::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 is_associated_with::N-terminal of the recombination activating gene 2 component is thought to form a six-bladed propeller in the active core that serves as a binding scaffold for the tight association of the complex with DNA. A is_associated_with::C-terminal plant is_associated_with::homeodomain finger-like motif in this protein is necessary for interactions with is_associated_with::chromatin components, specifically with is_associated_with::histone H3 that is trimethylated at lysine 4.

Clinical significance
Mutations in this gene cause is_associated_with::Omenn syndrome, a form of severe combined is_associated_with::immunodeficiency associated with is_associated_with::autoimmune-like symptoms.