Also known as RAG-2, recombination activating gene 2, recombination activating 2
Recombination activating gene 2 protein (also known as RAG-2) is a lymphocyte-specific protein encoded by the RAG2 gene on human chromosome 11. Together with the RAG1 protein, RAG2 forms a V(D)J recombinase, a protein complex required for the process of V(D)J recombination during which the variable regions of immunoglobulin and T cell receptor genes are assembled in developing B and T lymphocytes. Therefore, RAG2 is essential for the generation of mature B and T lymphocytes.
This gene encodes a protein that is involved in the initiation of 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 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 six-bladed propeller in the active core that serves as a binding scaffold for the tight association of the complex 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. [provided by RefSeq, Jul 2008].
Recombination activating gene 2 protein (also known as RAG-2) is a lymphocyte-specific protein encoded by the RAG2 gene on human chromosome 11. Together with the RAG1 protein, RAG2 forms a V(D)J recombinase, a protein complex required for the process of V(D)J recombination during which the variable regions of immunoglobulin and T cell receptor genes are assembled in developing B and T lymphocytes. Therefore, RAG2 is essential for the generation of mature B and T lymphocytes.
== Structure == RAG2 is a 527-amino acid long protein. Its N-terminal part is thought to form a six-bladed propeller in the active core. RAG2 is conserved among all species that carry out V(D)J recombination and its expression pattern correlates precisely with V(D)J recombinase activity. RAG2 is expressed in immature lymphoid cells. While the amount of RAG1 is constant during the cell cycle, the RAG2 accumulates mainly in the G0 and G1 phases of the cell cycle and it undergoes rapid degradation when the cell enters the S phase. This serves as an important regulatory mechanism of V(D)J recombination and a prevention of genomic instability.
Cellular component
via MyGene.info
Discovered by embedding cosine similarity (sentence-transformers MiniLM, 384-dim).