RAG2 Rabbit anti-Human, Polyclonal, Bioss

Description

• RAG2 (V(D)J recombination-activating protein 2) is a core component of the RAG complex - a multiprotein complex that mediates the DNA cleavage phase during V(D)J recombination
• V(D)J recombination assembles a diverse repertoire of immunoglobulin and T-cell receptor genes in developing B and T-lymphocytes through rearrangement of different V (variable), in some cases D (diversity) and J (joining) gene segments
• DNA cleavage by the RAG complex occurs in 2 steps: 1) first a nick is introduced in the top strand immediately upstream of the heptamer, generating a 3′-hydroxyl group that can attack the phosphodiester bond on the opposite strand in a direct transesterification reaction, thereby creating 4 DNA ends
• 2) hairpin coding ends and 2 blunt, 5′-phosphorylated ends are created
• The chromatin structure plays an essential rold in the V(D)J recombination reactions and the presence of histone H3 trimethylated at ′Lys-4′ (H3K4me3) stimulates both the nicking and hair pinning expressed on individual B-lymphocytes
• The introduction of DNA breaks by the RAG complex on one immunoglobulin allele induces ATM-dependent repositioning of the other allele to pericentromeric heterochromatin
• This prevents accessibility to the RAG complex and recombination of the second allele
• In the RAG complex, RAG2 is not the catalytic component but is required for all known catalytic activities mediated by RAG1
• Mutations affecting the gene can results in combined cellular and humoral immune defects with granulomas, severe combined immunodeficiency autosomal recessive T-cell-negative/B-cell-negative/NK-cell-positive, and Omenn syndrome.