NP_000439.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
106,179 Da
NCBI Official Full Name
V(D)J recombination-activating protein 1
NCBI Official Synonym Full Names
recombination activating 1
NCBI Official Synonym Symbols
NCBI Protein Information
V(D)J recombination-activating protein 1
UniProt Protein Name
V(D)J recombination-activating protein 1
UniProt Synonym Protein Names
RING finger protein 74Including the following 2 domains:Endonuclease RAG1 (EC:3.1.-.-)E3 ubiquitin-protein ligase RAG1 (EC:2.3.2.27)Alternative name(s):RING-type E3 ubiquitin transferase RAG1Curated
UniProt Synonym Gene Names
NCBI Summary for RAG1
The protein encoded by this gene is involved in activation of immunoglobulin V-D-J recombination. The encoded protein is involved in recognition of the DNA substrate, but stable binding and cleavage activity also requires RAG2. Defects in this gene can be the cause of several diseases. [provided by RefSeq, Jul 2008]
UniProt Comments for RAG1
RAG1: Catalytic 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. In the RAG complex, RAG1 mediates the DNA-binding to the conserved recombination signal sequences (RSS) and catalyzes the DNA cleavage activities by introducing a double-strand break between the RSS and the adjacent coding segment. RAG2 is not a catalytic component but is required for all known catalytic activities. DNA cleavage occurs in 2 steps: a first 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. The chromatin structure plays an essential role in the V(D)J recombination reactions and the presence of histone H3 trimethylated at 'Lys-4' (H3K4me3) stimulates both the nicking and haipinning steps. The RAG complex also plays a role in pre-B cell allelic exclusion, a process leading to expression of a single immunoglobulin heavy chain allele to enforce clonality and monospecific recognition by the B-cell antigen receptor (BCR) 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, preventing accessibility to the RAG complex and recombination of the second allele. In addition to its endonuclease activity, RAG1 also acts as a E3 ubiquitin-protein ligase that mediates monoubiquitination of histone H3. Histone H3 monoubiquitination is required for the joining step of V(D)J recombination. Mediates polyubiquitination of KPNA1. Homodimer. Component of the RAG complex composed of core components RAG1 and RAG2, and associated component HMGB1 or HMGB2. Maturing lymphoid cells. Belongs to the RAG1 family.
Protein type: DNA-binding; EC 3.1.-.-; EC 6.3.2.-; EC 6.3.2.19; Ligase; Ubiquitin conjugating system; Ubiquitin ligase
Chromosomal Location of Human Ortholog: 11p12
Cellular Component: nucleoplasm; nucleus
Molecular Function: DNA binding; endonuclease activity; histone binding; metal ion binding; protein binding; protein homodimerization activity; sequence-specific DNA binding; ubiquitin-protein ligase activity; zinc ion binding
Biological Process: B cell differentiation; DNA recombination; histone monoubiquitination; immune response; pre-B cell allelic exclusion; protein autoubiquitination; T cell differentiation in the thymus; V(D)J recombination
Disease: Alpha/beta T-cell Lymphopenia With Gamma/delta T-cell Expansion, Severe Cytomegalovirus Infection, And Autoimmunity; Combined Cellular And Humoral Immune Defects With Granulomas; Omenn Syndrome; Severe Combined Immunodeficiency, Autosomal Recessive, T Cell-negative, B Cell-negative, Nk Cell-positive
Research Articles on RAG1
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Pathways associated with RAG1 elisa kit
Diseases associated with RAG1 elisa kit
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