Human V(D)J recombination-activating protein 2 ELISA Kit | RAG2 elisa kit

Human V(D)J recombination-activating protein 2 ELISA Kit

Cat. Num. AAA2881540

• Gene Names: RAG2; RAG-2
• Reactivity: Human
• Synonyms: V(D)J recombination-activating protein 2; Human V(D)J recombination-activating protein 2 ELISA Kit; RAG-2; RAG2; RAG2 elisa kit

• Reactivity: Human
• Sequence Length: 527

• Assay Type: Sandwich
• Detection Range: 0.312-20 ng/mL
• Sensitivity: 0.12ng/mL
• Intra-Assay CV: <=3.8%
• Inter-Assay CV: <=6.7%
• Recovery: 87%
• Preparation and Storage: For long term storage, please store the entire kit at -20 degree C.

Typical Testing Data/Standard Curve (for reference only)

NCBI and Uniprot Product Information

• NCBI GI #: 151301080
• NCBI GeneID: 5897
• NCBI Accession #: NP_000527.2
• NCBI GenBank Nucleotide #: NM_000536.3
• UniProt Accession #: P55895; Q8TBL4; A8K9E9
• Molecular Weight: 59,241 Da
• NCBI Official Full Name: V(D)J recombination-activating protein 2
• NCBI Official Synonym Full Names: recombination activating gene 2
• NCBI Official Symbol: RAG2
• NCBI Official Synonym Symbols: RAG-2
• NCBI Protein Information: V(D)J recombination-activating protein 2
• UniProt Protein Name: V(D)J recombination-activating protein 2
• Protein Family: V(D)J recombination-activating protein
• UniProt Gene Name: RAG2
• UniProt Synonym Gene Names: RAG-2
• UniProt Entry Name: RAG2_HUMAN

NCBI Description

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]

Uniprot Description

RAG2: 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: 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 the RAG complex, RAG2 is not the catalytic component but is required for all known catalytic activities mediated by RAG1. It probably acts as a sensor of chromatin state that recruits the RAG complex to H3K4me3. Component of the RAG complex composed of core components RAG1 and RAG2, and associated component HMGB1 or HMGB2. Cells of the B- and T-lymphocyte lineages. Belongs to the RAG2 family.

Protein type: Enzyme, misc.

Chromosomal Location of Human Ortholog: 11p13

Cellular Component: nucleoplasm

Molecular Function: chromatin binding; DNA binding; methylated histone residue binding; phosphatidylinositol-3,4,5-triphosphate binding; phosphatidylinositol-3,4-bisphosphate binding; phosphatidylinositol-4,5-bisphosphate binding; phosphoinositide binding; zinc ion binding

Biological Process: B cell differentiation; B cell homeostatic proliferation; B cell lineage commitment; chromatin modification; positive regulation of organ growth; pre-B cell allelic exclusion; protein ubiquitination; T cell differentiation in the thymus; T cell lineage commitment; V(D)J recombination

Disease: Combined Cellular And Humoral Immune Defects With Granulomas; Omenn Syndrome; Severe Combined Immunodeficiency, Autosomal Recessive, T Cell-negative, B Cell-negative, Nk Cell-positive