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Anti-RAG2 Antibody

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产品名称: Anti-RAG2 Antibody
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Anti-RAG2 Antibody


Anti-RAG2 Antibody  的详细介绍
Name: Anti-RAG2 Antibody
See all RAG2 primary antibodies
Description: Rabbit polyclonal antibody to RAG2
Applications: WB, IHC, IF
Dilutions: Western blotting: 1:500-1:3000 Immunohistochemistry: 1:100-1:500 Immunofluorescence: 1:100-1:200
Reactivity: Human
Immunogen: Recombinant protein fragment contain a sequence corresponding to a region within amino acids 271 and 519 of RAG2
Host: Rabbit
Clonality: Polyclonal
Conjugate: Unconjugated
Purification: Purified by antigen-affinity chromatography.
Predicted MW: 59kd
Formulation: Supplied in 0.1M Tris-buffered saline with 10% Glycerol (pH7.0). 0.01% Thimerosal was added as a preservative.
Storage: Store at -20?C for long term preservation (recommended). Store at 4?C for short term use.
Function: 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 (By similarity).
Tissue Specificity: Cells of the B- and T-lymphocyte lineages.
Involvement in Disease: Combined cellular and humoral immune defects with granulomas: Immunodeficiency disease with granulomas in the skin, mucous membranes, and internal organs. Other characteristics include hypogammaglobulinemia, a diminished number of T and B-cells, and sparse thymic tissue on ultrasonography.

Severe combined immunodeficiency autosomal recessive T-cell-negative/B-cell-negative/NK-cell-positive: A form of severe combined immunodeficiency (SCID), a genetically and clinically heterogeneous group of rare congenital disorders characterized by impairment of both humoral and cell-mediated immunity, leukopenia, and low or absent antibody levels. Patients present in infancy recurrent, persistent infections by opportunistic organisms. The common characteristic of all types of SCID is absence of T-cell-mediated cellular immunity due to a defect in T-cell development.

Omenn syndrome: Severe immunodeficiency characterized by the presence of activated, anergic, oligoclonal T-cells, hypereosinophilia, and high IgE levels.
Sequence Similarities: Belongs to the RAG2 family.
Cellular Location: Nucleus.
Database Links:
  • Entrez Gene: 5897?Human
  • Omim: 179616?Human
  • SwissProt: P55895?Human
  • Unigene: 714519?Human
    		•
    Synonyms:
  • RAG 2 Antibody
  • RAG-2 Antibody
  • RAG2 Antibody
  • RAG2_HUMAN Antibody
  • Recombination activating gene 2 Antibody
  • V(D)J recombination activating protein 2 Antibody
  • V(D)J recombination-activating protein 2 Antibody
    		•
    Information: Target information shown above is from the UniProt Consortium.
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