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GeneQ18031023· pop 8· linked from 21 articles

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.

In the Vinony graph

Within Vinony's link graph, RAG2 is referenced by 21 other articles, and connects out to PubMed, N-terminus and human chromosome 11.

It is catalogued under the topic Genes on human chromosome 11.

Its subject is documented across 8 Wikipedia language editions.

Gene data

RAG2
Name
recombination activating 2
Type
protein-coding
Position
36,575,574–36,598,279 (−)
Aliases
RAG-2
RefSeq RNA
NM_000536.4, NM_001243785.2, NM_001243786.2, NR_033666.1, XM_047427385.1
RefSeq protein
NP_000527.2, NP_001230714.1, NP_001230715.1, XP_047283341.1, XP_047283342.1

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].

via MyGene.info

Gene · Ensembl

recombination activating 2

Symbol
RAG2
Biotype
Protein coding
Organism
Homo sapiens
Location
11:36,575,574-36,598,279
Strand
Reverse (−)
Assembly
GRCh38
View on Ensembl →

via Ensembl · EMBL-EBI

Wikidata facts

Instance of
gene
Show 8 more facts
HomoloGene ID
7507
found in taxon
Homo sapiens
genomic end
36619829
genomic start
36597124
cytogenetic location
11p12
expressed in
muscle tissue
Sources (7)

via Wikidata · CC0

~4 min read

Encyclopedic overview

5 sections
Contents
  • Structure
  • Function
  • Clinical significance
  • RAG2 knockout mice
  • References

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.

Excerpted from Wikipedia’s “RAG2” article, available under the CC BY-SA 4.0 licence.

Available in 8 languages

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