V(D)J recombinase-2 (V(D)J recombination-activating protein, RAG2)
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Function
- role in V(D)J recombination
- RAG1/RAG2 complex initiates V(D)J recombination by binding to the conserved recombination signal sequences (RSS) & introducing a double-strand break between the RSS & the adjacent coding segment
- breaks are generated in 2 steps, nicking of one strand (hydrolysis), followed by hairpin formation (transesterification)
- RAG1/RAG2 complex functions as a transposase in vitro, has RSS-independent endonuclease activity (end processing) & hairpin opening
- RAG1 alone can bind to RSS but stable, efficient binding requires RAG2
- all known catalytic activities require the presence of both proteins
- RAG complexes appear to contain 3-5 molecules of RAG2 for each molecule of RAG1
Structure
belongs to the RAG2 family
Compartment
Expression
cells of the B- & T-lymphocyte lineages
Pathology
- defects in RAG2 are a cause of severe combined immunodeficiency, B-cell-negative
- Omenn syndrome
More general terms
Component of
References
- ↑ Jackson SP, Jeggo PA. DNA double-strand break repair and V(D)J recombination: involvement of DNA-PK. Trends Biochem Sci. 1995 Oct;20(10):412-5. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/8533154
- ↑ RAG2base: RAG2 deficiency database http://bioinf.uta.fi/RAG2base/
- ↑ GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=RAG2
- ↑ UniProt http://www.uniprot.org/uniprot/P55895.html