Contents

Search

V(D)J recombinase-2 (V(D)J recombination-activating protein, RAG2)

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: nucleus Expression: cells of the B- & T-lymphocyte lineages Pathology: - defects in RAG2 are a cause of severe combined immunodeficiency, B-cell-negative - Omenn syndrome

Related

recombination-activating gene-2

General

recombinase

Properties

SIZE: entity length = 527 aa MW = 59 kD COMPARTMENT: cell nucleus CELL: lymphocyte MOTIF: active site

Database Correlations

OMIM correlations MORBIDMAP 179616 UniProt P55895 Pfam PF03089 Kegg hsa:5897

References

  1. 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: 8533154
  2. RAG2base: RAG2 deficiency database http://bioinf.uta.fi/RAG2base/
  3. GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=RAG2
  4. UniProt :accession P55895

Component-of

RAG1/RAG2 complex