Printable factfile
Gene Information
Names
HUGO name: RAG1
Alias(es):
- recombination activating gene 1
- V(D)J recombination activating protein 1 (RAG-1)
Localization
Reference sequences
Chromosomal location
Maps
Other gene-based resources
Protein Information
Description
Protein function:
During lymphocyte development, the genes encoding immunoglobulins and T cell receptors are assembled from Variable (V), Diversity (D), and Joining (J) gene segments. This combinatorial process, known as V(D)J recombination, allows the generation of an enormous range of binding specificities from a limited amount of genetic information. The RAG1/RAG2 complex initiates this process by binding to the conserved Recombination Signal Sequences (RSS) and introducing a double-strand break between the RSS and the adjacent coding segment. These breaks are generated in two steps, nicking of one strand (hydrolysis), followed by hairpin formation (transesterification). RAG1/2 has also been shown to function as a transposase in vitro, and to possess RSS-independent endonuclease activity (end processing) and hairpin opening. RAG1 alone can bind to RSS but stable, efficient binding requires RAG2. All known catalytic activities require the presence of both proteins.
Subcellular location:
Nuclear
Cofactor:
Binds 1 magnesium or manganese ion per subunit (by similarity)
Structures (PDB)
| 1RMD | Rag1 Dimerization Domain |