The RAG proteins are comprised of core endonuclease domains and non-core regions that modulate endonuclease activity. Mutation or deletion of non-core RAG regions in humans causes immunodeficiency and altered TCR repertoire, and mice expressing core but not full-length Rag1 (Rag1C/C) or Rag2 (Rag2C/C) exhibit lymphopenia, reflecting impaired V(D)J recombination and lymphocyte development. Rag1C/C mice display reduced D-to-J and V-to-DJ rearrangements of TCRβ and IgH loci, while Rag2C/C mice show decreased V-t…
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