Journal of Experimental Medicine
Human light chain genes are used in a κ before λ order. Accompanying this hierarchy is the rearrangement of a κ-deleting element (Kde) which eliminates the kappa locus before λ gene rearrangement. In approximately 60% of rearrangements the Kde recombines at a conserved heptamer within the Jκ-Cκ intron. We demonstrated that aberrant V/J rearrangements possessing apparent "N" nucleotides existed 5' to the Jκ-Kde rearrangements. This suggests that the Kde may selectively eliminate nonfunctional V/J alleles. A κ-producing cell that displayed the unusual finding of λ gene rearrangement demonstrated a rearranged Kde. This rearrangement was a Vκ/Kde recombination and the heptamer-11 bp spacer-nonamer flanking the Vκ is the target site of the Kde 40% of the time. The mouse possesses a counterpart to the Kde (recombining sequence [RS]) and the highly conserved regions surround the heptamer-spacer-nonamer signals. No complete protein product was predicted from the germline Kde near its break-point and no consistent fusion product was predicted from either the V/Kde or V/J-Kde rearrangements. A distal portion of the Kde is duplicated and is present at 2q11 as well as 2p11. The evolutionary conservation of the kappa-elimination event, the duplication and maintenance of the Kde indicates that it has a function. A portion of the Kde may still prove to encode a trans-acting factor that directly affects λ rearrangement. A certain role for the Kde is its site-specific rearrangement, which destroys ineffective κ genes and sets the stage for λ gene utilization.
Graninger, Winfried B.; Paula L. Goldman; Cynthia C. Morton; Stephen J. O'Brien; and Stanley J. Korsmeyer. 1988. "The κ-Deleting Element: Germline and Rearranged, Duplicated and Dispersed Forms." Journal of Experimental Medicine 167, (2): 488-501. https://nsuworks.nova.edu/cnso_bio_facarticles/197