The kappa-deleting element. Germline and rearranged, duplicated and dispersed forms

W B Graninger, P L Goldman, C C Morton, S J O'Brien, S J Korsmeyer

Research output: Contribution to journalArticlepeer-review


Human light chain genes are used in a kappa before lambda order. Accompanying this hierarchy is the rearrangement of a kappa-deleting element (Kde) which eliminates the kappa locus before lambda gene rearrangement. In approximately 60% of rearrangements the Kde recombines at a conserved heptamer within the J kappa-C kappa intron. We demonstrated that aberrant V/J rearrangements possessing apparent "N" nucleotides existed 5' to the J kappa-Kde rearrangements. This suggests that the Kde may selectively eliminate nonfunctional V/J alleles. A kappa-producing cell that displayed the unusual finding of lambda gene rearrangement demonstrated a rearranged Kde. This rearrangement was a V kappa/Kde recombination and the heptamer-11 bp spacer-nonamer flanking the V kappa 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 lambda rearrangement. A certain role for the Kde is its site-specific rearrangement, which destroys ineffective kappa genes and sets the stage for lambda gene utilization.

Original languageEnglish
Pages (from-to)488-501
Number of pages14
JournalThe Journal of experimental medicine
Issue number2
Publication statusPublished - 1 Feb 1988


  • Alleles
  • Amino Acid Sequence
  • Base Sequence
  • Chromosome Deletion
  • Cloning, Molecular
  • Germ Cells
  • Humans
  • Immunoglobulin Joining Region
  • Immunoglobulin Variable Region
  • Immunoglobulin kappa-Chains
  • Molecular Sequence Data
  • Multigene Family
  • Recombination, Genetic
  • Transcription, Genetic
  • Journal Article
  • Research Support, Non-U.S. Gov't


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