The approximate to 1-Mb leukocyte receptor complex at 19q13.4 is a key poly
morphic immunoregion containing all of the natural killer-receptor KIR and
related ILT genes. When the organization of the leukocyte receptor complex
was compared from two haplotypes, the gene content in the KIR region varied
dramatically, with framework loci flanking regions of widely variable gene
content. The ILT genes were more stable in number except for ILT6, which w
as present only in one haplotype. Analysis of Alu repeats and comparison of
KIR gene sequences, which are over 90% identical, are consistent with a re
cent origin. KIR genesis was followed by extensive duplication/deletion as
well as intergenic sequence exchange, reminiscent of MHC class I genes, whi
ch provide KIR ligands.