Schematic representation of KIR region located on long arm of chromosome 19 (19q13.4) within the leukocyte receptor complex (LRC). The LRC complex constitutes a large cluster of genes that encode proteins containing immunoglobulin-like domains (NCR, KIR, LILR, and LAIR), signaling proteins (SIGLECs) and CD66 family proteins. A total of 14 functional KIR genes and 2 pseudogenes have been identified and organized into two broad haplotypes termed A and B. KIR genes of one haplotype are divided into centromeric and telomeric regions by the framework genes KIR3DL2, KIR2DL4, KIR3DP1 and KIR3DL3. KIR genes are highly variable with regard to both gene content and allelic polymorphism with 1110 alleles known to date (IPD-KIR Database on 1 October 2020).