The killer cell immunoglobulin-like receptors (KIR) are a recently discovered family of activating and inhibitory receptors which control natural killer (NK) cell function. KIR exist as a diverse family of receptors that have evolved rapidly by both gene duplication and recombination events. These findings were unexpected for a family of genes involved primarily in the innate immune response. Human leukocyte antigen (HLA) class I molecules serve as ligands for the KIR. Several disease association studies indicate a role for interactions between these loci in infectious diseases, autoimmune/inflammatory disorders, cancer and reproduction. Emerging functional data supports a mechanism based on a continuum of inhibition to activation through various compound KIR-HLA genotypes in diseases. This review summarizes the major features of these genes and discusses how they may be involved in both disease pathogenesis and its amelioration.

Keywords: Innate Immunity, NK Cells, KIR, HLA, Disease

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