title: The killer immunoglobulin-like receptor gene cluster: tuning the genome for defense.
abstract: Killer immunoglobulin-like receptors (KIRs) are molecules expressed on the surface of natural killer (NK) cells, which play an important role in innate immunity. KIR recognition of major histocompatability complex (MHC) class I allotypes represents one component of the complex interactions between NK cells and their targets in determining NK cell reactivity. KIRs are encoded by a gene cluster at human chromosome 19q13.4. Despite their high degree of sequence identity, KIR genes encode proteins that have diverse recognition patterns (specific HLA class I allotypes) and confer opposing signals (activating or inhibitory) to the NK cell. The KIR gene cluster is highly polymorphic, with individual genes exhibiting allelic variability and individual haplotypes differing in gene content. The polymorphism of the KIR locus parallels that of the MHC, facilitating the adaptation of the immune system to a dynamic, challenging environment. This variation is associated with a growing number of human diseases, which is likely to extend to levels observed for the HLA loci. Here we review current progress in understanding KIR biology and genetics.
authors.affiliation: Johns Hopkins University School of Medicine, Baltimore, Maryland 21231, USA.
authors.forename: Arman A
authors.initials: AA
authors.lastname: Bashirova
authors.forename: Maureen P
authors.initials: MP
authors.lastname: Martin
authors.forename: Daniel W
authors.initials: DW
authors.lastname: McVicar
authors.forename: Mary
authors.initials: M
authors.lastname: Carrington
mesh_terms: D015894:Genome, Human / Q000276:immunology*; D006801:Humans; D005810:Multigene Family; D011110:Polymorphism, Genetic / Q000276:immunology; D054340:Receptors, KIR / Q000235:genetics* / Q000276:immunology
chemical_list: D054340:Receptors, KIR