KIR2DL4

killer cell immunoglobulin like receptor, two Ig domains and long cytoplasmic tail 4

Summary

Killer cell immunoglobulin-like receptors (KIRs) are transmembrane glycoproteins expressed by natural killer cells and subsets of T cells. The KIR genes are polymorphic and highly homologous and they are found in a cluster on chromosome 19q13.4 within the 1 Mb leukocyte receptor complex (LRC). The gene content of the KIR gene cluster varies among haplotypes, although several "framework" genes are found in all haplotypes (KIR3DL3, KIR3DP1, KIR3DL4, KIR3DL2). The KIR proteins are classified by the number of extracellular immunoglobulin domains (2D or 3D) and by whether they have a long (L) or short (S) cytoplasmic domain. KIR proteins with the long cytoplasmic domain transduce inhibitory signals upon ligand binding via an immune tyrosine-based inhibitory motif (ITIM), while KIR proteins with the short cytoplasmic domain lack the ITIM motif and instead associate with the TYRO protein tyrosine kinase binding protein to transduce activating signals. The ligands for several KIR proteins are subsets of HLA class I molecules; thus, KIR proteins are thought to play an important role in regulation of the immune response. This gene is one of the "framework" loci that is present on all haplotypes. Alternate alleles of this gene are represented on multiple alternate reference loci (ALT_REF_LOCs). Alternative splicing results in multiple transcript variants, some of which may not be annotated on the primary reference assembly. [provided by RefSeq, Jul 2016]

Known Variants60 total

rsidPosition (GRCh37)AllelesClassClinVar
rs89555428519:55,315,120C/Tuncertain significance
rs60407619:55,315,326A/Cbenign
rs74888866319:55,315,348T/Auncertain significance
rs54109998319:55,315,369T/Guncertain significance
rs36903332219:55,315,372G/Auncertain significance
rs145184315419:55,316,256G/Auncertain significance
rs251659207619:55,316,292G/Cuncertain significance
rs121058194019:55,316,295C/Tuncertain significance
rs76809476019:55,316,310G/Auncertain significance
rs61883519:55,316,329A/Cmissense variant
rs75811139419:55,316,331C/Tuncertain significance
rs86684760719:55,316,345C/Auncertain significance
rs37128127819:55,316,347T/Cuncertain significance
rs134742997819:55,316,353C/Tuncertain significance
rs143372137219:55,316,364A/Glikely benign
rs77452647019:55,316,375C/Glikely benign
rs123155504919:55,316,414T/Clikely benign
rs145350148719:55,316,438A/Tlikely benign
rs76375666219:55,316,444C/Guncertain significance
rs103253638219:55,316,459A/Cuncertain significance
rs79613892319:55,316,465A/Glikely benign
rs57741055619:55,316,477G/Alikely benign
rs79670726919:55,316,498G/Alikely benign
rs92668456919:55,316,511C/Auncertain significance
rs53967137719:55,317,414G/Auncertain significance
rs20141980019:55,317,425G/Alikely benign
rs77863488619:55,317,428T/Clikely benign
rs20048515919:55,317,439C/Guncertain significance
rs79613959319:55,317,489C/Auncertain significance
rs74634334019:55,317,490A/Glikely benign
rs11324804819:55,317,497C/Alikely benign
rs11226638719:55,317,506C/Glikely benign
rs76598654819:55,317,531G/Auncertain significance
rs124714650919:55,317,570G/Auncertain significance
rs95402314719:55,317,584C/Tlikely benign
rs54604416819:55,317,585G/Auncertain significance
rs120969626019:55,317,590C/Tlikely benign
rs75249408219:55,317,594C/Guncertain significance
rs117662938819:55,317,599T/Clikely benign
rs76265935419:55,317,612G/Auncertain significance
rs206051692619:55,317,637C/Auncertain significance
rs139721120819:55,317,647A/Clikely benign
rs120157383219:55,317,650T/Alikely benign
rs87897759019:55,317,656C/Tlikely benign
rs127220608719:55,317,666G/Auncertain significance
rs139967458019:55,317,668C/Guncertain significance
rs53017131819:55,317,670C/Tuncertain significance
rs7686940919:55,320,692A/Gintron variant
rs63171719:55,322,975G/A
rs206096865019:55,324,595T/Guncertain significance
rs64921619:55,324,635T/Cupstream gene variant
rs66077319:55,324,860G/C
rs65267119:55,325,123T/Cupstream gene variant
rs18759743319:55,325,173G/Clikely benign
rs131649317319:55,325,176A/Glikely benign
rs19982311919:55,325,194G/Alikely benign
rs20184091919:55,325,332C/Tlikely benign
rs144919443219:55,325,388G/Auncertain significance
rs37086965619:55,325,571G/Alikely benign
rs67644319:55,326,073T/Cupstream gene variant

Gene information from NCBI Gene. Variant classifications from ClinVar.