KIR Genes

The first KIRs to be defined were inhibitory receptors, and when initially coined the acronym stood for 'Killer-cell Inhibitory Receptor'. With appreciation that this family of molecules included both activating and inhibitory receptors, the KIR acronym was retained and is now accepted as an abbreviation for Killer-cell Immunoglobulin-like Receptor (1). Unlike HLA genes, which for practical and historical reasons are named by the WHO Nomenclature Committee for Factors of the HLA System, the naming of KIR genes is the responsibility of the HUGO Genome Nomenclature Committee (HGNC) (2).

The names given to the KIR genes are based on the structures of the molecules they encode. The first digit following the KIR acronym corresponds to the number of Ig-like domains in the molecule, and the 'D' denotes 'domain'. The D is followed by either an 'L' indicating a 'Long' cytoplasmic tail, an 'S' indicating a 'Short' cytoplasmic tail, or a 'P' for pseudogenes. The final digit indicates the number of the gene encoding a protein with this structure. Thus KIR2DL1, KIR2DL2 and KIR2DL3 all encode receptors having two extracellular Ig-like domains and a long cytoplasmic tail (3).

Where two or more genes have very similar structures and have very similar sequences, they may be given the same number but distinguished by a final letter: for example, the KIR2DL5A and KIR2DL5B genes (4). The similarity of these two genes suggests they are related by a recent gene duplication event.

KIR Gene Names


Gene Symbol Protein Symbol Description Aliases
KIR2DL1 KIR2DL1 killer cell immunoglobulin-like receptor, two domains, long cytoplasmic tail, 1 47.11, CD158a,
cl-42, nkat1, p58.1
KIR2DL2 KIR2DL2 killer cell immunoglobulin-like receptor, two domains, long cytoplasmic tail, 2 CD158b1, cl-43, nkat6
KIR2DL3 KIR2DL3 killer cell immunoglobulin-like receptor, two domains, long cytoplasmic tail, 3 CD158b2, cl-6, nkat2, nkat2a, nkat2b, p58
KIR2DL4 KIR2DL4 killer cell immunoglobulin-like receptor, two domains, long cytoplasmic tail, 4 15.212, 103AS, CD158d
KIR2DL5A KIR2DL5A killer cell immunoglobulin-like receptor, two domains, long cytoplasmic tail, 5A CD158f, KIR2DL5.1
KIR2DL5B KIR2DL5B killer cell immunoglobulin-like receptor, two domains, long cytoplasmic tail, 5B KIR2DL5.2, KIR2DL5.3, KIR2DL5.4
KIR2DS1 KIR2DS1 killer cell immunoglobulin-like receptor, two domains, short cytoplasmic tail, 1 CD158h, EB6Actl, EB6Actll
KIR2DS2 KIR2DS2 killer cell immunoglobulin-like receptor, two domains, short cytoplasmic tail, 2 183Actl, CD158j,
cl-49, nkat5
KIR2DS3 KIR2DS3 killer cell immunoglobulin-like receptor, two domains, short cytoplasmic tail, 3 nkat7
KIR2DS4 KIR2DS4 killer cell immunoglobulin-like receptor, two domains, short cytoplasmic tail, 4 CD158i, cl-39, KKA3, nkat8
KIR2DS5 KIR2DS5 killer cell immunoglobulin-like receptor, two domains, short cytoplasmic tail, 5 CD158g, nkat9
KIR2DP1 KIR2DP1 killer cell immunoglobulin-like receptor, two domains, pseudogene 1 KIR2DL6, KIR15, KIRY, KIRZ
KIR3DL1 KIR3DL1 killer cell immunoglobulin-like receptor, three domains, long cytoplasmic tail, 1 AMB11, CD158e1, cl-2, cl-11, KIR, nkat3, NKB1, NKB1B
KIR3DL2 KIR3DL2 killer cell immunoglobulin-like receptor, three domains, long cytoplasmic tail, 2 CD158k, cl-5, nkat4, nkat4a, nkat4b
KIR3DL3 KIR3DL3 killer cell immunoglobulin-like receptor, three domains, long cytoplasmic tail, 3 CD158z, KIR3DL7, KIR44, KIRC1
KIR3DS1 KIR3DS1 killer cell immunoglobulin-like receptor, three domains, short cytoplasmic tail, 1 CD158e2, nkat10
KIR3DP1 KIR3DP1 killer cell immunoglobulin-like receptor, three domains, pseudogene, 1 CD158c, KIR2DS6, KIR3DS2P, KIR48, KIRX

References

All references are linked to the PubMed abstract where possible.

  1. Long EO, Colonna M, Lanier LL.
    Inhibitory MHC class I receptors on NK and T cells: a standard nomenclature.
    Immunology Today (1996) 17:100 (Abstract)
  2. Marsh SGE, Parham P, Dupont B, Geraghty DE, Trowsdale J, Middleton D, Vilches C, Carrington M, Witt C, Guethlein LA, Shilling H, Garcia CA, Hsu KC, Wain H.
    Killer-cell Immunoglobulin-like Receptors (KIR) Nomenclature Report, 2002
    Tissue Antigens (2003) 62:79-86 (Abstract)
    Immunogenetics (2003) 55:220-226 (Abstract)
    Human Immunology (2003) 64:648-654 (Abstract)
    European Journal of Immunogenetics (2003) 30:229-234 (Abstract)
    Journal of Immunological Methods (2003) 281:1-8
  3. Vilches C, Parham P.
    KIR: diverse, rapidly evolving receptors of innate and adaptive immunity.
    Annual Review of Immunology (2002) 20:217-51 (Abstract)
  4. Gomez-Lozano N, Gardiner CM, Parham P, Vilches C.
    Some human KIR haplotypes contain two KIR2DL5 genes: KIR2DL5A and KIR2DL5B.
    Immunogenetics (2002) 54:314-9 (Abstract)

IPD-KIR