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Nomenclature of the human LRC (Leucocyte Receptor Complex) genes: LILR/ILT and KIR (19q13.42)

There are two major cluster of genes for human NK receptors, the NKC (Natural Killer Complex) at 12p13.2 and the LRC (Leucocyte Receptor Complex) at 19q13.42. The LRC genes at 19q13.42 [1] comprises activating and inhibitory MHC receptors.

LRC genes are listed at 19q13.42 from the centromeric end of the LRC region (top of the table) to the telomeric end of the LRC region (bottom of the table). The number of KIR genes is different between haplotypes. None of the haplotypes described so far contained all the genes and some genes are only present in some haplotypes [2].

KIR haplotype A: 3DL3, 2DL3, 2DP1(Z), 2DL1, 3DP1(X), 2DL4, 3DL1, 2DS4, 3DL2
KIR haplotype B (AL133414): 3DL3, 2DL2, 3DP1(X), 2DL4, 3DS1, 2DL5, 2DS5, 2DS1, 2DS2, 3DL2

LILR/ILT are orthologous in humans and mice so primordial genes must have been present before speciation between man and rodents (about 80 million years ago), but KIR originally thought to be completely absent in rodents and only developed in primates [3], have now been detected in rodents [6].

Cells HUGO nomenclature Aliases CD designation [4] Sequences C (1) Function Motif Ligands
Dentritic cells
B lymphocytes
LILRB3 ILT5 LIR3, HL9 CD85a U91928 4 Inhibitory ITIM ?
LILR? ILT8 - CD85b AF041262    
LILRB5 - LIR8 CD85c AF025534 4 Inhibitory ITIM
LILRB2 ILT4 LIR2, MIR10 CD85d AF000574 4 Inhibitory ITIM HLA-A, B, F and G
LILRA3 ILT6 LIR4, HM31, HM43 CD85e U91926 4 (2) ?
LILR? ILT11 - CD85f AF212842 2 (3)
LILR? ILT7 - CD85g AF041261   (3)
LILRA2 ILT1 LIR7 CD85h U82275 4 (3)
LILRA1 - LIR6 CD85i AF025530 2 ou 3  
LILRB1 ILT2 LIR1, MIR7 CD85j AF009220 4 Inhibitory ITIM HLA-A, B, F and G
LILRB4 ILT3 LIR5, HM18 CD85k U82979 2 Inhibitory ITIM ?
LILR? ILT9 - CD85l AF072102    
LILR? ILT10 - CD85m AF072100    
NK cells
T cell subsets
KIR3DL3 A, B KIRC1, KIR44, KIR3DL7 CD158z AL133414 3 Inhibitory ITIM ?
KIR2DL3 A* p58.3 CD158b2 - 2 Inhibitory ITIM HLA-C (S77-N80, -Cw1, -CW3, -Cw7,  -Cw8)
KIR2DL2 B* p58.2 CD158b1 AL133414
KIR2DP1 A* KIRZ (P) - AF204908 - - -
KIR2DL1 A* p58.1 CD158a - 2 Inhibitory ITIM HLA-C (N77-K80, -Cw2, -Cw4, -Cw5, -Cw6)
CD158c AF133414 -   -
KIR2DL4 A, B   CD158d AL133414 2 Inhibitory ITIM HLA-G
KIR3DL1 A** p70 CD158e1 - 3 Inhibitory ITIM HLA-B (I80, Bw4)
KIR3DS1 B** p70 CD158e2 AL133414 Activator (4) ?
KIR2DL5A (5) B   CD158f AL133414 2 Inhibitory ITIM ?
KIR2DL5B (5)     - - 2 Inhibitory ITIM ?
KIR2DS3 -   - - 2 Activator (4) ?
KIR2DS1 B p50.1 CD158h AL133414 2 Activator (4) HLA-C (N77-K80, -Cw2, -Cw4, -Cw5, -Cw6)
KIR2DS4 A*** p50.3 CD158i - 2 Activator (4) ?
KIR2DS5 B***   CD158g AL133414 2 Activator (4) ?
KIR2DS2 B p50.2 CD158j AL133414 2 Activator (4) ?
KIR3DL2 A, Bp140 CD158k AL133414 3 Inhibitory ITIM HLA-A3, -A11?

HUGO nomenclature for LILR
HUGO nomenclature for KIR


IMGT notes:
(1)Number of C-LIKE -DOMAINs
(2)No transmembrane region, probably soluble
(3)Associated to FcRgamma (to be checked)
(4)Associated to TYROBP/DAP12/Karap
(5)Two related nonallelic KIR2DL5 genes were identified: KIR2DL5A and KIR2DL5B [5]. Haplotypes may comprise 0, 1 or 2 KIR2DL5 genes.
A and B indicates the presence of the gene in the haplotypes A and/or B, respectively. Asterisks (*, ** ou ***) indicate that the genes belonging to A and B haplotypes respectively, may be alleles of the same gene but this needs confirmation.

[1]Suto, Y. et al., Immunogenetics, 48, 235-241 (1998)
[2]Trowsdale, J., Immunity, 15, 363-374 (2001)
[3]Khakoo, S.I. et al., Immunity, 12, 687-698 (2000)
[4]André, P. et al., Nature Immunology, 2, 661 (2001)
[5]Gómez-Lozano, N. et al., Immunogenetics, 54, 314-319 (2002)
[6]The KIR Gene Cluster, Mary Carrington and Paul Norman, Bethesda (MD), National Library of Medicine (US), NCBI, 2003

Created: 14/11/2001
Last updated: Wednesday, 29-Jun-2011 17:01:52 CEST
Author: Marie-Paule Lefranc
Editors: Elodie Foulquier, Chantal Ginestoux
Contact: Marie-Paule Lefranc

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