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Details on Person UniProt:P10321 HLA-C

Class:IdReferenceGeneProduct:49109
_chainChangeLogsignal peptide:1-24 added on Fri February 6 2015;chain:25-366 added on Fri February 6 2015
_displayNameUniProt:P10321 HLA-C
_timestamp2024-11-03 19:51:33
chainsignal peptide:1-24
chain:25-366
checksum59C23D95FD1D0BC8
commentFUNCTION Antigen-presenting major histocompatibility complex class I (MHCI) molecule with an important role in reproduction and antiviral immunity (PubMed:11172028, PubMed:20104487, PubMed:20439706, PubMed:20972337, PubMed:24091323, PubMed:28649982, PubMed:29312307). In complex with B2M/beta 2 microglobulin displays a restricted repertoire of self and viral peptides and acts as a dominant ligand for inhibitory and activating killer immunoglobulin receptors (KIRs) expressed on NK cells (PubMed:16141329). In an allogeneic setting, such as during pregnancy, mediates interaction of extravillous trophoblasts with KIR on uterine NK cells and regulate trophoblast invasion necessary for placentation and overall fetal growth (PubMed:20972337, PubMed:24091323). During viral infection, may present viral peptides with low affinity for KIRs, impeding KIR-mediated inhibition through peptide antagonism and favoring lysis of infected cells (PubMed:20439706). Presents a restricted repertoire of viral peptides on antigen-presenting cells for recognition by alpha-beta T cell receptor (TCR) on HLA-C-restricted CD8-positive T cells, guiding antigen-specific T cell immune response to eliminate infected cells, particularly in chronic viral infection settings such as HIV-1 or CMV infection (PubMed:11172028, PubMed:20104487, PubMed:28649982). Both the peptide and the MHC molecule are recognized by TCR, the peptide is responsible for the fine specificity of antigen recognition and MHC residues account for the MHC restriction of T cells (By similarity). Typically presents intracellular peptide antigens of 9 amino acids that arise from cytosolic proteolysis via proteasome. Can bind different peptides containing allele-specific binding motifs, which are mainly defined by anchor residues at position 2 and 9. Preferentially displays peptides having a restricted repertoire of hydrophobic or aromatic amino acids (Phe, Ile, Leu, Met, Val and Tyr) at the C-terminal anchor (PubMed:25311805, PubMed:8265661).FUNCTION ALLELE C*01:02: The peptide-bound form interacts with KIR2DL2 and KIR2DL3 inhibitory receptors on NK cells. The low affinity peptides compete with the high affinity peptides impeding KIR-mediated inhibition and favoring lysis of infected cells (PubMed:20439706). Presents to CD8-positive T cells a CMV epitope derived from UL83/pp65 (RCPEMISVL), an immediate-early antigen necessary for initiating viral replication (PubMed:12947002).FUNCTION ALLELE C*04:01: Presents a conserved HIV-1 epitope derived from env (SFNCGGEFF) to memory CD8-positive T cells, eliciting very strong IFNG responses (PubMed:20104487). Presents CMV epitope derived from UL83/pp65 (QYDPVAALF) to CD8-positive T cells, triggering T cell cytotoxic response (PubMed:12947002).FUNCTION ALLELE C*05:01: Presents HIV-1 epitope derived from rev (SAEPVPLQL) to CD8-positive T cells, triggering T cell cytotoxic response.FUNCTION ALLELE C*06:02: In trophoblasts, interacts with KIR2DS2 on uterine NK cells and triggers NK cell activation, including secretion of cytokines such as GMCSF that enhances trophoblast migration.FUNCTION ALLELE C*07:02: Plays an important role in the control of chronic CMV infection. Presents immunodominant CMV epitopes derived from IE1 (LSEFCRVL and CRVLCCYVL) and UL28 (FRCPRRFCF), both antigens synthesized during immediate-early period of viral replication. Elicits a strong anti-viral CD8-positive T cell immune response that increases markedly with age.FUNCTION ALLELE C*08:01: Presents viral epitopes derived from CMV UL83 (VVCAHELVC) and IAV M1 (GILGFVFTL), triggering CD8-positive T cell cytotoxic response.FUNCTION ALLELE C*12:02: Presents CMV epitope derived from UL83 (VAFTSHEHF) to CD8-positive T cells.FUNCTION ALLELE C*15:02: Presents CMV epitope derived from UL83 CC (VVCAHELVC) to CD8-positive T cells, triggering T cell cytotoxic response.SUBUNIT Heterotrimer that consists of an alpha chain HLA-C, a beta chain B2M and a peptide (peptide-HLA-C-B2M) (PubMed:10850706, PubMed:24990997, PubMed:28649982). Early in biogenesis, HLA-C-B2M dimer interacts with the components of the peptide-loading complex composed of TAPBP, TAP1-TAP2, TAPBPL, PDIA3/ERP57 and CALR (PubMed:18420581). Interacts with TAP1-TAP2 transporter via TAPBP; this interaction is obligatory for the loading of peptide epitopes delivered to the endoplasmic reticulum (ER) by TAP1-TAP2 transporter (By similarity). Being very selective in the peptide binding, forms a stable interaction with TAP1-TAP2, often leading to the accumulation of free heavy chains in the ER (PubMed:18420581). Only optimally assembled peptide-HLA-C-B2M trimer translocates to the surface of antigen-presenting cells, where it interacts with TCR and CD8 coreceptor on the surface of T cells. HLA-C (via polymorphic alpha-1 and alpha-2 domains) interacts with antigen-specific TCR (via CDR3 domains) (By similarity). One HLA-C molecule (mainly via nonpolymorphic alpha-3 domain) interacts with one CD8A homodimer (via CDR-like loop); this interaction ensures peptide-HLA-C-B2M recognition by CD8-positive T cells only (By similarity). The peptide-HLA-C-B2M complex also interacts with KIRs. HLA-C type 1 (C1, with Asn104), including HLA-C*02, C*04, C*05, C*06 and C*15, interact with KIR2DL1 and KIR2DS1, and HLA-C type 2 (C2, with Lys104), including HLA-C*01, C*03, C*07 and C*08, interact with KIR2DL2 and KIR2DL3 (PubMed:10850706, PubMed:11323700, PubMed:16141329, PubMed:20439706, PubMed:20972337, PubMed:24091323).SUBUNIT (Microbial infection) Interacts with HTLV-1 p12I accessory protein.INTERACTION Ubiquitous. Highly expressed in fetal extravillous trophoblasts in the decidua basalis (at protein level).DOMAIN The alpha-1 domain is a structural part of the peptide-binding cleft.DOMAIN The alpha-2 domain is a structural part of the peptide-binding cleft (PubMed:10850706, PubMed:24990997, PubMed:28649982). Mediates the interaction with TAP1-TAP2 complex.DOMAIN The alpha-3 Ig-like domain mediates the interaction with CD8 coreceptor.DOMAIN The VL9 peptide/epitope (VMAPRT[V/L][L/V/I/F]L) derived from the signal sequence is loaded onto HLA-E and enables HLA-E expression at the plasma membrane. Distinct VL9 peptides presented by HLA-E variably affect its recognition by KLRD1-KLRC1 or KLRD1-KLRC2 receptors on NK cells, setting NK cell activation threshold. Common HLA-C allotypes contain functional VL9 peptides (VMAPRTLIL and VMAPRTLLL). VL9 peptides (VMAPRTALL and VMAPRQALL) derived from HLA-C*07, C*17 and C*18 allotypes display low affinity for HLA-E and fail to drive NK cell activation.PTM N-linked glycosylation at Asn-110 is required for efficient interaction with CANX and CALR chaperones and appropriate HLA-C-B2M folded conformers prior to peptide loading.POLYMORPHISM Displays lower polymorphism than HLA-A and HLA-B. Polymorphic residues encode for alpha-1 and alpha-2 domains of the peptide-binding cleft, where they contribute to variations in peptide binding and TCR recognition among different alleles. The human population is estimated to have millions of HLA-C alleles. But only 14 common HLA-C alleles are considered core alleles, representing all functionally significant variation (polymorphism) in alpha-1 and alpha-2 domains. These are: C*01:02; C*02:02; C*03:02; C*04:01; C*05:01; C*06:02; C*07:01; C*07:04; C*08:01; C*12:02; C*14:02; C*15:02; C*16:01 and C*17:01. Among these, C*01:02; C*02:02; C*03:02; C*08:01; C*12:02; C*14:02 and C*15:02, were likely passed by introgression from archaic to modern humans. Functional alleles of more recent origin (non-core) were derived by recombination (PubMed:28650991). The sequence shown is that of C*07:02. The sequences of core alleles and common representative alleles of serologically distinct allele groups are described as variants of C*07:02. Allelic variations of HLA-C signal peptide regulate HLA-E recognition by KLRD1-KLRC1 and KLRD1-KLRC2 receptors in viral infection and tumorigenesis by affecting its processing and by changing the affinity of HLA-E-VL9 complex for KLRD1-KLRC1 and KLRD1-KLRC2 receptors.DISEASE Disease susceptibility is associated with variants affecting the gene represented in this entry. Allele C*06:02 presents a melanocyte autoantigen ADAMTSL5 (VRSRRCLRL) to Valpha3S1/Vbeta13S1 TCR on CD8-positive T cells, and may trigger an autoimmune response against melanocytes.MISCELLANEOUS A transcript of allele C*16:01. This isoform lacks the transmembrane domain and is predicted to be a secreted protein.
descriptionrecommendedName: HLA class I histocompatibility antigen, C alpha chain shortName: HLA-C alternativeName: HLA-Cw alternativeName: Human leukocyte antigen C
geneNameHLA-C
HLAC
identifierP10321
isSequenceChangedFALSE
keyword3D-structure
Adaptive immunity
Alternative splicing
Cell membrane
Disulfide bond
Endoplasmic reticulum
Glycoprotein
Host-virus interaction
Immunity
Innate immunity
Membrane
MHC I
Phosphoprotein
Proteomics identification
Reference proteome
Signal
Transmembrane
Transmembrane helix
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9841277] Weiser, Joel
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9862192] Weiser, Joel, 2024-02-26
[InstanceEdit:9917590] Weiser, Joel, 2024-08-09
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
nameHLA-C
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8962164] ENSEMBL:ENSG00000204525 HLA-C [Homo sapiens]
secondaryIdentifierHLAC_HUMAN
O02863
O02864
O02865
O02866
O02958
O19505
O19652
O19676
O62879
O62882
O62883
O62888
O78060
O78061
O78062
O78063
O78067
O78068
O78069
O78072
O78083
O78090
O78091
O78149
O78165
O78166
O78178
O78202
O78203
O78211
O78214
P04222
P30499
P30500
P30501
P30502
P30503
P30504
P30505
P30506
P30507
P30508
P30509
P30510
P79498
Q07000
Q29631
Q29641
Q29643
Q29652
Q29743
Q29768
Q29862
Q29864
Q29865
Q29867
Q29921
Q29959
Q29960
Q29963
Q29986
Q29989
Q29990
Q29991
Q29992
Q29993
Q30192
Q31605
Q31627
Q860R1
Q860R2
Q95463
Q95603
Q95604
Q99528
Q9BD28
Q9GIK4
Q9GIK8
Q9GJ33
Q9MY30
Q9MY31
Q9MY35
Q9MY49
Q9MY74
Q9MYI3
Q9TNN7
Q9TNZ8
Q9TPS4
Q9TPV8
Q9TPX2
Q9TQB4
Q9TQJ5
Q9TQP9
Q9UM32
Q9UM33
Q9UM42
Q9UQS9
sequenceLength366
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:9666228] UniProt:P10321-1 HLA-C [Homo sapiens]
[ReferenceIsoform:9666229] UniProt:P10321-2 HLA-C [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:199595] HLA class I histocompatibility antigen, Cw-7 alpha chain precursor [plasma membrane] [Homo sapiens]
[EntityWithAccessionedSequence:983044] HLA class I histocompatibility antigen, Cw-7 alpha chain precursor [lumenal side of endoplasmic reticulum membrane] [Homo sapiens]
[EntityWithAccessionedSequence:983354] HLA class I histocompatibility antigen, Cw-7 alpha chain precursor [ER to Golgi transport vesicle membrane] [Homo sapiens]
[EntityWithAccessionedSequence:983387] HLA class I histocompatibility antigen, Cw-7 alpha chain precursor [Golgi membrane] [Homo sapiens]
[EntityWithAccessionedSequence:1236760] HLA class I histocompatibility antigen, Cw-7 alpha chain precursor [phagocytic vesicle membrane] [Homo sapiens]
[EntityWithAccessionedSequence:1236799] HLA class I histocompatibility antigen, Cw-7 alpha chain precursor [early endosome membrane] [Homo sapiens]
[EntityWithAccessionedSequence:6803292] HLA class I histocompatibility antigen, Cw-7 alpha chain precursor [secretory granule membrane] [Homo sapiens]
[EntityWithAccessionedSequence:8863915] HLA class I histocompatibility antigen, Cw-7 alpha chain precursor [recycling endosome membrane] [Homo sapiens]
(referenceSequence)[GroupModifiedResidue:9985191] N4-(N-acetylamino)glucosyl-L-asparagine (N-(α-Glc-(1→3)-α-Man-(1→2)-α-Man-(1→2)-α-Man-(1→3)-(α-Man-(1→2)-α-Man-(1→3)-(α-Man-(1→2)-α-Man-(1→6))-α-Man-(1→6))-β-Man-(1→4)-β-GlcNAc-(1→4)-β-GlcNAc)-L-Asn residue) at 110
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